Diseases of the Skin Appendages. Alopecia areata Characterized by rapid and complete loss of hair in one, or more often several round or oval patches

Diseases of the Skin Diseases of the Skin AppendagesAppendages

Alopecia areataAlopecia areata

Characterized by rapid and complete Characterized by rapid and complete loss of hair in one , or more often loss of hair in one , or more often several round or oval patchesseveral round or oval patches

Usually on the scalp, bearded areas, Usually on the scalp, bearded areas, eyebrows and eyelasheseyebrows and eyelashes

1-5 cm 1-5 cm May progress to total loss of scalp hair May progress to total loss of scalp hair

– alopecia totalis, or hair loss involving – alopecia totalis, or hair loss involving the entire body – alopecia universalisthe entire body – alopecia universalis

Ophiasis/sisaiphoOphiasis/sisaipho

Alopecia areataAlopecia areata

Alopecia totalisAlopecia totalis

Usually occurs Usually occurs without associated without associated diseasedisease

However, there is a However, there is a higher incidence in higher incidence in patients with atopic patients with atopic dermatitis, Down dermatitis, Down syndrome, LP, LE, syndrome, LP, LE, thyroiditis, MG and thyroiditis, MG and vitiligovitiligo

Nails may develop Nails may develop uniform pits that uniform pits that may form transverse may form transverse or longitudinal lines or longitudinal lines – 10 %– 10 %

etiologyetiology

Cause unknownCause unknown Most evidence points toward its being an Most evidence points toward its being an

autoimmune disease mediated by the cellular autoimmune disease mediated by the cellular arm and modified by genetic factorsarm and modified by genetic factors

25% report family history25% report family history Patients with early onset, severe, familial Patients with early onset, severe, familial

clustering alopecia areata have a unique and clustering alopecia areata have a unique and highly significant association with HLA highly significant association with HLA antigens DR4, DR11, and DQ7antigens DR4, DR11, and DQ7

histologyhistology In early disease there is a helper T-cell In early disease there is a helper T-cell

lymphocytic infiltrate in the peribulbar area lymphocytic infiltrate in the peribulbar area of anagen or early catagen folliclesof anagen or early catagen follicles

DDXDDX Tinea capitis, early lupus erythematosis, Tinea capitis, early lupus erythematosis,

syphilis, congenital triangular alopecia, syphilis, congenital triangular alopecia, alopecia neoplastica and trichotillomaniaalopecia neoplastica and trichotillomania

TXTX

Intralesional injections of corticosteroid Intralesional injections of corticosteroid suspensions, q 6 weekssuspensions, q 6 weeks

High-strength topical steroid creamsHigh-strength topical steroid creams AnthralinAnthralin Minoxidil alone or combined therapyMinoxidil alone or combined therapy

Spontaneous recovery is extremely commonSpontaneous recovery is extremely common Tendency is for spontaneous recovery in Tendency is for spontaneous recovery in

patients who are post pubertal at onsetpatients who are post pubertal at onset Predictors of poor prognosis include the Predictors of poor prognosis include the

presence of atopic dermatitis, childhood onset, presence of atopic dermatitis, childhood onset, widespread involvement, ophiasis, duration of widespread involvement, ophiasis, duration of longer than 5 years, and onychodystrophylonger than 5 years, and onychodystrophy

Telogen effluviumTelogen effluvium

Early and excessive loss of normal club Early and excessive loss of normal club hairs from normal resting follicles in the hairs from normal resting follicles in the scalpscalp

Loss results from traumatization of the Loss results from traumatization of the normal hair by some stimulus, such as normal hair by some stimulus, such as surgery, parturition, fever, drugs, or surgery, parturition, fever, drugs, or traction which precipitates the anagen traction which precipitates the anagen phase into catagen and telogen phasesphase into catagen and telogen phases

Follicle is not diseased and Follicle is not diseased and inflammation is absentinflammation is absent

Telogen effluviumTelogen effluvium

““lots of hairs coming out by the roots”lots of hairs coming out by the roots” Loss is diffuse and only infrequently Loss is diffuse and only infrequently

causing clinically perceptible thinning of causing clinically perceptible thinning of hairhair

Normal telogen count is below 10%Normal telogen count is below 10% Can be estimated by the pull testCan be estimated by the pull test Grasping 40 hairs firmly between thumb Grasping 40 hairs firmly between thumb

and forefinger, followed by a slow pulland forefinger, followed by a slow pull Greater than 4-6 club hairs is abnormalGreater than 4-6 club hairs is abnormal

100 – 150 hairs lost daily100 – 150 hairs lost daily 150 – 400 lost in telogen effluvium150 – 400 lost in telogen effluvium

Postpartum telogen effluviumPostpartum telogen effluvium

Between 2-6 months postpartumBetween 2-6 months postpartum Diffuse pattern, may be first noted over Diffuse pattern, may be first noted over

anterior scalpanterior scalp May continue for another 2-6 monthsMay continue for another 2-6 months

Drug induced telogen effluviumDrug induced telogen effluvium

Amphetamines, aminosalicyclic acid, Amphetamines, aminosalicyclic acid, bromocriptine, captopril, coumarin, bromocriptine, captopril, coumarin, carbamazepine, cimetidine, danazol, carbamazepine, cimetidine, danazol, enalapril, lithium carbonate, levodopa, enalapril, lithium carbonate, levodopa, metyrapone, metoprolol, propranolol, metyrapone, metoprolol, propranolol, pyridostigimine and trimethadionepyridostigimine and trimethadione

TXTX

No specific therapyNo specific therapy In the majority of cases it will stop In the majority of cases it will stop

spontaneously within a few months and spontaneously within a few months and the hair will regrowthe hair will regrow

Chronic form may occurChronic form may occur 5% minoxidil solution5% minoxidil solution educationeducation

Anagen effluviumAnagen effluvium

Seen frequently following the administration of Seen frequently following the administration of cancer chemotherapeutic agents, such as cancer chemotherapeutic agents, such as antimetabolites, alkylating agents, and mitotic antimetabolites, alkylating agents, and mitotic inhibitorsinhibitors

Only anagen hairs are involvedOnly anagen hairs are involved With cessation of the drug the follicle resumes With cessation of the drug the follicle resumes

normal activity within a few weeksnormal activity within a few weeks Process being entirely reversibleProcess being entirely reversible Topical minoxidil may decrease the period of Topical minoxidil may decrease the period of

baldness by an average of 50 daysbaldness by an average of 50 days

Anagen effluviumAnagen effluvium

Androgenetic alopeciaAndrogenetic alopeciamale-pattern baldnessmale-pattern baldness

Shows itself during the twenties of early Shows itself during the twenties of early thirties by gradual loss of hair, chiefly from thirties by gradual loss of hair, chiefly from the vertex and frontotemporal regionsthe vertex and frontotemporal regions

Several patterns of this type occurSeveral patterns of this type occur Most frequent is the biparietal recession Most frequent is the biparietal recession

with loss of hair on the vertexwith loss of hair on the vertex Rate variesRate varies

The exact mechanisms responsible for The exact mechanisms responsible for androgenetic alopecia are still unknown, androgenetic alopecia are still unknown, however, there is no doubt that inherited however, there is no doubt that inherited factors and the effect of androgens on the factors and the effect of androgens on the hair follicle are most responsiblehair follicle are most responsible

Pathogenesis involves lengthening of Pathogenesis involves lengthening of telogen phase and shortening of the telogen phase and shortening of the anagen phase anagen phase

TXTX

MinoxidilMinoxidil 2% and 5%2% and 5% Indefinite treatment to maintain a Indefinite treatment to maintain a

responseresponse 1/3 cases grow cosmetically useful hair1/3 cases grow cosmetically useful hair

FinasterideFinasteride Effective in preventing further hair loss and Effective in preventing further hair loss and

in increasing the hair countsin increasing the hair counts

Hair transplantationHair transplantation

Androgenetic alopecia in Androgenetic alopecia in womenwomen

Generally diffuse hair loss throughout Generally diffuse hair loss throughout the midscalp, sparing the frontal hairline the midscalp, sparing the frontal hairline except for slight recessionexcept for slight recession

The midline part is an important clinical The midline part is an important clinical clue to the diagnosis, revealing this clue to the diagnosis, revealing this central thinning by the appearance of central thinning by the appearance of the “Christmas tree pattern”the “Christmas tree pattern”

The cause is now believed to be a The cause is now believed to be a genetic predisposition in combination genetic predisposition in combination with an excessive androgen response with an excessive androgen response

TXTX

Topical minoxidil 2%Topical minoxidil 2%

Androgenetic alopecia in Androgenetic alopecia in womenwomen

Other forms of alopeciaOther forms of alopecia

trichotillomaniatrichotillomania

A neurotic practice of plucking or breaking hair A neurotic practice of plucking or breaking hair from the scalp or eyelashesfrom the scalp or eyelashes

Usually localized but may be widespreadUsually localized but may be widespread Areas of alopecia characteristically contain hairs Areas of alopecia characteristically contain hairs

of various lengthsof various lengths Seen mostly in girls under 10, may also be seen Seen mostly in girls under 10, may also be seen

in boys and adultsin boys and adults

It has been suggested that one ask the child not It has been suggested that one ask the child not if but rather how the removal is doneif but rather how the removal is done

Shave 3 X 3 cm area and watch the hair regrow Shave 3 X 3 cm area and watch the hair regrow normally. Hairs in this ‘skin window” will be too normally. Hairs in this ‘skin window” will be too short for pluckingshort for plucking

Biopsy – high number of catagen hairs, Biopsy – high number of catagen hairs, pigmentary defects and casts, trichomalacia and pigmentary defects and casts, trichomalacia and heorrhageheorrhage

trichotillomaniatrichotillomania May be a May be a

manifestation of manifestation of obsessive-obsessive-compulsive disordercompulsive disorder

May be assoc with May be assoc with depression or depression or anxietyanxiety

TX – psychotherapy, TX – psychotherapy, behavioral therapy behavioral therapy or an appropriate or an appropriate psychopharmacologipsychopharmacologic medicationc medication

fluoxetinefluoxetine

trichotillomaniatrichotillomania

Hot comb alopeciaHot comb alopecia

Develops characteristically on the crown Develops characteristically on the crown and spreads peripherally to form a large and spreads peripherally to form a large oval area of partial hair lossoval area of partial hair loss

Initially reported in black women who Initially reported in black women who straightened their hair with hot combsstraightened their hair with hot combs

The hot petrolatum used with the iron The hot petrolatum used with the iron causes thermal damage to the hair folliclecauses thermal damage to the hair follicle

Pseudopelade of BrocqPseudopelade of Brocq(alopecia cicatrisata)(alopecia cicatrisata)

A rare form of scarring alopeciaA rare form of scarring alopecia Destruction of the hair follicle produces Destruction of the hair follicle produces

multiple, round, oval, or irregularly shaped, multiple, round, oval, or irregularly shaped, hairless, cicatricial patches of varying hairless, cicatricial patches of varying sizessizes

Coin sized and white or slightly pink Coin sized and white or slightly pink colored with a smooth, shiny, marble like colored with a smooth, shiny, marble like or ivory, atrophic, “onion skin” surfaceor ivory, atrophic, “onion skin” surface

Inflammation is completely absentInflammation is completely absent No pustules, crusts, or broken off hairsNo pustules, crusts, or broken off hairs Insidious onsetInsidious onset Permanent and slowly progressivePermanent and slowly progressive Topical and intralesional steroids, long-Topical and intralesional steroids, long-

term TCN in antiinflammatory doses- not term TCN in antiinflammatory doses- not often successfuloften successful

pseudopeladepseudopelade

Traction alopeciaTraction alopecia

Occurs from prolonged tension on the hairOccurs from prolonged tension on the hair Either from the hair tightly braided or in a Either from the hair tightly braided or in a

ponytailponytail

Tractional alopeciaTractional alopecia

Pressure alopeciaPressure alopecia

Pressure alopeciaPressure alopecia

Occurs frequently on the occipital areas of Occurs frequently on the occipital areas of babies lying on their backsbabies lying on their backs

In adults it is seen most often after In adults it is seen most often after prolonged pressure on the scalp during prolonged pressure on the scalp during general anesthesia, with the head fixed in general anesthesia, with the head fixed in one positionone position

Most likely due to pressure induced Most likely due to pressure induced ischemiaischemia

Loose anagen syndromeLoose anagen syndrome

Loose anagen syndromeLoose anagen syndrome

Disorder in which anagen hairs may be Disorder in which anagen hairs may be pulled from the scalp with little effortpulled from the scalp with little effort

Occurs mostly in blonde girls Occurs mostly in blonde girls Improves with age Improves with age

Alopecia syphiliticaAlopecia syphilitica

May have a typical moth-eaten May have a typical moth-eaten appearance on the occipital scalp, a appearance on the occipital scalp, a generalized thinning, or a combination of generalized thinning, or a combination of the twothe two

Eyebrows and lashes, and other body hair Eyebrows and lashes, and other body hair may be involvedmay be involved

May be the first sign of a syphilis infectionMay be the first sign of a syphilis infection



Follicular mucinosisFollicular mucinosis(alopecia mucinosa)(alopecia mucinosa)

Most commonly occurs on the scalp or beard Most commonly occurs on the scalp or beard areaarea

Manifest as deposition of mucin in the outer Manifest as deposition of mucin in the outer root sheath and sebaceous glandsroot sheath and sebaceous glands

Inflammatory reaction produces alopecia, and Inflammatory reaction produces alopecia, and at times hypopigmentationat times hypopigmentation

Primary cases occur either as localized Primary cases occur either as localized lesions of the head or neck typically resloving lesions of the head or neck typically resloving within a yearwithin a year

Follicular mucinosisFollicular mucinosis(alopecia mucinosa)(alopecia mucinosa)

More generalized lesion have a longer More generalized lesion have a longer coursecourse

Young people are primarily affectedYoung people are primarily affected A secondary type – associated with A secondary type – associated with

cutaneous T-cell lymphoma, usually more cutaneous T-cell lymphoma, usually more widespread and in older personswidespread and in older persons

Follicular mucinosisFollicular mucinosis

Inflammatory alopeciaInflammatory alopecia

May be seen in lichen simplex chronicus May be seen in lichen simplex chronicus and various eczematous changes on the and various eczematous changes on the scalp, including kerionscalp, including kerion

DLE, lichen planopilaris, sarcoidosis, and DLE, lichen planopilaris, sarcoidosis, and folliculitis decalvans are the commonest folliculitis decalvans are the commonest inflammatory causes of cicatricial alopeciainflammatory causes of cicatricial alopecia

Vascular or neurologic alopeciaVascular or neurologic alopecia

Most often of the lower extremitiesMost often of the lower extremities May be seen in diabetes mellitus or May be seen in diabetes mellitus or

atherosclerosisatherosclerosis

Endocrinologic alopeciaEndocrinologic alopecia

May occur in various endocrinologic May occur in various endocrinologic disordersdisorders

Hypothyroidism – hair becomes dry, Hypothyroidism – hair becomes dry, brittle, coarse and sparsebrittle, coarse and sparse

Hyperthyroidism – hair becomes Hyperthyroidism – hair becomes extremely fine and sparseextremely fine and sparse

Some women develop telogen effluvium Some women develop telogen effluvium 2-4 months after discontinuing 2-4 months after discontinuing anovulatory agentsanovulatory agents

Tumor alopeciaTumor alopecia

Refers to hair loss in the immediate Refers to hair loss in the immediate vicinity of either benign or malignant vicinity of either benign or malignant tumors of the scalptumors of the scalp

Syringomas, nerve sheath myxomas, Syringomas, nerve sheath myxomas, and steatocytoma multiplexand steatocytoma multiplex

Alopecia neoplastica – hair loss from Alopecia neoplastica – hair loss from metastatic tumors, most commonly metastatic tumors, most commonly breast carcinomabreast carcinoma

Congenital alopeciaCongenital alopecia

Occurs either as total or partial hair loss, or Occurs either as total or partial hair loss, or lack of initial growthlack of initial growth

Accompanied usually by other ectodermal Accompanied usually by other ectodermal defects of the nails, teeth and bonedefects of the nails, teeth and bone

Hair is light and sparse, and grows slowlyHair is light and sparse, and grows slowly Congenital triangular alopecia and aplasia Congenital triangular alopecia and aplasia

cutis congenita are examples of congenital cutis congenita are examples of congenital localized absence of hairlocalized absence of hair

Hidrotic ectodermal dysplasia - diffuseHidrotic ectodermal dysplasia - diffuse

Syndromes that include Syndromes that include abnormalities of the hairabnormalities of the hair

Albright’s diseaseAlbright’s disease(polyostotic fibrous dysplasia)(polyostotic fibrous dysplasia)

May present as slowly progressive lifelong May present as slowly progressive lifelong unilateral hair lossunilateral hair loss

Scalp, pubic, axillary, and palpebralScalp, pubic, axillary, and palpebral

Sickle cell disease is often characterized Sickle cell disease is often characterized by scantiness of body and facial hairby scantiness of body and facial hair

Cronkhite-Canada syndrome Cronkhite-Canada syndrome characterized by alopecia, skin characterized by alopecia, skin pigmentation, onychodystrophy, pigmentation, onychodystrophy, malabsorption, and generalized malabsorption, and generalized gastrointestinal polyposisgastrointestinal polyposis

Marinesco-Sjorgren syndromeMarinesco-Sjorgren syndrome

Cerebellar ataxia, mental retardation, Cerebellar ataxia, mental retardation, congenital cataracts, inability to chew congenital cataracts, inability to chew food, thin brittle fingernails, and sparse food, thin brittle fingernails, and sparse hairhair

Autosomal recessive inheritanceAutosomal recessive inheritance

trichothiodystrophytrichothiodystrophy

Brittle hair with Brittle hair with markedly reduced markedly reduced sulfur contentsulfur content

Sulfur reduced 50% Sulfur reduced 50% of normal valueof normal value

Distinctive features Distinctive features under polarized light under polarized light and scanning electron and scanning electron microscopymicroscopy


With light microscopy With light microscopy trichoschisis (clean trichoschisis (clean fractures) may be fractures) may be seenseen

With polarized With polarized microscopy the hair microscopy the hair shows alternating shows alternating bright and dark bright and dark regionsregions



Hair findings present in all casesHair findings present in all cases Other variable features include short Other variable features include short

stature, mental deficiency, ichthyosis, nail stature, mental deficiency, ichthyosis, nail dystrophy, ocular dysplasia, dystrophy, ocular dysplasia, photosensitivity and infertilityphotosensitivity and infertility

Various names associated with different Various names associated with different combinations Brown’s syndrome, BIDS, combinations Brown’s syndrome, BIDS, IBIDS, PIBIDS and Tay’s syndromeIBIDS, PIBIDS and Tay’s syndrome


Different mutations in the ERCC2 DNA Different mutations in the ERCC2 DNA repair gene may lead to different repair gene may lead to different phenotypic expressions of the diseasephenotypic expressions of the disease

Generalized hair follicle Generalized hair follicle hamartoma syndromehamartoma syndrome

Generalized trichoepitheliomas, alopecia Generalized trichoepitheliomas, alopecia and myasthenia gravisand myasthenia gravis

Localized case reportLocalized case report

POEMS syndromePOEMS syndrome(Crow-Fukase syndrome)(Crow-Fukase syndrome)

PPolyneuropathyolyneuropathy OOrganomegalyrganomegaly EEndocrinopathyndocrinopathy MM-protein-protein SSkin changeskin changes

Diffuse hyperpigmentation, dependent Diffuse hyperpigmentation, dependent edema, skin thickening, hyperhidrosis edema, skin thickening, hyperhidrosis and hypertrichosisand hypertrichosis

POEMS syndromePOEMS syndrome(Crow-Fukase syndrome)(Crow-Fukase syndrome)

POEMS POEMS syndromesyndrome

Cartilage-hair hypoplasiaCartilage-hair hypoplasia

Short limbed dwarfism and abnormally fine Short limbed dwarfism and abnormally fine and sparse hair in childrenand sparse hair in children

Especially susceptible to viral infections Especially susceptible to viral infections and recurrent respiratory infectionsand recurrent respiratory infections

AIDS patientsAIDS patients

Many black patient with AIDS have Many black patient with AIDS have experienced softening, straightening, experienced softening, straightening, lightening and thinning of their hairlightening and thinning of their hair

HIV patients may also experience HIV patients may also experience elongated eyelashes and telogen effluviumelongated eyelashes and telogen effluvium

Tricho-rhino-phalangeal Tricho-rhino-phalangeal syndromesyndrome

Genetic disorder consisting of fine and Genetic disorder consisting of fine and sparse scalp hair, thin nails, pear-shaped sparse scalp hair, thin nails, pear-shaped broad nose, and cone shaped epiphyses broad nose, and cone shaped epiphyses of the middle phalanges of some fingers of the middle phalanges of some fingers and toesand toes

Autosomal dominant and recessive typesAutosomal dominant and recessive types

Lipedematous alopeciaLipedematous alopecia

Consists of shortened hairs, with Consists of shortened hairs, with thickening of the scalp associated with an thickening of the scalp associated with an increase in subcutaneous fatincrease in subcutaneous fat

Scalp may be as much as 15 mm thickScalp may be as much as 15 mm thick Primarily affects persons of colorPrimarily affects persons of color

Hallerman-Streiff syndromeHallerman-Streiff syndrome

Rare conditionRare condition Bird-like facies with a pronounced Bird-like facies with a pronounced

beaklike nose, microphthalmia, beaklike nose, microphthalmia, micrognathia, congenital cataracts, and micrognathia, congenital cataracts, and hypotrichosishypotrichosis

Hair is diffusely sparse and brittleHair is diffusely sparse and brittle Sutural alopecia is characteristic of this Sutural alopecia is characteristic of this

syndrome – hair loss following the lines syndrome – hair loss following the lines of the cranial suturesof the cranial sutures

Hallerman-Streiff syndromeHallerman-Streiff syndrome

Small face with Small face with disproportionately disproportionately large headlarge head

ProgeriaProgeria(Hutchinson-Gilford syndrome)(Hutchinson-Gilford syndrome)

Characterized by the appearance of Characterized by the appearance of premature agingpremature aging

Marked by failure to develop normally in Marked by failure to develop normally in growth after the first year of lifegrowth after the first year of life

Large bald head and lack of eyebrows and Large bald head and lack of eyebrows and eyelashes are distinctiveeyelashes are distinctive

Skin is wrinkled, pigmented and atrophicSkin is wrinkled, pigmented and atrophic


Nails are thin and atrophicNails are thin and atrophic Most patient lack subcutaneous fatMost patient lack subcutaneous fat Intelligence remains intactIntelligence remains intact Arteriosclerosis, anginal attacks, and Arteriosclerosis, anginal attacks, and

hemiplegia may occur, followed by death hemiplegia may occur, followed by death from coronary heart disease at an early from coronary heart disease at an early ageage


Papillon-Lefevre syndromePapillon-Lefevre syndrome

Hyperkeratosis palmaris et plantaris, Hyperkeratosis palmaris et plantaris, periodontosis, and sparsity of the hairperiodontosis, and sparsity of the hair

Hyperhidrosis and other signs and Hyperhidrosis and other signs and symptoms begin early in lifesymptoms begin early in life

Autosomal recessiveAutosomal recessive

Klippel-Feil syndromeKlippel-Feil syndrome

Consists of low posterior scalp hairline Consists of low posterior scalp hairline extending onto the shoulders, with a extending onto the shoulders, with a short neck, limiting movement of the short neck, limiting movement of the neck, and suggestive of webbingneck, and suggestive of webbing

Cervical vertebrae are fusedCervical vertebrae are fused Strabismus, nystagmus, cleft palate, Strabismus, nystagmus, cleft palate,

bifid uvula, and high palate are other bifid uvula, and high palate are other featuresfeatures

Occurs mostly in girlsOccurs mostly in girls

Klippel-Feil syndromeKlippel-Feil syndrome

McCusick’s syndromeMcCusick’s syndrome

Includes short-limbed dwarfism and fine, Includes short-limbed dwarfism and fine, sparse, hypoplastic and dysmorphic hairsparse, hypoplastic and dysmorphic hair

Turner’s syndromeTurner’s syndrome

Short stature, webbing of the neck, low Short stature, webbing of the neck, low posterior hairline margin, increased posterior hairline margin, increased carrying angle of the elbows (cubitus carrying angle of the elbows (cubitus valgus), and infantile development of the valgus), and infantile development of the breasts, vagina and uterusbreasts, vagina and uterus

Coarctation of the aorta is frequentCoarctation of the aorta is frequent Alopecia of the frontal scalp and cutis laxaAlopecia of the frontal scalp and cutis laxa

Turner’s syndromeTurner’s syndrome

Caused by ovarian dysgenesisCaused by ovarian dysgenesis Only 45 chromosomes presentOnly 45 chromosomes present Sex chromosomes have an XO patternSex chromosomes have an XO pattern

Noonan’s syndromeNoonan’s syndrome

Short stature with atypical webbing of the neck, Short stature with atypical webbing of the neck, low hairline in the back, prominent and low-set low hairline in the back, prominent and low-set ears, and cubitus valgus.ears, and cubitus valgus.

Similar to Turner’s and frequently termed male Similar to Turner’s and frequently termed male Turner’s syndromeTurner’s syndrome

Absence of coarctation of the aortaAbsence of coarctation of the aorta Valvular pulmonary stenosis is the typical heart Valvular pulmonary stenosis is the typical heart

lesionlesion

Noonan’s syndromeNoonan’s syndrome

Werner’s syndromeWerner’s syndrome

Shortness of stature, cataracts, skin changes, Shortness of stature, cataracts, skin changes, premature graying and alopecia, atrophy of premature graying and alopecia, atrophy of the muscles and subcutaneous tissue, and the muscles and subcutaneous tissue, and bone atrophy of the extremities to produce bone atrophy of the extremities to produce spindly extremitiesspindly extremities

Skin changes include poikiloderma, Skin changes include poikiloderma, scleroderma, atrophy, hyperkeratosis and leg scleroderma, atrophy, hyperkeratosis and leg ulcersulcers

Skin has a dark gray or blackish diffuse Skin has a dark gray or blackish diffuse pigmentationpigmentation


A high-pitched voice and hypogonadism in A high-pitched voice and hypogonadism in both sexes are distinctive in this syndromeboth sexes are distinctive in this syndrome

Diabetes mellitus is frequently presentDiabetes mellitus is frequently present Because most of the signs are not fully Because most of the signs are not fully

manifested before the age of 30, the manifested before the age of 30, the diagnosis is usually made in middle agediagnosis is usually made in middle age

Autosomal recessiveAutosomal recessive DNA helicase mutationDNA helicase mutation Patients usually die before they are 50 from Patients usually die before they are 50 from

malignant disease or vascular accidentmalignant disease or vascular accident


Rothmund-Thomson syndromeRothmund-Thomson syndrome

Characterized by early onset poikiloderma, Characterized by early onset poikiloderma, short stature, sun sensitivity, bone defects, short stature, sun sensitivity, bone defects, and hypogonadismand hypogonadism

Sparseness of eyelashes, eyebrows, or Sparseness of eyelashes, eyebrows, or scalp hair has been reported in 60% of scalp hair has been reported in 60% of casescases

Mutation in a DNA helicase geneMutation in a DNA helicase gene

Rothmund-Thomson syndromeRothmund-Thomson syndrome

Rothmund-Thomson Rothmund-Thomson syndromesyndrome

HAIR COLORHAIR COLOR

Black and brown hair – eumalaninBlack and brown hair – eumalanin Blonde and red hair – pheomelaninBlonde and red hair – pheomelanin Changes in hair color occur in various Changes in hair color occur in various

metabolic disordersmetabolic disorders Phenylketonuria hair becomes blond Phenylketonuria hair becomes blond Bleaching effect in homocystinuriaBleaching effect in homocystinuria Oasthouse diseaseOasthouse disease albinismalbinism

Triparanol assoc. with hypopigmented hairTriparanol assoc. with hypopigmented hair Minoxidil causes darkeningMinoxidil causes darkening Diazoxide gives reddish tintDiazoxide gives reddish tint Chloroquine therapy may cause whiteningChloroquine therapy may cause whitening Kwashiorkor red-blonde color in infantsKwashiorkor red-blonde color in infants B12 deficiency and interferon therapy may B12 deficiency and interferon therapy may

cause whiteningcause whitening Canities segmentata sideropenica – Canities segmentata sideropenica –

alternating light and dark bands seen with alternating light and dark bands seen with iron-deficiency anemiairon-deficiency anemia

Gray hair (canities) melanogenic activity Gray hair (canities) melanogenic activity is decreased as a result of fewer is decreased as a result of fewer melanocytes and melanosomes as well melanocytes and melanosomes as well as gradual loss of tyrosinease activityas gradual loss of tyrosinease activity

Genetically determined and may start at Genetically determined and may start at any ageany age

Early graying (before age 20 in whites Early graying (before age 20 in whites 30 in blacks) usually familial, may occur 30 in blacks) usually familial, may occur in progeria, Rothmund-Thomson in progeria, Rothmund-Thomson syndrome, Book’s syndrome, and syndrome, Book’s syndrome, and Werner’s syndromeWerner’s syndrome

poliosispoliosis

Gray hair occurring in circumscribed patchesGray hair occurring in circumscribed patches Waardenburg’s syndrome, piebaldism, and Waardenburg’s syndrome, piebaldism, and

tuberous sclerosistuberous sclerosis Also assoc with vitiligo and Vogt-Koyanagi Also assoc with vitiligo and Vogt-Koyanagi

syndromesyndrome May be seen in alopecia areata with new hair May be seen in alopecia areata with new hair

growthgrowth Tietze’s syndrome, Alezzandrini’s syndrome, Tietze’s syndrome, Alezzandrini’s syndrome,

and NFand NF May signal a regressing area of a scalp May signal a regressing area of a scalp

melanomamelanoma

poliosis in vitiligopoliosis in vitiligo

poliosispoliosis

Green hair has been trace to copper in the Green hair has been trace to copper in the water of swimming poolswater of swimming pools

Occurs only in blonde and light hairOccurs only in blonde and light hair Premature whitening of the scalp hair is Premature whitening of the scalp hair is

usually caused by vitiligo, sometimes usually caused by vitiligo, sometimes without recognized, or actually without, without recognized, or actually without, lesions of glabrous skinlesions of glabrous skin

HAIR STRUCTURE HAIR STRUCTURE DEFECTSDEFECTS

Hair castsHair casts

Mimics nits closelyMimics nits closely Affects women chieflyAffects women chiefly Results from long term and frequent tractionsResults from long term and frequent tractions Many scalp hairs bear a white keratinous Many scalp hairs bear a white keratinous

sleeve about 3-5 mm long, which lies within sleeve about 3-5 mm long, which lies within 1-3 cm of the scalp surface1-3 cm of the scalp surface

Unlike a nit can be slid along the hair shaft Unlike a nit can be slid along the hair shaft Their bluish yellow fluorescence under Their bluish yellow fluorescence under

Wood’s light may cause them to be confused Wood’s light may cause them to be confused with tinea capitiswith tinea capitis

Hair castsHair casts

Formed by retention and desquamation of Formed by retention and desquamation of segments of the root sheathssegments of the root sheaths

0.025% tretinoin lotion has been found to 0.025% tretinoin lotion has been found to be effectivebe effective

Pili tortiPili torti(twisted hairs)(twisted hairs)

A malformation of hair characterized by A malformation of hair characterized by twisting of the hair shaft on its own axistwisting of the hair shaft on its own axis

The hair shaft is segmentally thickened The hair shaft is segmentally thickened and light and dark segments are seenand light and dark segments are seen

Scalp, eyebrows, and eyelashesScalp, eyebrows, and eyelashes Hairs are brittle and easily brokenHairs are brittle and easily broken Typically occurs in childhood and Typically occurs in childhood and

improves by pubertyimproves by puberty


May be associated with patchy alopeciaMay be associated with patchy alopecia Follows a dominant inheritance patternFollows a dominant inheritance pattern May be assoc with Bjornstad’s syndrome, May be assoc with Bjornstad’s syndrome,

citrullinemia, Menkes’ kinky hair citrullinemia, Menkes’ kinky hair syndrome, Bazex’s follicular atrophoderma syndrome, Bazex’s follicular atrophoderma syndrome trichothiodystrophysyndrome trichothiodystrophy

Assoc with etretinate and isotretinoin Assoc with etretinate and isotretinoin

Pili tortiPili torti

Pili tortiPili torti


Menkes’ Kinky Hair SyndromeMenkes’ Kinky Hair Syndrome(steely hair disease)(steely hair disease)

Patients have deficiency of serum copper Patients have deficiency of serum copper and copper-dependent enzymesand copper-dependent enzymes

Pili torti, and often monilethrix and Pili torti, and often monilethrix and trichorrhexis nodosa are commontrichorrhexis nodosa are common

Sex-linked recessive disorderSex-linked recessive disorder Seen only in boysSeen only in boys Characteristic ivory color of the hair Characteristic ivory color of the hair

appears between 1 and 5 monthsappears between 1 and 5 months


Drowsiness, lethargy, convulsive seizures, and Drowsiness, lethargy, convulsive seizures, and severe neurologic deterioration, with periodic severe neurologic deterioration, with periodic hypothermia ensuehypothermia ensue

Hairs become wiry, sparse, fragile, and twisted Hairs become wiry, sparse, fragile, and twisted about their long axisabout their long axis

Skin is pale and the face is pudgySkin is pale and the face is pudgy Upper lip has an exaggerated “cupid’s bow”Upper lip has an exaggerated “cupid’s bow”



A distinctive neurochemical pattern in the A distinctive neurochemical pattern in the plasma allows for early diagnosisplasma allows for early diagnosis

Institution of copper histidine treatment Institution of copper histidine treatment has shown promising results in some has shown promising results in some infantsinfants

The occipital horn syndrome is a milder The occipital horn syndrome is a milder variantvariant

UncombableUncombablehair syndromehair syndrome

Uncombable hair syndromeUncombable hair syndrome(pili triangulati et canaliculi)(pili triangulati et canaliculi)

Noted in the first years of life as dry, blonde, Noted in the first years of life as dry, blonde, shiny hair that stands straight out from the scalp shiny hair that stands straight out from the scalp and cannot be combedand cannot be combed

Longitudinal grooves make hair rigidLongitudinal grooves make hair rigid Autosomal dominant inheritanceAutosomal dominant inheritance Sporadic cases reportedSporadic cases reported Some cases improve spontaneously in Some cases improve spontaneously in

childhoodchildhood

UncombableUncombablehair syndromehair syndrome

MonilethrixMonilethrix(beaded hairs)(beaded hairs)

Characterized by dryness, fragility, and Characterized by dryness, fragility, and sparseness of the scalp hair, with fusiform or sparseness of the scalp hair, with fusiform or spindle-shaped swellings of the hair shaft spindle-shaped swellings of the hair shaft separated by narrow atrophic segmentsseparated by narrow atrophic segments

Hair tends to break at the delicate internodesHair tends to break at the delicate internodes Involvement of the hair may occur during Involvement of the hair may occur during

pregnancy and resolve after deliverypregnancy and resolve after delivery


Improvement may occur with ageImprovement may occur with age May be seasonal improvement during the May be seasonal improvement during the

summersummer Autosomal dominantAutosomal dominant Has been described in assoc with Menkes’Has been described in assoc with Menkes’ Disease of the hair cortexDisease of the hair cortex No effective treatmentNo effective treatment



Trichorrhexis nodosaTrichorrhexis nodosa

Affected hair shafts may have small white Affected hair shafts may have small white nodes arranged at irregular intervalsnodes arranged at irregular intervals

The nodes are the sites of fracture of the hair The nodes are the sites of fracture of the hair cortexcortex

Hairs soon break at the nodesHairs soon break at the nodes Found mostly on the scalpFound mostly on the scalp Often in a small area or areasOften in a small area or areas Pubic area, axillae and chest may be involvedPubic area, axillae and chest may be involved


Has been described in assoc with Has been described in assoc with arginosuccinicaciduria, Menkes’ kinky hair arginosuccinicaciduria, Menkes’ kinky hair syndrome, Netherton’s syndrome, syndrome, Netherton’s syndrome, hypothyroidismhypothyroidism


ProximalProximal Distal (split ends)Distal (split ends) Acquired localizedAcquired localized

TreatmentTreatment Avoid traumatization Avoid traumatization

to the hairto the hair

TrichorrhexisTrichorrhexisnodosanodosa

Trichorrhexis invaginataTrichorrhexis invaginata(bamboo hair)(bamboo hair)

Caused by intussusception of the hair shaft at Caused by intussusception of the hair shaft at the zone where keratinization beginsthe zone where keratinization begins

The invagination is caused by the softness of The invagination is caused by the softness of the cortex in the keratogenous zonethe cortex in the keratogenous zone

patient has nodosa ball and socket patient has nodosa ball and socket deformitiesdeformities

Associated with Netherton’s syndrome with Associated with Netherton’s syndrome with ichthyosiform erythroderma or ichthyosis ichthyosiform erythroderma or ichthyosis linearis circumflexalinearis circumflexa

Trichorrhexis invaginataTrichorrhexis invaginata



Occ only the proximal half of the abnormality Occ only the proximal half of the abnormality is seen – is seen – golf tee hairsgolf tee hairs

Seen on scalpSeen on scalp Also eyebrows, eyelashes, and rarely in other Also eyebrows, eyelashes, and rarely in other

hairy areashairy areas Hair sparsity is noted all over the bodyHair sparsity is noted all over the body Bamboo hairs may become normal in a few Bamboo hairs may become normal in a few

yearsyears Autosomal recessive mode of inheritanceAutosomal recessive mode of inheritance

Netherton’s syndromeNetherton’s syndrome

Netherton’sNetherton’ssyndromesyndrome

Pili annulatiPili annulati(Ringed Hair)(Ringed Hair)

The hair seems banded by alternating The hair seems banded by alternating segments of light and dark color when seen in segments of light and dark color when seen in reflected lightreflected light

Light bands are caused by clusters of Light bands are caused by clusters of abnormal air-filled cavities which scatter lightabnormal air-filled cavities which scatter light

Hair growth is normalHair growth is normal No other assoc abnormalitiesNo other assoc abnormalities Autosomal dominantAutosomal dominant Begins in infancy and requires no treatmentBegins in infancy and requires no treatment

Pili annulatiPili annulati(Ringed Hair)(Ringed Hair)

Pili pseudoannulatiPili pseudoannulati

Mimics pili annulatiMimics pili annulati Bright segments are caused by reflection Bright segments are caused by reflection

and refraction of light by flattened, twisted and refraction of light by flattened, twisted surfaces of hairsurfaces of hair

Variant of normal hairVariant of normal hair

Kinked hairKinked hair

Acquired progressive kinking of the hairAcquired progressive kinking of the hair Has a structural abnormality of kinking Has a structural abnormality of kinking

and twisting of the hair shaft at irregular and twisting of the hair shaft at irregular intervalsintervals

Begins in men in the late teens and Begins in men in the late teens and early twentiesearly twenties

Begins on the frontotemporal or vertex Begins on the frontotemporal or vertex regions and progresses to both the regions and progresses to both the parietal and frontal areasparietal and frontal areas

Kinked hairKinked hair

Usually straight, light brown hair becomes Usually straight, light brown hair becomes curly, frizzy, and lusterlesscurly, frizzy, and lusterless

May be precursor to male-pattern hair lossMay be precursor to male-pattern hair loss May be induced by drugs, notably May be induced by drugs, notably

retinoidsretinoids May occur in AIDS patientsMay occur in AIDS patients

Woolly hairWoolly hair

Present at birth and usually most severe Present at birth and usually most severe during childhoodduring childhood

Variable amelioration in adulthoodVariable amelioration in adulthood Four subgroupsFour subgroups

Hereditary woolly hair (AD)Hereditary woolly hair (AD) Familial woolly hair (AR)Familial woolly hair (AR) Woolly hair nevusWoolly hair nevus Naxos diseaseNaxos disease


Tend to unite into Tend to unite into lockslocks

No assoc cutaneous No assoc cutaneous or systemic diseasesor systemic diseases


Woolly hairWoolly hairNOTNOT

Plica neuropathicaPlica neuropathica

Curling, looping, intertwisting, and felting Curling, looping, intertwisting, and felting or matting of the hair in localized areas of or matting of the hair in localized areas of the scalpthe scalp

Predisposing factors include kinky hairs Predisposing factors include kinky hairs and neurotic mental stateand neurotic mental state

Pseudofolliculitis barbaePseudofolliculitis barbae

Hairs that appear at the surface and curve Hairs that appear at the surface and curve back and pierce the skin as ingrowing hairsback and pierce the skin as ingrowing hairs

Results in inflammatory papules and pustulesResults in inflammatory papules and pustules May scarMay scar Chief cause is close shaving of curly hairChief cause is close shaving of curly hair Seen in more than 50% of black menSeen in more than 50% of black men Whites uncommonly affectedWhites uncommonly affected



TreatmentTreatment Laser hair removal Laser hair removal stop shavingstop shaving Deforming keloids may resultDeforming keloids may result

Pili multigeminiPili multigemini

Rare malformation of the pilary apparatusRare malformation of the pilary apparatus Characterized by the presence of Characterized by the presence of

bifurcated or multiple divided hair matrices bifurcated or multiple divided hair matrices and papillae, giving rise to the formation of and papillae, giving rise to the formation of multiple hair shafts within the individual multiple hair shafts within the individual folliclesfollicles

No treatmentNo treatment

Pili bifurcatiPili bifurcati

Described in a 3 year old seen for hair lossDescribed in a 3 year old seen for hair loss Bifurcation was found in short segments Bifurcation was found in short segments

along the shafts of several hairsalong the shafts of several hairs Anomaly was transientAnomaly was transient

Trichostasis spinulosaTrichostasis spinulosa

A common disorder of the hair follicles that A common disorder of the hair follicles that clinically gives the impression of follicular clinically gives the impression of follicular keratosiskeratosis

Follicles are filled with funnel-shaped horny Follicles are filled with funnel-shaped horny plugs within which are bundles of vellus hairsplugs within which are bundles of vellus hairs

Hairs are round at their proximal ends and Hairs are round at their proximal ends and are shredded distallyare shredded distally

Occurs on the nose and forehead of elderlyOccurs on the nose and forehead of elderly Shoulders and backShoulders and back


Results from retention of telogen hairsResults from retention of telogen hairs Treatment with keratolytics after using a Treatment with keratolytics after using a

wax depilatorywax depilatory Tretinoin 0.05% solutionTretinoin 0.05% solution



Trichostasis spinulosaTrichostasis spinulosathe removed plugthe removed plug

Microscopically the Microscopically the tufts are made up of tufts are made up of several dozen fine, several dozen fine, stubbly hairs stubbly hairs

Intermittent Hair-Follicle Intermittent Hair-Follicle DystrophyDystrophy

New disorder of the hair follicle leading to New disorder of the hair follicle leading to increased fragility of the shaft, with no increased fragility of the shaft, with no identifiable biochemical disturbanceidentifiable biochemical disturbance

Bubble Hair DeformityBubble Hair Deformity

Reported in a 16 year old girlReported in a 16 year old girl Developed brittle, fragile hairs in localized Developed brittle, fragile hairs in localized

areas of he scalpareas of he scalp Hairs became straight and stiffHairs became straight and stiff Small bubble-like defects were found within Small bubble-like defects were found within

the hairs shafts on light and electron the hairs shafts on light and electron microscopymicroscopy

Proved to be caused by heating of the hairProved to be caused by heating of the hair All hair will develop bubbles of gas when All hair will develop bubbles of gas when

exposed to heatexposed to heat

HypertrichosisHypertrichosis

An overgrowth of hair not localized to the An overgrowth of hair not localized to the androgen-dependent areas of the skin.androgen-dependent areas of the skin.

Several forms existSeveral forms exist

Localized acquired Localized acquired hypertrichosishypertrichosis

Dermal tumors, such as melanocytic nevi, Dermal tumors, such as melanocytic nevi, smooth muscle hamartoma, meningioma smooth muscle hamartoma, meningioma or Becker’s nevi may have excessive or Becker’s nevi may have excessive terminal hair growthterminal hair growth

PCT and Variegate porphyriaPCT and Variegate porphyria May be more generalized in the Gunther May be more generalized in the Gunther

variety of erythropoietic porphyriavariety of erythropoietic porphyria

Becker’sBecker’snevusnevus

Becker’s nevusBecker’s nevus

PCTPCT

Localized congenital Localized congenital hypertrichosishypertrichosis

Progressive, excessive growth of Progressive, excessive growth of lanugo hairs that often begins in infancylanugo hairs that often begins in infancy

Hairs may reach a length of 10 cmHairs may reach a length of 10 cm May become coarseMay become coarse Regression in adolescence has been Regression in adolescence has been

observedobserved Familial cases and sporadicFamilial cases and sporadic Appears to be only of cosmetic Appears to be only of cosmetic

significancesignificance

Localized congenital Localized congenital hypertrichosishypertrichosis

Other causes include congenital nevocytic Other causes include congenital nevocytic nevi, anterior cervical hypertrichosis, nevi, anterior cervical hypertrichosis, simple nevoid hypertrichosis, or as a sign simple nevoid hypertrichosis, or as a sign of underlying spinal dysraphism (when of underlying spinal dysraphism (when occurring over the sacral midlineoccurring over the sacral midline

GeneralizedGeneralized Congenital HypertrichosisCongenital Hypertrichosis(congenital hypertrichosis lanuginosa)(congenital hypertrichosis lanuginosa)

Rare type of excessive and generalized Rare type of excessive and generalized hairinesshairiness

Fully penetrant X-linked dominant traitFully penetrant X-linked dominant trait Entire body covered with fine vellus hairs 2-10 Entire body covered with fine vellus hairs 2-10

cm long, spares palms and solescm long, spares palms and soles Scalp hair is normalScalp hair is normal May be assoc with dental anomalies and May be assoc with dental anomalies and

gingival fibromatosisgingival fibromatosis

““dog-faced boy” “human werewolf”dog-faced boy” “human werewolf”“human Skye terrier”“human Skye terrier”

Generalized Congenital HypertrichosisGeneralized Congenital Hypertrichosis(congenital hypertrichosis lanuginosa)(congenital hypertrichosis lanuginosa)

Other cases may be secondary to drug Other cases may be secondary to drug ingestion by the motheringestion by the mother

Fetal hydantoin syndromeFetal hydantoin syndrome Fetal alcohol syndromeFetal alcohol syndrome Minoxidil – case of generalized Minoxidil – case of generalized

hypertrichosis and multiple congenital hypertrichosis and multiple congenital defects in a baby born to a mother who defects in a baby born to a mother who used minoxidil throughout pregnancyused minoxidil throughout pregnancy

Generalized or patterned Generalized or patterned acquired hypertrichosisacquired hypertrichosis

These cases include those caused by These cases include those caused by acquired hypertrichosis lanuginosa, those acquired hypertrichosis lanuginosa, those associated with various syndromes, and associated with various syndromes, and those secondary to drug intakethose secondary to drug intake

Also an ominous sign of internal Also an ominous sign of internal malignancymalignancy

SyndromesSyndromes

Lipoatrophic diabetes, stiff skin syndrome, Lipoatrophic diabetes, stiff skin syndrome, Rubenstein-Taybi syndrome, Laband Rubenstein-Taybi syndrome, Laband syndrome, Cornelia de Lange’s syndrome, syndrome, Cornelia de Lange’s syndrome, Hurler’s syndrome, Morogu’s syndrome, Hurler’s syndrome, Morogu’s syndrome, leprechaunism, , Winchester’s syndrome, leprechaunism, , Winchester’s syndrome, the Schynzel-Giedier syndromethe Schynzel-Giedier syndrome

Brachmann-Brachmann-de Lange syndromede Lange syndrome

Hunter syndromeHunter syndrome

drugsdrugs

Minoxidil, cyclosporine, diphenylhydantoin, Minoxidil, cyclosporine, diphenylhydantoin, diazoxide, streptomycin, penicillamine, diazoxide, streptomycin, penicillamine, corticosteroids, danazol, psoralens, corticosteroids, danazol, psoralens, hexachlorobenzene, PUVA, topical hexachlorobenzene, PUVA, topical steroids and topical androgenssteroids and topical androgens

cyclosporinecyclosporine

minoxidilminoxidil

HirsutismHirsutism

An excess of terminal hair growth in women An excess of terminal hair growth in women in a pattern more typical of menin a pattern more typical of men

Androgen-dependent growth areas affected Androgen-dependent growth areas affected include the upper lip, cheeks, chin, central include the upper lip, cheeks, chin, central chest, breasts, lower abdomen and groinchest, breasts, lower abdomen and groin

May or may not be assoc with other signs of May or may not be assoc with other signs of virilizationvirilization

Acne may be seenAcne may be seen When virilization accompanies hirsutism, When virilization accompanies hirsutism,

especially when progression is rapid, a especially when progression is rapid, a neoplastic cause is likelyneoplastic cause is likely

HirsutismHirsutismpathogenesispathogenesis

Racial variationRacial variation May result either from excessive of May result either from excessive of

androgens from either the ovary or the androgens from either the ovary or the adrenal gland, or from excessive adrenal gland, or from excessive stimulation by pituitary tumorsstimulation by pituitary tumors

All cases of progressive or severe All cases of progressive or severe hirsutism should be investigated for an hirsutism should be investigated for an endocrinopathyendocrinopathy


Ovarian causes include polycystic ovarian Ovarian causes include polycystic ovarian disease and a variety of ovarian tumors, disease and a variety of ovarian tumors, both benign and malignantboth benign and malignant

Stein-Leventhal syndrome characterized y Stein-Leventhal syndrome characterized y hirsutism 50%hirsutism 50%

Serum free testosterone is generally Serum free testosterone is generally elevated as is luteinizing hormoneelevated as is luteinizing hormone


Adrenal causes include congenital adrenal Adrenal causes include congenital adrenal hyperplasia and adrenal tumors such as hyperplasia and adrenal tumors such as adrenal adenomas and carcinomasadrenal adenomas and carcinomas

Adrenogenital syndrome is an autosomal Adrenogenital syndrome is an autosomal dominant disorder resulting from deficiencies dominant disorder resulting from deficiencies in the following enzymes:in the following enzymes: 21-hydroxylase21-hydroxylase 11B-hydroxylase11B-hydroxylase 3B-hydroxy steroid dehydrogenase3B-hydroxy steroid dehydrogenase


Pituitary causes include Cushing’s Pituitary causes include Cushing’s disease, acromegaly, and prolactin-disease, acromegaly, and prolactin-secreting adenomassecreting adenomas

Other conditions in which prolactin levels Other conditions in which prolactin levels may be elevated and that may lead to may be elevated and that may lead to hirsutism include hypothyroidism, hirsutism include hypothyroidism, phenothiazine intake, and hepatorenal phenothiazine intake, and hepatorenal failurefailure


Other causes include the exogenous Other causes include the exogenous intake of androgens and certain high-intake of androgens and certain high-progesterone bcp (uncommonly)progesterone bcp (uncommonly)

Minoxidil, diazoxide, corticosteroids and Minoxidil, diazoxide, corticosteroids and phenytoin, which have been reported to phenytoin, which have been reported to cause hirsutism, generally cause cause hirsutism, generally cause hypertrichosishypertrichosis

HirsutismHirsutismevaluationevaluation

History should focus on onset and History should focus on onset and progression, virilization, menstrual progression, virilization, menstrual history, and family/racial backgroundhistory, and family/racial background

Laboratory evaluation should include a Laboratory evaluation should include a total testosterone and a total testosterone and a dehydroepiandrosterone sulfate level for dehydroepiandrosterone sulfate level for relatively stable and mild hirsutismrelatively stable and mild hirsutism

Dexamethasone suppression test to Dexamethasone suppression test to screen for Cushing’s diseasescreen for Cushing’s disease

HirsutismHirsutismevaluationevaluation

In patients with menstrual dysfunction add In patients with menstrual dysfunction add prolactin level and an LH/FSH ratio to prolactin level and an LH/FSH ratio to evaluate suspected polycystic ovarian evaluate suspected polycystic ovarian diseasedisease

diagnosisdiagnosis

Refer to endocrinologistRefer to endocrinologist Refer to gynecologistRefer to gynecologist Major elevation in the DHEA sulfate level Major elevation in the DHEA sulfate level

(above 7000 ng/ml) suggests an adrenal (above 7000 ng/ml) suggests an adrenal neoplasm CT is recommendedneoplasm CT is recommended

treatmenttreatment

Once appropriate testing has led to diagnosis Once appropriate testing has led to diagnosis and referral of patients requiring special and referral of patients requiring special methods of specific treatment, such as methods of specific treatment, such as surgical intervention, therapeutic alternatives surgical intervention, therapeutic alternatives including cosmetic treatments, nonspecific including cosmetic treatments, nonspecific suppressive therapy, and specific suppressive therapy, and specific antiandrogensantiandrogens

Shaving, wax depilatories, chemical Shaving, wax depilatories, chemical depilatories, bleaching of the hair, laser hair depilatories, bleaching of the hair, laser hair removal, and electrolysisremoval, and electrolysis

treatmenttreatment

Oral contraceptives and glucocorticoidsOral contraceptives and glucocorticoids bcp helpful in 75% of hirsute womenbcp helpful in 75% of hirsute women Antiandrogens include, cimetidine, Antiandrogens include, cimetidine,

cyproterone acetate, spironolactone, cyproterone acetate, spironolactone, flutamide, and ketoconazoleflutamide, and ketoconazole

FinasterideFinasteride Gonadotropin-releasing hormone agonist Gonadotropin-releasing hormone agonist

such as leuprolide and nafarelinsuch as leuprolide and nafarelin

Trichomycosis AxillarisTrichomycosis Axillaris

Characterized by 1-2 mm nodules of different Characterized by 1-2 mm nodules of different colors occurring on the affected hair shafts in the colors occurring on the affected hair shafts in the axillary or pubic areasaxillary or pubic areas

Attached firmly to the hair shaftAttached firmly to the hair shaft Color may vary from yellow, red or blackColor may vary from yellow, red or black Hyperhidrosis is usually presentHyperhidrosis is usually present Large number of corynebacterium are present in Large number of corynebacterium are present in

the concretionsthe concretions


Topical antibiotic preparationsTopical antibiotic preparations Treat hyperhidrosisTreat hyperhidrosis shavingshaving


Documents

Diseases of the Skin Appendages. Alopecia areata Characterized by rapid and complete loss of hair in one, or more often several round or oval patches