Answers for Self-Assessmentexamination of the AmericanAcademy of Dermatology

Identification No. 880-208

August. 1980. issue of the JOURNAL OF TIlE A~tERICAN

ACADEMY OF DERMATOLOGY

QUESTIONS 1-4

Of the possible clinical diagnoses listed, syphi­lis is the best. Its appearance is incompatible witheither condyloma latum or condyloma acumina­tum. The appearance would also certainly be atyp­ical for the other two diseases. Dark-fieldcon finn ation is necessary, however, because ofother possible ulcerative diseases of the glanspenis, e.g., herpes simplex, chancroid. Serologictests for syphilis are often still negative in the pri­mary stage. Only if the dark-field examinationwere negative would any of the other tests be ap­propriate. The treatment of choice for primarysyphilis is penicillin G benzathine, 2.4 million

.U 1M one dose only or penicillin G procaine,600.000 U 1M daily for 8 days. If the patient isallergic to penicillin, alternate therapy should betetracycline HCI, 500 mg q .i.d. for 15 days orerythromycin, 5QO mg q.i.d. for 15 days.

For this series. the recommended choices are: 1,c; 2, c; 3. c; 4. b, d.

REFERENCES

I . Nicholas L: Treponemalinfections.iIIMoschellaSL.etal, editors : Dermatology. Philadelphia. 1975, W. B.Saunders Co., pp. 710-711.

2. Treatment of syphilis and gonorrhea. Med Lett DrugsTher 19:105, 1977.

QUESTIONS 5-8

The history and physical findings in this caseare characteristic of eczema hiemalis, althoughwith a background of dry adherent rhomboidalscale, reflecting xerosis, one should consider theichthyoses . However, these would be unlikely tomanifest first in adulthood, and a positive .familyhistory would be Iikcly . The absence of Iichcn-

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ification would essentially exclude atopic eczema,and the absence of hypcrpigrnentation in a lo­calized vascular pattern would rule out erythemaab igne. A generalized allergic contact dermatitiswould be expected to occur in a diffuse rather thanreticulated pattern.

Several environmental factors in this case con­tribute to the skin disease. Hot water in some wayrenders the stratum corneum less able to retain itsusual water content and maintain its pliability.Antibacterial soaps seem to contribute to this also.The low relative humidity of winter weather, par­ticularly with the air dried out by heating, en­hances evaporation from the stratum corneum.Individuals who work outside. especially if theyare inadequately clothed. lower the dew point ofthe cutaneous surface by cooling. At a dew pointbelow 150 C (590 F). the stratum corneum starts tobecome brittle, and at lower levels it cracks. Thereis no evidence that laundry detergents or diet ordi­narily have any effect on this process.

Patients should be encouraged to minimizebathing and soap usage, lower the bath water tem­perature, and protect the skin from cold air. In­stallation of an air humidifier may be advisable.There is considerable disagreement about the op­timum topical therapy in this condition. Some ad­vise greasy ointments applied to still-wet skin.while others feel these are poorly tolerated andprefer less occlusive emollients . To a certain ex­tent, the success of topical therapy will depend onthe patient's acceptance and willingness to use themedication. The treatments listed in question 8 ,except for the emollient cream, would be inap­propriate or suboptimal because of their inabilityto promote rehydration of the stratum corneum.

Volume 3Number 2August, 1980

For this series, the recommended choices are: 5,a, c, d; 6, a; 7, a, c, e; 8, d.

REFERENCESI. Baughman RD, Porter PS: Eczema hiemalis, ill Demis OJ,

Dobson RL, McGuire J, editors: Clinical dermatology.New York, 1979, Harper & Row, Publishers, Inc., unit13-4, pp. 1-2.

2. Daniels F Jr: Physiologic factors in the skin's reactions toheat and cold, ill Fitzpatrick TB, et al, editors: Dermatol­ogy in general medicine. New York, 1979, McGraw-HiliBook Co., p. 925.

QUESTIONS 9-11

The diagnosis is most likely drug eruption fromiodine. It is unlikely that folliculitis or der­matophytosis would produce concurrent hives andpustules. The other choices are very remote. Inestablishing a diagnosis (which in this case wasdue to the patient's taking Kelp tablets along withvitamins), the PBI would be most helpful. If in­fection were considered, culture of an intact pus­tule could be obtained. No growth would be ex­pected in a pustule of iododerma, The other testswould be of little or no value. For treatment of theiododerma, salt tablets would enhance renal tubu­lar excretion of iodides. The other drugs would beinappropriate.

For this series, the recommended choices are: 9,a, b; 10, a; 11, a.

REFERENCEI. Domonkos AN: Andrew's Diseases of the skin, ed. 6.

Philadelphia, 1971, W. B. Saunders Co., p. 114.

QUESTIONS 12-15

The correct diagnosis is chronic radioder­matitis. Because of the distribution described, theradiation was probably administered for hyper­trichosis. Years ago unlicensed practitionerswould travel from town to town with poorly me­tered radiation equipment and offer to remove un­wanted facial hair. The treatment was effective,but today we see the sequelae. This patient'schanges are mild and observation is all that is nec­essary at this time. Ulceration, actinic keratoses,and both squamous cell and basal cell carcinomasmight arise, but treatment does not appear to beindicated. The patient' should be protected fromsunlight.

Self-Assessment examination answers 43A

For this series, the recommended choices are:12, d; 13, c; 14, c; 15, b.

REFERENCEI. Rudolph RI, Goldschmidt H: Radiodermatitis and other

adverse sequelae of cutaneous irradiation, ill GoldschmidtH, editor: Physical modalities in derrnatologic therapy.Berlin, 1978, Springer- Verlag, pp. 49-64.

QUESTIONS 16-18

The most likely diagnosis is Waldenstromshypergammaglobulinemia (purpura hyperglobu­linemica). This usually affects middle-aged womenand is exacerbated by prolonged standing or walk­ing. The sedimentation rate is usually elevated;polyclonal gammopathy and leukocytoclastic vas­culitis may occur. Immunoelectrophoresis wouldbetter define the immunoglobulin abnormality.Rheumatoid factor is often present at a high titer. Adrug hypersensitivity can produce palpable purpurabut is usually not associated with the protein ab­normalities present in this patient.

Hypergammaglobulinemia may be secondary toa number of conditions including multiple my­eloma, systemic lupus erythematosus, chroniclymphocytic leukemia, lymphomas, Sjogren'ssyndrome, etc. Neither multiple myeloma normacroglobulinemia is likely in this case, sincethere is a polyclonal rather than a monoclonalgamrnopathy. A biopsy of the oral labial mucosais indicated since this is an extremely helpful pro­cedure in the diagnosis of Sjogren's syndrome.Cold agglutinins do not ordinarily have sig­nificance relative to purpura.

In many cases there is no apparent cause for thehyperglobulinemia (idiopathic or primary). Thistype usually appears before the age of 40.

For this series, the recommended choices are:16, a, d; 17, b, c, e; 18, e.

REFERENCEI. Waldenstrom J: Dysproteinemias, ill Fitzpatrick TB, et al,

editors: Dermatology in general medicine, ed. 2. NewYork, 1979, McGraw-Hili Book Co., pp. 1057-1058.

QUESTIONS 19-21

This is one of the extrahepatic syndromes result­ing from hepatitis B virus (HB V) infection and ispresented to emphasize this type of host response

44A Self-Assessment examination answers

to infection with HB V. In addition to cryo­globulinemia, the HB V-host encounter may pro­duce infantile papular acrodermatitis, polyarthritis,polyrnyalgia, and nephritis. In these disorders,overt liver disease is often not suspected or not aprominent feature of the disease. Now thatserologic tests for detection of HB V infection are ingeneral usc, additional extrahepatic syndromes willprobably be defined.

In the first group of questions "vasculitis" isthe best choice based upon palpable purpura of thelower extremities, although a drug eruption shouldbe considered. Mixed cryoglobulinemia is the se­lection in the second group. Rheumatoid factor isfrequently found in cryoglobulinemia with jointsymptoms . Also, the information provided doesnot fulfill the criteria to diagnose rheumatoid ar­thritis . A drug reaction cannot be diagnosed on thedata given .

HB V I infection is the best choice in the thirdgroup and will account for the signs, symptoms ,and laboratory results in this patient. The presenceof anti -Hll, alone in the serum is consistent withactive hepatitis and, unlike finding anti-Hll, alone,does not suggest the absence of infectivity.

For this series, the recommended choices are:19, c; 20, c; 21, c.

REFERENCES

I . Czaja Albert J: Serologic markers of hepatitis A and 8 inacute and chrohic liver disease. Mayo Clin Proc 54:721­732, 1979.

2. Levo Y, Gorevic P, Kassab H, et al: Association betweenhep atiti s 8 virus and essential mixed cryoglobulinemia. NEngl J Med 296:1501-1504, 1977.

QUESTIONS 22-25

The patient has lymphomatoid papulosis whichis characterized by a continuing, self-healing, clin­ically benign and histologically malignant papularand papulonecrotic eruption. Some investigatorshave considered lymphomatoid papulosis to be aclinical variant of Mucha-Habermann disease.Histologically, it has been considered to representa Iymphoreticular malignancy or cutaneous Hodg­kin's disease. The life cycle of individual lesionsis about 6 weeks. The eruption persists in­definitely, with lesions waxing and waning forperiods of 5 to 20 years. The histopathology of

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Mucha-Habermann disease differs in that theinfiltrate is less pleomorphic and is composedmostly of small round cells. Also, large hyper­chromatic cells arc less frequently seen in Mucha­Habermann disease. Mucha-Habermann disease isnot a precursor of parapsoriasis en plaque and ismore common in younger male patients . Theetiology is unknown . Lymphomatoid papulosisoccurs more commonly after the age of 25, andwomen outnumber men 2: I. The cells of lym­phomatoid papulosis have been shown to be 100%T cells. Cases have been reported which haveprogressed to malignancy. Macaulay suggestedthe term "rhythmic paradoxical eruptions" for thegroup of diseases that have a clinically benigncourse and the histology of a malignant lym­phoma.

For this series, the recommended choices are:22, a , b, d, e; 23 , d; 24, b, c; 25, a, c, d.

REFERENCES

I . Mac aulay WL : Lymphomatoid papulosis: A continuing,self-healing eruption, clinically benign-histologicallymalignant. Arch Dermatol 97:23-30, 1968.

2. Macaulay WL: Lymphomatoid papulo sis, Int J Dermatol17:204-212, 1978.

3. Mchregan AM: Pseudomalignancies of the skin. Cut is10:305-309, 1972.

4 . Pinku s H: Proliferation of "pseudo" dermatoses: Progressor regre ssion? Int J Derm atol 15:326-336, 1976.

QUESTIONS 26-29

Although one could proceed immediately withcosmetic removal of the xanthelasmata, additionalinformation would be pertinent since it mightinfluence treatment decisions. Fifty percent of pa­tients with xanthelasma may have elevated lipids,but the condition may even be seen in nor­molipidemic xanthomatoses such as xanthomadisseminatum and planar xanthoma. Normolipi­demic xanthelasmata are especially common afterthe age of 40.

It would seem most logical to suggest thescreening tests, serum cholesterol and serum tri­glycerides . A 2-hour postprandial blood sugar testshould also be done to rule out possible coexistentdiabetes, and the patient should receive a completephysical examination to look for associated car­diovascular disease. This type of plane xanthomais not associated with leukemia or multiple my-

Volume 3Number 2August, 1980

eloma. Most patients with hyperlipidemic xan­thelasmata also have tendinous xanthomata.

A number of patients with xanthelasma mayhave received local treatment for their conditionprior to consulting you. It is helpful to find out thetreatment previously rendered and the results ofthis therapy.

Local treatment will be based upon the locationand extent of the lesions. Small to large lesionslocated on redundant eyelid skin will yield to 50%to full strength trichloroacetic acid lightly andcarefully done at intervals of 6 weeks.

In regard to the method of application, the phy­sician should spin a small amount of cotton on atoothpick or other pointed wooden applicator. Thecotton-tipped applicator, after being dipped in thetrichloroacetic acid, is gently pressed against theinside of the neck of the bottle to remove excessacid. To further guard against possible "laking"on the patient's eyelid, the physician should touchthe applicator to his fingernail. A moistened gauzesponge is kept within reach to dilute and wash acidaway if need be.

In extensive lesions involving the lower eyelids,

Self-Assessment examination answers 45A

the sides of the nose adjacent to the eye and le­sions near the canthus, care must be taken becauseof possible hypertrophic scarring and ectropion.When in doubt, treat small test areas and awaitresults prior to treating large areas.

No matter which method of therapy is elected, itis helpful to discuss the possibility and reasons forrecurrence of lesions. Recurrences are seen in40% or more of cases, and are especially likelyin the presence of familial hyperlipidemia, in­volvement of all four eyelids, and history of pre­vious recurrences.

For this series, the recommended choices are:26, e; 27, a, b, e; 28, a, b; 29, b, c, d.

REFERENCES

I. Mendelson BC, Masson JK: Xanthelasma: Follow-up onresults after surgical excision. J Plast Reconstr Surg58:535-538, 1976.

2. Moschella SL: Diseases of nutrition and metabolism, illMoschella SL, et al, editors: Dermatology. Philadelphia,1975, \Y. B. Saunders Co., p. 1277.

3. Parker F: Xanthomas, ill Demis OJ, Dobson RL, McGuireJ, editors: Clinical dermatology. New York, 1979, Harper& Row, Publishers, Inc., unit 12-1, pp. 11, 18-19,30.