P6480Cardiac arrest and dilated cardiomyopathy in a patient with epidermolysisbullosa simplex and hidradenitis suppurativa

Catherine Foley, MBBCh, St. James’s Hospital, Dublin, Ireland; Benvon Moran,MBBCh, St. James’s Hospital, Dublin, Ireland; Katherine Sweeney, St. James’sHospital, Dublin, Ireland; Rosemarie Watson, MD, St. James’s Hospital, Dublin,Ireland

A 20-year-old man with epidermolysis bullosa simplex (EBS) and hidradenitissuppurativa (HS) presented to the emergency department with a presyncopalepisode. He had not had any skin blistering for 18 months. His HS was active, withinflammatory nodules in the axillae and groin, and he was taking minocycline 100mg daily. In the preceding year, he reported 2 syncopal episodes, which wereinvestigated with electrocardiograms and no cause was identified. While in theemergency department he suffered a ventricular fibrillation cardiac arrest precededby a run of ventricular tachycardia. Return of circulation was achieved after 9minutes of cardiopulmonary resuscitation and 2 shocks with the defibrillator. Anechocardiogram showed an ejection fraction of 15% to 20% with globally reducedleft ventricular function and normal valvular function. He was intubated andtransferred to the intensive care unit for 18 days and an implantable cardioverter-defibrillator was inserted. His HS improved during admission. Genetic testing for aplectin mutation was negative and a serum selenium level was within normal limits.Cardiac magnetic resonance imaging 3 months later showed a dilated left ventriclewith globally impaired function and an ejection fraction of 42%. Previous testing forkeratin 5 and keratin 14 mutations was negative; his underlying EB mutation has notbeen identified. Dilated cardiomyopathy has been reported in severe EB subtypes. Amulticentre retrospective descriptive study reported 15 patients with EB and dilatedcardiomyopathy. Of these, 73% (11) were male, 87% (13) had recessive dystrophicEB, and 13% (2) had junctional EB. Cardiomyopathy in 2 patients with EBS haspreviously been reported in association with amutation in the plectin gene (PLEC1).Our patient does not have this mutation. There is 1 case report of cardiomyopathy inHS, which responded to radical debridement of the involved areas. Continuedimprovement in his HS has mirrored the improvement in ejection fraction and hehas not had any further arrhythmias recorded on the ICD. His HS is being treatedaggressively with antibiotics and surgery is being considered. He has now beentaken off the cardiac transplantation waiting list. We wish to present this case as anexample of life-threatening cardiomyopathy occurring in a patient with EB simplex,possibly exacerbated by hidradenitis suppurativa.

APRIL 20

cial support: None identified.

Commer

P6847Ashy dermatosis

Diana Marcal Marques Gouvea, BWS, S~ao Paulo, Brazil; Carolina Arruda Borini,BWS, S~ao Paulo, Brazil; Juliana Mauro Caramel, BWS, S~ao Paulo, Brazil; JulianaTeixeira De Carvalho, BWS, S~ao Paulo, Brazil; Rafael Pessanha De Paula, BWS, S~aoPaulo, Brazil; Rafael Soares, BWS, S~ao Paulo, Brazil

Background: Erytema dyscromicum perstans (EDP) is a relatively rare skin disease,included in the group of idiopathic acquired hypermelanosis. It is a chronic benigndisease of unknown etiology. The disease is common in dark-skinned people,especially women in their first or second decade of life.

Case Report: Patient L.M.O., white, female, 50 years, a native of Piau�ı and raised inS~ao Paulo, came to the dermatology service at BWS with complaints of blight on theface and neck for about 2 years. On dermatologic examination was observed agrayish macula, with areas of healthy skin in between, affecting the face, ear andneck, and periorbital edema, and pinkish in Wood’s lamp. Griseofulvin 500 mg,12/12 hours introduced for 3 months as initial treatment, after which time thepatient was reevaluated with little improvement of the lesions. Erytema dyscromi-cum perstans is a skin disease characterized by hyperpigmented macules with avariety of sizes in the face, trunk, and extremities. A number of studies onimmunopathologic active lesions have shown that immune-mediated EDP mayinvolve immunologic phenomena. This chronic dermatitis is of insidious nature andasymptomatic. The histopathology of EDP is not specific. Histologic examination ofthe active phase shows lichenoid dermatitis with vacuolization of the basal layer,occasionally colloid bodies, and increased epidermal melanin. The changes arereported by dermal edema of the papillary dermis, a mild or moderate patchylymphohistiocytic infiltrate and dermal melanophages. Currently, there is noestablished therapy. Nevertheless, the use of topical corticosteroids, keratolyticagents, chloroquine, sunscreen, diaminodiphenylsulfone, griseofulvin, and clofaz-imine have been effective, probably because of the results of antinflammatoryeffects. Griseofulvin has been reported to induce the complete resolution of thedisease, despite the tendency of the lesions return after discontinuation oftreatment. Some authors have suggested the therapeutic efficacy of clofazimineand dapsone on EDP, but in Brazil only clofazimine is dispensed through compulsorynotification of leprosy cases. Dapsone, besides its antimicrobial potency, is effectivefor dermatosis rich in polymorphonuclear cells, as well as dermatosis rich inlymphocytes, and possibly participates in the regulation of immune reactionsinvolved in the pathogenesis of EDP.

cial support: None identified.

Commer

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P6468Circumscribed palmar hypokeratosis: One case and review

Rafael Rojo Espana, Hospital Morales Meseg€uer, Murcia, Spain; Beatriz PerezSuarez, Hospital Morales Meseg€uer, Murcia, Spain; Eduardo Alcaraz Mateos,Hospital Morales Meseg€uer, Murcia, Spain; Pedro Mercader Garcia, HospitalMorales Meseg€uer, Murcia, Spain

Case report: We report a 64-year-old woman presented for evaluation of anonhealing lesion on the right palm. It had slowly enlarged over 2 years and wasasymptomatic. It was an erythematous and atrophic circular patch measuring 1 cm.

Methods: Skin biopsy.

Results: The skin biopsy showed orthohyperkeratosis with abrupt transition to areaof hypokeratosis.

Conclusions: Circumscribed palmar hypokeratosis (CPH) is a rare skin disorder thattypically develops on the palms of middleaged or elderly women. It was firstreported in 2002 by Perez et al, who described the largest case series involving 10patients with a characteristic epidermal malformation of the palms and soles. Theyconsidered circumscribed palmar hypokeratosis to be a benign clonal epidermalmalformation, but several hypothetical pathogeneses have been proposed, such as aprimary keratinizing disorder of the granular and horny layers, trauma, or humanpapilloma virus type 4 infection. The most common clinical differential diagnosis atpresentation was Bowen disease, actinic keratosis, or palmoplantar porokeratosis.The diagnosis of CPH is based on its clinical manifestation and anatomic site. Thereare currently no well-established treatments; various single and combination topicaltherapies have been used, including corticosteroids, retinoids, and calcipotriol,mostly without improvement. Other studies have shown partial remission withphotodynamic therapy and total resolution with cryotherapy. It had not beenreported malign degeneration, therefore the treatment is not necessary. We report anew case of circumscribed palmar hypokeratosis and literature review.

cial support: None identified.

Commer

P6754Clinical exuberance of cutaneous sarcoidosis: Case report

Maria Vict�oria Quaresma, MD, IDPRDA - Instituto de Dermatologia ProfessorRubem David Azulay, Rio de Janeiro, Brazil; Fred Bernardes, MD, IDPRDA -Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeiro, Brazil;Gabriel Monteiro de Castro, IDPRDA - Instituto de Dermatologia ProfessorRubem David Azulay, Rio de Janeiro, Brazil; Jo~ao Carlos Regazzi, MD, IDPRDA -Instituto de Dermatologia Professor Rubem David Azulay, rio de Janeiro, Brazil

Background: Sarcoidosis is a multisystem granulomatous disease of unknownetiology, which may have exclusively cutaneous or affect several organs, causing awide spectrum of clinical manifestations. Cutaneous sarcoidosis is known as a greatmimic of other diseases, because of its polymorphism, representing an importantdiagnostic challenge. The skin is affected in about 20% to 35% of cases, thedermatologist plays an important role in the diagnosis, which is based on clinical andhistologic findings of noncaseating epithelioid granulomas. The choice of treatmentshould be performed considering the extention, severity of symptoms and thepossibility of disease progression with loss of function of the affected organ.Chloroquine (250-500 mg/d) and hydroxychloroquine (200-400 mg/d) have beenused as first-line drugs for the treatment of chronic skin sarcoidosis. In our case, theextent of skin involvement, with disfigurement, was instrumental in startingtreatment.

Case report: Male, black, age 54, as relates rise of asymptomatic papules andnodules, which started 5 years ago in the neck and progressively spread to the trunkand limbs. Dermatologic examination showed the presence of papules anderythematous violaceous nodules, grouped on the neck, trunk, upper limbs, andtuberous lesions in the extensor surface of the lower right limb. Chest radiograph,eye examination, electrocardiogram (ECG) testing, and laboratory tests (bloodcount, liver and kidney function, calcium, and C-reactive protein) showed nochanges. Negative smear. We decided, as therapy, the introduction of hydroxy-chloroquine 400 mg daily. The therapeutic choice was based on the extent andchronicity of skin lesions, and antimalarial drugs are effective in controlling chroniccutaneous sarcoidosis.

Discussion: The relationship between cutaneous and systemic sarcoidosis has beenevaluated. About 30% of patients with isolated cutaneous lesions, develop systemicinvolvement after a period of 1 month to 1 year. Systemic manifestations ofsarcoidosis are variable. Lung disease and hilar lymphadenopathy occur in approx-imately 90% of patients with systemic sarcoidosis. In the case described, there areonly cutaneous manifestation of the disease with characteristic histopathologicfeatures, without any evidence of systemic involvement; however, close monitoringis essential, because according to the literature, most patients develop later systemicinvolvement.

cial support: None identified.

Commer

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