Linear chronic discoid lupus erythematosus following the lines of Blaschko.

Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. This report describes the case of a 15-year old boy with a 2-year history of discoid, erythematous, hyper and hypopigmented lesions with central atrophy, situated along the lines of Blaschko on his right arm. Histopathology showed epidermal atrophy, hyperkeratosis, follicular plugging, thickening of the basement membrane zone, and superficial and deep chronic perivascular and periadnexal inflammatory infiltrate, with dermal mucin deposit, thus confirming diagnosis. A total of 14 cases have been described of this variant, the onset of which is often in childhood. There is no difference in incidence between genders. Lesions most commonly develop on the face. There have been no reports of any association with systemic disease.

Lúpus eritematoso crônico discoide nas linhas de Blaschko / Linear chronic discoid lupus erythematosus following the lines of Blaschko

O lúpus eritematoso crônico discoide linear é manifestação rara da doença lúpica cutânea, em que lesões eritêmato-atrófico-discrômicas dispõem-se nas linhas de Blaschko. Descrevemos o caso de um menino de 15 anos, com dois anos de história de lesões discoides eritêmato-atróficas, hipo e hiperpigmentadas, dispostas nas linhas de Blaschko do membro superior direito. O exame histopatológico revelou atrofia da epiderme, hiperqueratose, rolhas córneas, espessamento da zona da membrana basal, infiltrado inflamatório crônico perianexial e perivascular superficial e profundo, depósito de mucina na derme, confirmando o diagnóstico. Há, no total, 14 casos descritos dessa variante que se inicia frequentemente na infância e que não apresenta predomínio quanto ao sexo. As lesões ocorrem preferencialmente na face. Não há relatos de associação com doença sistêmica.

Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. This report describes the case of a 15-year old boy with a 2-year history of discoid, erythematous, hyper and hypopigmented lesions with central atrophy, situated along the lines of Blaschko on his right arm. Histopathology showed epidermal atrophy, hyperkeratosis, follicular plugging, thickening of the basement membrane zone, and superficial and deep chronic perivascular and periadnexal inflammatory infiltrate, with dermal mucin deposit, thus confirming diagnosis. A total of 14 cases have been described of this variant, the onset of which is often in childhood. There is no difference in incidence between genders. Lesions most commonly develop on the face. There have been no reports of any association with systemic disease.

Case for diagnosis. Actinic prurigo.

A 13-year-old black boy had pruritic papular and nodular lesions on his forearms associated to edema of the lower lip, photophobia, conjunctivitis and pterygium. Skin biopsy of the lower lip revealed acanthosis, spongiosis with dermal perivascular mononuclear cell infiltration composed by lymphocytes, plasma cells and eosinophils consistent with actinic prurigo. Lesions improved considerably with the use of thalidomide 100mg/ day.

Caso para diagnóstico / Case for diagnosis

Paciente do sexo masculino, negro, 13 anos, apresenta há dois anos lesões pruriginosas, pápulonodulares nos antebraços, associadas a edema do lábio inferior, fotofobia, conjuntivite e pterígio. O exame histopatológico do lábio inferior revelou acantose, espongiose e infiltrado inflamatório perivascular superficial, composto por linfócitos, plasmócitos e eosinófilos, compatível com o diagnóstico de prurigo actínico. As lesões regrediram com o uso de talidomida 100 mg/dia.

A 13-year-old black boy had pruritic papular and nodular lesions on his forearms associated to edema of the lower lip, photophobia, conjunctivitis and pterygium. Skin biopsy of the lower lip revealed acanthosis, spongiosis with dermal perivascular mononuclear cell infiltration composed by lymphocytes, plasma cells and eosinophils consistent with actinic prurigo. Lesions improved considerably with the use of thalidomide 100mg/ day.

Granulomas não-infecciosos: sarcoidose: [revisão] / Noninfectious granulomas: sarcoidosis: [review]

A sarcoidose é doença granulomatosa não infecciosa de etiologia desconhecida, de cuja patogênese parecem participar os fatores genéticos, imunológicos, ambientais e infecciosos. Vários órgãos podem ser afetados, causando amplo espectro de manifestações clínicas. A pele é acometida em cerca de 20 a 35 por cento dos casos, proporcionando ao dermatologista importante papel no diagnóstico da doença. Epidemiologia, imunologia e tratamento também são discutidos para prover melhor entendimento dessa enfermidade.

Sarcoidosis is a noninfectious granulomatous disease of unknown etiology, in which genetic, immunological, environmental and infectious factors seem to participate in the pathogenesis. Any organ or system can be affected with a wide range of clinical manifestations. As involvement of the skin occurs in approximately 20 to 35 percent of the cases, the dermatologist may play an important role in the diagnosis of the disease. Epidemiology, immunology and treatment are also discussed to provide a better understanding of this entity.

Anetoderma associated with primary antiphospholipid syndrome.

We report the case of a 34-year-old white man with a 7-year history of nodules on the face, neck, upper torso, and arms which evolved to anetodermic lesions. After onset of these lesions, deep vein thrombosis developed in the patient's right arm and leg in association with circulating antiphospholipid antibodies.

Hypertrophic lupus erythematosus: a clinicopathological study of 14 cases.

BACKGROUND: Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of lupus erythematosus (LE). It is characterized by verrucous lesions, chronic in its course, and resistant to treatment. The purpose of this study was to identify clinical and histological characteristics of HLE. METHODS: We review our experience with 14 cases of HLE identified in a group of 220 patients with different forms of LE, at the UNICAMP Hospital, between 1976 and 2002. RESULTS: All patients presented verrucous plaques concomitantly with discoid lesions. The most common sites of involvement were the face and the arms. Histology of HLE lesions revealed pseudoepitheliomatous hyperplasia engulfing elastotic material. Elastic fibers were seen in migration throughout the epidermis. Classic features of LE were noted in all cases. Three of the patients developed hyperkeratotic papules with central keratinous plug on their arms at the previous LE sites. These lesions resemble clinically and histopathologically keratoacanthomas. In one patient, HLE lesion progressed to squamous cell carcinoma (SCC), 26 years after the onset of the disease. CONCLUSIONS: Transepithelial elimination of the elastotic material may be a feature of HLE. Some HLE lesions may present as keratoacanthoma, but classical features of LE aid the correct diagnosis. SCC may arise on a long-standing HLE lesion; therefore HLE requires clinical and histopathological follow up.