Case for diagnosis.

Focal acral hyperkeratosis is a rare genodermatosis with an autosomal dominant pattern of inheritance. It is characterized by usually asymptomatic keratotic papules along the borders of the hands and/or feet. The main differential diagnosis is acrokeratoelastoidosis of Costa, which differs from the former only by not presenting elastorrhexis in histopathological examination, thus requiring this exam for a correct diagnosis.

Case for diagnosis

Focal acral hyperkeratosis is a rare genodermatosis with an autosomal dominant pattern of inheritance. It is characterized by usually asymptomatic keratotic papules along the borders of the hands and/or feet. The main differential diagnosis is acrokeratoelastoidosis of Costa, which differs from the former only by not presenting elastorrhexis in histopathological examination, thus requiring this exam for a correct diagnosis.

Primary essential cutis verticis gyrata - case report.

Cutis verticis gyrata is characterized by excessive formation of scalp skin. It may be primary (essential and nonessential) or secondary. In the primary essential form it presents only folding skin formation on the scalp, mimicking cerebral gyri, without associated comorbidities. We report a rare case of a 28 year-old male patient with primary essential cutis verticis gyrata.

Primary essential cutis verticis gyrata - Case report

Cutis verticis gyrata is characterized by excessive formation of scalp skin. It may be primary (essential and nonessential) or secondary. In the primary essential form it presents only folding skin formation on the scalp, mimicking cerebral gyri, without associated comorbidities. We report a rare case of a 28 year-old male patient with primary essential cutis verticis gyrata.

Linear IgA bullous dermatosis: report of an exuberant case.

Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

Linear IgA bullous dermatosis: report of an exuberant case / Dermatose por IgA Linear: relato de um caso exuberante

Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

A dermatose por imunoglobulina A (IgA) linear é doença bolhosa autoimune rara, porém a mais comum das dermatoses bolhosas autoimunes da infância. Relatamos caso típico e exuberante de dermatose por IgA linear em uma criança de 10 meses, que apresentou boa evolução com o tratamento com corticóide e dapsona.

Calcinose cutânea distrófica em adolescente: relato de caso / Dystrophic calcinosis cutis in an adolescent: a case report

Calcinose cutânea é a precipitação de cristais de cálcio na pele. Pode ser dividida em quatro categorias: distrófica, metastática, iatrogênica e idiopática. A forma distrófica é a mais comum, com níveis séricos normais decálcio e de fósforo. Relatamos caso de calcinose cutânea distrófica localizada e assintomática no joelho direito de umadolescente. Os exames clínico e laboratorias revelaram telangectasias periungueais e fator antinuclear positivo (título maior que 1/1280 de padrão nucleolar), o que sugere aassociação da calcinose cutânea com uma colagenose...

Calcinosis cutis is the precipitation of calcium crystals in the skin. Can be divided in four categories: dystrophic,metastatic, idiopathic and iatrogenic. The dystrophic form is the most common, with normal serum calcium and phosphorus. Report a case of dystrophic calcinosis cutis asymptomatic and located in the right knee of a teenager. The clinical and laboratory examinations revealed periungual telangiectasia and positive antinuclear factor (Title greater than 1/1280 of nucleolar pattern), suggesting the association of cutaneous calcinosis with a collagen disease...

The importance of trichological examination in the diagnosis of alopecia areata.

In this article we discuss the propedeutic aspects of alopecia areata, especially those found by dermatoscopy, an invaluable tool for diagnosis of the condition. Dermatoscopy facilitates the early detection of the characteristic changes in alopecia areata hair such as exclamation-point hairs, cadaverous hair, fuzzy hair, vellus type hair and yellow spots.

A importância do exame tricológico no diagnóstico da alopecia areata / The importance of trichological examination in the diagnosis of alopecia areata

Neste artigo, destacam-se os aspectos propedêuticos da alopecia areata, em especial, os encontrados na dermatoscopia, ferramenta muito útil para o diagnóstico. A dermatoscopia facilita a detecção precoce das alterações características dos cabelos na alopecia areata, como: pelos em ponto de exclamação, pelos cadavéricos, fuzzy, pelos tipo velo e pontos amarelos.

In this article we discuss the propedeutic aspects of alopecia areata, especially those found by dermatoscopy, an invaluable tool for diagnosis of the condition. Dermatoscopy facilitates the early detection of the characteristic changes in alopecia areata hair such as exclamation-point hairs, cadaverous hair, fuzzy hair, vellus type hair and yellow spots.

Psoríase pustulosa palmoplantar tratada com etanercepte: relato de caso / Palmoplantar pustular psoriasis treated with etanercept

Contexto: A psoríase pustulosa palmoplantar (PPPP) é uma das apresentações clínicas da psoríase, muitas vezes de difícil tratamento, podendo-se utilizar diversos medicamentos tópicos e sistêmicos. O uso dos anti-TNFα no tratamento das formas pustulosas de psoríase é controverso, visto que a resposta clínica é variável, além desta classe de medicação biológica poder desencadear psoríase pustulosa. Descrição do Caso: Doente feminina, 60 anos, branca, com diagnóstico de PPPP há 12 anos e artrite psoriásica há seis anos. Antecedentes pessoais relevantes: hipertensão arterial sistêmica, hipertrigliceridemia e obesidade. Apresentou refratariedade tanto aos tratamentos tópicos instituídos (corticóides e emolientes) quanto aos sistêmicos (metotrexate, dapsona, colchinina e acitretina), tendo evoluído com excelente resposta terapêutica com etanercepte (administrado semanalmente, por via subcutânea, na dose de 50 mg). Discussão: Os anti-TNFα são eficazes no tratamento da psoríase em placas moderada a grave.Mas, o tratamento da PPPP com anti-TNFα não é classicamente preconizado. Segundo a literatura a resposta terapêutica com este tipo de medicamento é variável na PPPP. Além disso, os anti-TNFα podem desencadear quadro de pustulose palmoplantar. No entanto, há relatos de sucesso terapêutico com anti-TNFα em casos refratários de PPPP. Em função das comorbidades da paciente e após terem sido esgotadas as possibilidades terapêuticas clássicas, optamos pelo uso do etanercepte, que se mostrou eficaz.A doente iniciou tratamento em abril de 2008 e mantém o uso do etanercepte até a presente data, estando em remissão da doença. Conclusão: Destacamos a possibilidade do uso de anti-TNFα em paciente com PPPP refratária ao tratamento convencional. No nosso caso, o etanercepte mostrou-se eficaz e seguro, não tendo a doente apresentado nenhum efeito adverso grave.

Background: Palmoplantar pustular psoriasis (PPPP) is one of the clinical presentations of psoriasis, often difficult to treat, can be used several topical and systemic. The use of anti-tumor necrosis factor alpha (anti-TNFα) in the treatment of pustular forms of psoriasis is controversial, with a variable clinical response, outside this class of biological medication may trigger pustular psoriasis. CASE REPORT: Patient 60, female, white, with a diagnosis of PPPP for 12 years and psoriatic arthritis for 6 years. Relevant personal history: hypertension, hypertriglyceridemia andobesity. Presented refractory to topical treatments (corticosteroids and emollients) and systemic (methotrexate, dapsone, colchicineand acitretin), having evolved with excellent response to treatment with etanercept (administered weekly by subcutaneous injection at a dose of 50 mg). Discussion: The anti-TNFα are effective in the treatment of psoriasis in moderate to severe plaque. But, inthe treatment of PPPP is not classically recommended. According to the literature, the therapeutic response with this type of drug is variable in PPPP. In addition, anti-TNFα may trigger clinical of palmoplantar pustulosis. However, there are reports of therapeutic success with anti-TNFα in refractory cases of PPPP. Due to patient’s comorbidities, and after having exhausted the possibilities classical therapies, we chose the use of etanercept, which proved effective. The patient began treatment in April 2008 and keeps the use of etanercept until the present date, being in remission.Conclusion: We emphasize the possibility of using anti-TNFα in patients with PPPP refractory to conventional treatment. In our case, etanercept was effective and safe, not having the patient presented any serious adverse events.

Bullous pemphigoid in younger adults: three case reports.

Bullous pemphigoid is an autoimmune subepidermal bullous dermatosis more commonly observed in the elderly (over 70 years old). Autoantibodies are produced for specific antigens of the epidermal basement membrane zone: BP 180 and BP 230 (hemidesmosome proteins). We report three cases of bullous pemphigoid in adults younger than 50 years old, discussing the clinical characteristics of the disease in younger patients.

Penfigoide bolhoso no adulto mais jovem: relato de três casos / Bullous pemphigoid in younger adults: three case reports

O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, mais comumente observada na população idosa (acima dos 70 anos). Autoanticorpos são formados contra antígenos específicos da zona de membrana basal: BP180 e BP230 (proteínas do hemidesmossomo). Apresentamos três casos de penfigoide bolhoso, em adultos com menos de 50 anos de idade, destacan do as peculiaridades clínicas na faixa etária mais jovem.

Bullous pemphigoid is an autoimmune subepidermal bullous dermatosis more commonly observed in the elderly (over 70 years old). Autoantibodies are produced for specific antigens of the epidermal basement membrane zone: BP 180 and BP 230 (hemidesmosome proteins). We report three cases of bullous pemphigoid in adults younger than 50 years old, discussing the clinical characteristics of the disease in younger patients.

Immunopathogenesis of psoriasis.

Psoriasis is a common inflammatory disease affecting 1% of the Brazilian population. Th17 and Th1cells are involved with the immunopathogenisis of psoriasis. In this article it is discussed the interaction between the innate immunity (especially dendritic cells and keratinocytes) and adaptive immunity (T lymphocytes) in the pathogenesis of psoriasis.

Imunopatogênese da psoríase / Immunopathogenesis of psoriasis

A psoríase é doença inflamatória comum, afetando cerca de 1 por cento da população brasileira. Os linfócito T auxiliares (Th17 e Th1) estão envolvidos na imunopatogênese da psoríase. Neste artigo é discutida a interação entre a imunidade inata (especialmente células dendríticas e queratinócitos) e adquirida (linfócitos T) na patogênese da psoríase.

Psoriasis is a common inflammatory disease affecting 1 percent of the Brazilian population. Th17 and Th1cells are involved with the immunopathogenisis of psoriasis. In this article it is discussed the interaction between the innate immunity (especially dendritic cells and keratinocytes) and adaptive immunity (T lymphocytes) in the pathogenesis of psoriasis.

Caso para diagnóstico / Case for diagnosis

A sarcoidose é uma doença sistêmica crônica idiopática caracterizada histologicamente por um acúmulo de fagócitos mononucleares e células gigantes, que levam à formação de granulomas não-caseosos em diversos órgãos. O envolvimento cutâneo ocorre, em aproximadamente, 25 por cento dos casos, sendo representado por lesões específicas e não-específicas. Apresentamos caso raro de sarcoidose com lesões cutâneas papulosas e umbilicadas e envolvimento pulmonar.

Sarcoidosis is a chronic idiopathic disorder characterized by the accumulation of mononuclear phagocytes with the formation of noncaseating granulomas in multiple organs. Cutaneous involvement occurs in 20-25 percent of cases and specific and nonspecific lesions are observed. We reported a rare case of umbilicated papular sarcoidosis with pulmonary involvement.

Inflammatory epidermolysis bullosa acquisita: case report.

We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.

Epidermólise bolhosa adquirida inflamatória: relato de caso / Inflammatory epidermolysis bullosa acquisita: case report

Apresenta-se caso de epidermólise bolhosa adquirida inflamatória. Paciente do sexo masculino, 53 anos, há seis meses com erupção vesicobolhosa pruriginosa sobre base eritematosa no tronco, axilas e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos. A imunofluorescência direta revelou depósitos lineares de IgG, IgA, IgM e C3 na zona da membrana basal, sendo a imunofluorescência indireta e o Salt Split Skin indireto negativos. Anticorpos antinucleares não reagentes. Houve melhora do quadro com prednisona e cicatrização de algumas lesões com formação de milia. Trata-se de apresentação rara de epidermólise bolhosa adquirida, com lesões iniciais predominantemente inflamatórias.

We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.

[Case for diagnosis]. / Caso para diagnóstico.

Sarcoidosis is a chronic idiopathic disorder characterized by the accumulation of mononuclear phagocytes with the formation of noncaseating granulomas in multiple organs. Cutaneous involvement occurs in 20-25% of cases and specific and nonspecific lesions are observed. We reported a rare case of umbilicated papular sarcoidosis with pulmonary involvement.

Tungíase disseminada tratada com ivermectina / Generalized tungiasis treated with ivermectin

A tungíase é ectoparasitose causada pela penetração da Tunga penetrans na pele do hospedeiro. A infestação por essa pulga está associada à pobreza e ocorre em comunidades carentes na América do Sul e Central, no Caribe e na África sul-sahariana. No Brasil, é mais prevalente em assentamentos urbanos precários, áreas rurais e comunidades de pescadores em todo o país. O tratamento consiste na retirada dos parasitas e, nos casos com infecção secundária, antibioticoterapia. Até o momento, não há droga no mercado com eficácia clínica satisfatória. Relata-se caso de tungíase disseminada tratada com ivermectina oral.

Tungiasis is an ectoparasitic infection caused by the penetration of the sand flea Tunga penetrans into the skin of the host. Flea infestation is associated with poverty and occurs in resource-poor communities in South and Central America, the Caribbean and Sub-Saharan Africa. In Brazil, it is widespread in urban squatter settlements, villages in the rural hinterland and in traditional fishing communities along the coast. Standard therapy for tungiasis consists of removing the embedded flea with a sterile needle, and using an antibiotic agent in case of secondary infection. At present, there is no drug in the market with satisfactory clinical efficacy. We describe a case of generalized Tunga penetrans infestation that was treated with oral ivermectin.