Abstract Wolf's isotopic response designates the appearance of two subsequent unrelated dermatoses in the same anatomic location. We report the case of a 51-year-old man with a medical history of chronic lymphocytic leukemia without known extra-hematopoietic involvement. The patient developed a disseminated papulo-vesiculous eruption, diagnosed as varicella. Few days after recovering, an erythematous and violaceous papular dermatosis with histopathological examination compatible with leukemic infiltration appeared on the scars of previous herpetic lesions. Complete remission was obtained under systemic corticotherapy, without cutaneous recurrence or blastic transformation. Wolf's isotopic response is attributed to a localized immunologic imbalance following a certain stimulus. In this patient, herpetic infection acted as a local spur for inaugural cutaneous leukemic infiltration, with no impact on the prognosis for the underlying disease.

Abstract Porokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.

Uma mulher de 45 anos com história de carcinoma renal foi observada por pápulas cor da pele, faciais, cervicais e tronculares. Referia história familiar de achados cutâneos semelhantes e irmão com episódios repetidos de pneumotórax. Identificaram-se múltiplos quistos pulmonares por tomografia computorizada. Uma biópsia cutânea revelou fibroma perifolicular. O diagnóstico clínico de síndrome de Birt-Hogg-Dubé (BHDS) foi contudo corroborado pela identificação de uma nova mutação frameshift c.573delGAinsT (p.G191fsX31) em heterozigotia no exão 6 do gene da foliculina. A presença de múltiplos e típicos tumores benignos do folículo piloso, realça o papel do dermatologista no diagnóstico desta rara genodermatose, que está associada a um risco aumentado de tumores de células renais e cistos pulmonares, exigindo seguimento e aconselhamento pessoal e familiar.

A 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.

Uma mulher de 34 anos sem antecedentes patológicos conhecidos foi avaliada por apresentar múltiplos nódulos e pápulas castanhos, dolorosos, na face anterior do tronco. Referia história de achados cutâneos semelhantes na sua mãe. As biópsias de três lesões revelaram piloleiomiomas. As ecografias renal e suprarenal identificaram apenas cisto renal cortical simples, e as ecografias endovaginal e pélvica, dois miomas uterinos. O diagnóstico clínico de leiomiomatose herediária e câncer de células renais foi corroborado pela identificação de variante heterozigota no exon 5 do gene da Fumarato hidratase (c.578C>T p.T193I). O piloleimomioma é um tumor cuja identificação deve alertar o dermatologista para esta rara genodermatose, associada a um risco aumentado de tumores de células renais, exigindo seguimento multidisciplinar e aconselhamento pessoal e familiar.

A 34-year-old woman with no known medical history was evaluated for multiple painful brown nodules and papules on the anterior aspect of the trunk. She mentioned a history of similar cutaneous findings on her mother. Biopsies of three lesions revealed piloleiomyomata. Renal and adrenal ultrasound revealed an isolated simple cortical cyst, and pelvic and endovaginal ultrasound revealed two uterine myomata. The clinical diagnosis of hereditary leiomyomatosis and renal cell cancer was corroborated by the identification of a heterozygous variant on exon 5 of the fumarate hydratase gene (c.578C>T p.T193I). Identification of the tumor piloleiomyoma should alert the dermatologist to this rare genodermatosis, which is associated with an increased risk of renal cell tumors, demanding multidisciplinary follow-up, and personal and family counseling.

A neoplasia blástica de células dendríticas plasmocitóides é uma neoplasia hematodérmica rara, agressiva, com frequente envolvimento cutâneo e disseminação leucêmica. Relatamos o caso de um homem de 76 anos com quadro clínico com 2 meses de evolução caracterizado por nódulos e tumor de tonalidade violácea, de consistência pétrea, localizados na cabeça, e linfadenopatias mandibular, cervicais e supraclaviculares. Identificaram-se múltiplas adenopatias torácicas e abdominais em tomografia computorizada. A análise por citometria de fluxo de biópsias cutânea, ganglionar e óssea demonstrou a presença de precursores neoplásicos das células dendríticas plasmocitóides (fenótipo CD4+, CD45+, CD56+ e CD123+). Após remissão clínica e laboratorial completa inicial com quimioterapia, veio a falecer por recaída da doença associada ao aparecimento de massa cervical com compromisso medular.

Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.

Um homem de 27 anos, sem quaisquer antecedentes patológicos pessoais ou familiares conhecidos, mencionava dermatose com cerca de 10 anos de evolução, caracterizada por agrupamentos de telangiectasias, de distribuição blaschkóide, na face lateral direita do pescoço e assintomáticas. Negava quaisquer episódios de doença ou toma de fármacos que pudessem estar relacionados com esta doença. As análises sanguíneas do doente estavam inalteradas, nomeadamente os enzimas hepáticos e serologias infecciosas. Foi efectuado o diagnóstico clínico de Telangiectasia Nevóide Unilateral Adquirida, idiopática, uma malformação vascular benigna pouco comum. O doente prescindiu da realização de biópsia cutânea ou tratamento de intenção cosmética.

A 27 year-old-man, with no known personal or familial history of disease, mentioned a 10-year history of asymptomatic groups of telangiectasias, with a Blaschko lines distribution on the right lateral aspect of the neck and asymptomatic. He denied any episodes of disease or drug intake that could be associated with the disease. Blood work had no changes, namely of liver enzymes or infectious serologies. The clinical diagnosis of Idiopathic Acquired Unilateral Nevoid Telangiectasia was made, an uncommon, benign vascular malformation. The patient declined doing a cutaneous biopsy or treatment with a cosmetic intent.

Estima-se que cerca de 7% das crianças com idade inferior a dois anos tenha pelo menos um tipo de alteração ungueal e que estas alterações representem até 11% dos motivos de recurso à consulta de Dermatologia Pediátrica. As alterações ungueais adquiridas na criança são semelhantes às observadas no adulto, contudo diferem em prevalência e existem algumas particularidades específicas da infância. A patologia ungueal de origem traumática é a que mais motiva o recurso ao serviço de urgência. As restantes alterações, sobretudo as crónicas e assintomáticas, são frequentemente desvalorizadas; no entanto, podem constituir a primeira manifestação de doenças dermatológicas ou sistémicas. Abordam-se as principais alterações ungueais congénitas, traumáticas, infecciosas, secundárias a exposição farmacológica, neoplásicas e os sinais ungueais que podem ser identificados no contexto de outra doença cutânea ou sistémica.

About 7% of children under two years of age have at least one type of nail change and these disorders represent up to 11% of the motifs for consulting Pediatric Dermatology. Acquired nail changes in children are similar to those observed in adults, but there are differences in the prevalence and there are some particularities of childhood. Traumatic nail injuries are the most frequent nail disorder observed at the emergency department. Other disorders, especially chronic and asymptomatic ones, are often undervalued, although they might be the first manifestation of systemic or skin diseases. We focused the most important nail disorders, such as congenital, traumatic, infectious, drug-related, neoplastic and nail signs that can be identified in the context of skin or systemic diseases.