A hipomelanose macular progressiva é uma dermatose de etiopatogenia pouco conhecida. A participação do Propionibacterium acnes e a resposta ao tratamento com medicamentos com atividade para essa bactéria têm sido sugeridas. Relata-se uma série de casos de 13 pacientes com hipomelanose macular progressiva tratados com limeciclina e peróxido de benzoíla durante três meses, que apresentaram excelente resposta ao tratamento e nele se mantêm durante o período de seguimento do estudo
Oculoectodermal syndrome is a rare disease characterized by the association of
aplasia cutis congenita, epibulbar dermoids, and other abnormalities. This
report describes the twentieth case of the disease. We report a 4-year-old
female child who presented with the classical features of the syndrome: aplasia
cutis congenita and epibulbar dermoids. Our case expands the clinical spectrum
of the disease to include: diffuse hyperpigmentation (some following the
Blaschko´s lines); hypopigmented skin areas on the trunk; arachnoid cyst on the
right fronto-parietal border; rounded left side of the hippocampus; and dermoid
cyst underlying the bulb-medullary transition. Our patient also reported
infantile hemangioma on the right wrist and verrucous hemangioma on the left
leg, the latter not previously described in the literature.
Erythromelalgia is a rare clinical syndrome characterized by heat, redness and intermittent pain in the extremities, being most frequent the bilateral development in the lower extremities. Local cooling brings relief to symptoms, while heating, physical exercises and use of stockings/socks intensify the discomfort. This condition can be primary or idiopathic or secondary to haematological disorders and vascular inflammatory and degenerative diseases. It is reported the case of an eighteen-year-old male who presented, at the early age of two, development of the symptoms of erythema ,heat and pain followed by desquamation of hands and feet, in outbreaks, with intervals 4 to 5 years long between the crises. Keywords: Erythema; Erythromelalgia; Hot temperature; Pain Resumo: Eritromelalgia é uma síndrome clínica rara, caracterizada por calor, rubor e dor intermitente nas extremidades, sendo frequente o acometimento bilateral das extremidades inferiores. O resfriamento local provoca alívio dos sintomas, enquanto aquecimento, exercícios físicos e uso de luvas e meias intensificam o desconforto. A desordem pode ser primária ou idiopática, ou secundária a distúrbios hematológicos e doenças vasculares inflamatórias e degenerativas. Relata-se o caso de um jovem de dezoito anos, com início precoce aos dois anos de idade dos sintomas de eritema, calor e dor, seguidos de descamação nas mãos e pés, em surtos, com intervalos longos de 4 a 5 anos entre as crises.
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