RESUMO
Cardiofaciocutaneous syndrome is a rare genetic disorder characterized by dysmorphic facial features and neurologic, cardiac, ophthalmologic, and dermatologic findings. Previously reported skin and hair findings in cardiofaciocutaneous syndrome include sparse, slow-growing curly hair, atopic dermatitis, ichthyosis, follicular hyperkeratosis, and keratosis pilaris. We report the case of a 4-year-old boy who has cardiofaciocutaneous syndrome with previously unreported histopathologic findings of eccrine squamous metaplasia and periadnexal granuloma.
Assuntos
Granuloma/patologia , Proteínas Proto-Oncogênicas B-raf/genética , Dermatopatias/patologia , Anormalidades Múltiplas/genética , Anormalidades Múltiplas/patologia , Administração Tópica , Pré-Escolar , Clobetasol/uso terapêutico , Fármacos Dermatológicos/uso terapêutico , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/patologia , Humanos , Hidrocortisona/uso terapêutico , Masculino , Metaplasia/patologia , Furoato de Mometasona , Pregnadienodiois/uso terapêutico , Dermatopatias/genética , Glândulas Sudoríparas/patologia , Síndrome , Tacrolimo/análogos & derivados , Tacrolimo/uso terapêuticoRESUMO
BACKGROUND: Gloves and socks syndrome (GSS) is a recently described exanthem, most commonly caused by parvovirus B19. OBJECTIVE: Our purpose was to describe both early and late histopathologic features of GSS. METHODS: We performed histopathologic examination of biopsy specimens from a case of GSS and reviewed all the English-language literature reports of GSS to compare the reported histologic descriptions. RESULTS: A total of 46 cases of GSS have been reported in the English-language literature. In 18 of those, the pathologic features were described. The histologic features of the current case evolved from a nonspecific superficial perivascular lymphocytic infiltrate to a vacuolar interface dermatitis with necrotic keratinocytes, erythrocyte extravasation, and a superficial perivascular and interstitial lymphocytic infiltrate. CONCLUSION: Early lesions of GSS show nonspecific features common to viral exanthems. The late features, of a fully evolved exanthem, demonstrate a vacuolar interface dermatitis with necrotic keratinocytes, a superficial perivascular and interstitial infiltrate, and dermal hemorrhage. These late pathologic features, together with the clinical appearance of GSS, may help distinguish it from other entities.