RESUMEN
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.
Asunto(s)
Granuloma/patología , Proteínas Proto-Oncogénicas B-raf/genética , Enfermedades de la Piel/patología , Anomalías Múltiples/genética , Anomalías Múltiples/patología , Administración Tópica , Preescolar , Clobetasol/uso terapéutico , Fármacos Dermatológicos/uso terapéutico , Cardiopatías Congénitas/genética , Cardiopatías Congénitas/patología , Humanos , Hidrocortisona/uso terapéutico , Masculino , Metaplasia/patología , Furoato de Mometasona , Pregnadienodioles/uso terapéutico , Enfermedades de la Piel/genética , Glándulas Sudoríparas/patología , Síndrome , Tacrolimus/análogos & derivados , Tacrolimus/uso terapéuticoRESUMEN
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.