RESUMO
Langerhans cell histiocytosis (LCH) is a complex disease characterized by proliferation of the Langerhans cells. The clinical course is variable and ranges from a solitary lytic bone lesion or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The diagnosis is suspected by clinical signs and symptoms and radiological features commonly in craniofacial bones and skin lesions. Diagnosis is confirmed by biopsy and immunohistochemical studies. We present case of a 8 year old child presenting with proptosis, diabetes insipidus and hypopigmented macules on chest and back showing bilateral distribution which is a rare presentation. Skin biopsy revealed the diagnosis of Langerhans cell histiocytosis.
Assuntos
Criança , Exoftalmia/diagnóstico , Exoftalmia/epidemiologia , Exoftalmia/etiologia , Humanos , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/diagnóstico , Masculino , Transtornos da Pigmentação/diagnóstico , Transtornos da Pigmentação/epidemiologia , Transtornos da Pigmentação/etiologiaRESUMO
We report a 19-year-old patient with numerous mottled hyperpigmented and hypopigmented macules over his left upper and lower extremities and left side of trunk. which are unilateral and dermatomal distribution. He has had these macules for 5 years. The family history does not revealed similar pigmentary changes. The pigmented macules resemble those of dyschromatosis universalis herediteria or dyschromatosis symmetrica hereditaria. In addition, the histopathologic features were also simlar to those reported in dyschromatosis, but clinically our case had unilateral dermatomal distribution which was different from typical dyschromatosis. Therefore, We think our case as atypjcal dyschromatosis