RESUMO
Severe combined immunodeficiency includes a group of diseases characterized by different inherited immunological defects. A 4-month-old girl diagnosed with Omenn syndrome, a subtype of severe combined immunodeficiency presenting with generalized erythroderma, was referred to our hospital for an allogeneic stem cell transplantation. Days before transplantation, she developed hyperpigmented macules that increased in number in the following months. As the erythroderma resolved after transplantation, diffuse hypopigmentation was simultaneously noted together with the expansion of hyperpigmented lesions. Cutaneous biopsy samples were taken at different moments, showing features of Omenn syndrome at first, and 2 months later changes consistent with hypopigmentation and repigmentation were observed. Although pigmentary disorders are rarely described in this context, these must be taken into account as a possible alternative diagnosis to graft-versus-host disease and toxicoderma in immunosuppressed patients.
Assuntos
Transtornos da Pigmentação/complicações , Transtornos da Pigmentação/patologia , Imunodeficiência Combinada Severa/complicações , Imunodeficiência Combinada Severa/patologia , Feminino , Humanos , Lactente , Pigmentação da PeleRESUMO
Cold panniculitis has been described in children and young women following cold exposure. Histopathologically, cold panniculitis shows a mostly lobular panniculitis, which consists of an infiltrate of lymphocytes and histiocytes in the fat lobules. Usually, the dermis shows a superficial and deep perivascular infiltrate mostly composed of lymphocytes, with no vasculitis. Inflammation is most intense at the dermal-subcutaneous junction. Differential diagnosis of cold panniculitis should be established with subcutaneous fat necrosis of the newborn, sclerema neonatorum, poststeroid panniculitis, chilblains, and frostbites.
Assuntos
Temperatura Baixa/efeitos adversos , Exposição Ambiental/efeitos adversos , Paniculite/etiologia , Pele/patologia , Clima Frio/efeitos adversos , Diagnóstico Diferencial , Humanos , Paniculite/patologiaRESUMO
Annular elastolytic giant cell granuloma (AEGCG) is an uncommon entity clinically characterized by erythematous annular plaques with atrophic and hypopigmented center, that predominates in sun-exposed zones. The histology shows a granulomatous infiltrate without palisading image, made up of lymphocytes, histiocytes and giant cells, with phagocytosis of elastic fibers, without necrobiosis or mucin deposit. We present the case of a male patient with atypical clinical manifestation on the non-sun exposed skin and AEGCG characteristic histology.