RESUMO
RESUMEN La enfermedad de Rosai-Dorfman cutánea (ERDC) es una rara proliferación reactiva de histiocitos, que cursa con lesiones cutáneas sin compromiso generalizado. Las manifestaciones clínicas cutáneas son diversas y la variante clínica "tipo tumoración" es la forma más infrecuente. Se describe el caso de un paciente con ERDC que se presentó con esta forma inusual.
ABSTRACT Cutaneous Rosai-Dorfman disease (CRDD) is a rare reactive histiocytic proliferation, which presents with skin lesions without systemic involvement. The clinical cutaneous manifestations are diverse and the "tumor-like" clinical variant is the most infrequent. We describe the case of a patient with CRDD who presented with this unusual form.
RESUMO
Juvenile xanthogranuloma (JXG) predominantly occurs in infancy and childhood. Juvenile xanthogranulomas (JXGs) are asymptomatic; benign; self-healing; self-limiting red, yellow, or brownpapules and nodules composed of histiocytic cells. Most common site of occurrence is skin of the head and neck region and trunk but occurrence at rare sites such as nasal cavity, limbus of the eye, iris of the eye, anterior abdominal wall and tibia had been reported in literature. JXG is the most common form of non–Langerhans cell histiocytosis. Approximately 35% of cases of juvenile xanthogranuloma (JXG) occur at birth, with as many as 71% of cases occurring in the first year. The mean age at presentation is 22 months. Most JXGs resolve by age 5 years. Despite the term juvenile in the disease name, 10% of cases manifest in adulthood. Most of the lesions are solitary. Juvenile xanthogranuloma is a histiocytic inflammatory disorder capable of presenting as different histological patterns. The classic form consists of sheets of foamy histiocytes and numerous multinucleated Touton-type giant cells containing no to very few mitotic figures. We present a case of large juvenile xanthogranuloma (JXG) in a 7 year old child involving an unusual location and bilateral involvement of both the buttocks.
RESUMO
Benign cephalic histiocytosis is a rare non-Langerhans histiocytosis, which typically presents as a benign self-healing eruption of papules on the head and neck of infants and young children. We report the case of a 13-month-old boy who presented with many reticulated erythematous papules and macules on the face. The histologic examination showed relatively well-circimscribed band-like interstitial and perivascular infiltration of histiocytes, with vesicular nuclei and ample cytoplasms in the upper dermis. Immunohistochemically, the histiocytes were positive for CD68 while lacking expression of CD1a. Approximately 1 year after the first visit, the facial papules became flattened and hyperpigmented.