Assuntos
Dermoscopia , Histiocitose de Células não Langerhans/diagnóstico por imagem , Doenças Labiais/diagnóstico por imagem , Dermatopatias/diagnóstico por imagem , Adulto , Histiocitose de Células não Langerhans/patologia , Humanos , Doenças Labiais/patologia , Masculino , Dermatopatias/patologiaRESUMO
Stabilin-1 is an endocytotic scavenger receptor, specifically expressed by non-continuous sinusoidal endothelial cells in the liver, spleen and lymph nodes and by M2 or alternatively activated macrophages in human malignancies. We analysed paraffin-embedded tissue of melanocytic lesions and granulomatous diseases for stabilin-1 expression, using the human/murine RS1 antibody. The specificity of the RS1 staining was confirmed in a knockout model, as only M2-like tumor-associated macrophages and vessels of a B16F10 melanoma in wild type mice stained positive; while staining of tumor-associated macrophages and vessels originating from stabilin-1 deficient mice remained negative for stabilin-1 specific antibody RS1. In human specimens, the RS1 antibody stained tumor-associated macrophages in all pathological stages of melanoma. In addition, five cases of juvenile xanthogranulomas and one case of necrobiotic xanthogranuloma were strongly stabilin-1 positive, while Th-1 cytokine dominated granulomatous diseases such as sarcoidosis and granulomatous leprosy were negative. Stabilin-1 positive vessels were found in all analysed non-Langerhans cell histiocytoses and melanocytic lesions. No stabilin-1 positive vessels were present in any other granulomatous diseases.
Assuntos
Moléculas de Adesão Celular Neuronais/metabolismo , Histiocitose de Células não Langerhans/metabolismo , Macrófagos/metabolismo , Xantogranuloma Necrobiótico/metabolismo , Nevo Pigmentado/metabolismo , Receptores de Retorno de Linfócitos/metabolismo , Neoplasias Cutâneas/metabolismo , Xantogranuloma Juvenil/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Animais , Moléculas de Adesão Celular Neuronais/deficiência , Moléculas de Adesão Celular Neuronais/genética , Modelos Animais de Doenças , Feminino , Xenoenxertos , Histiocitose de Células não Langerhans/patologia , Humanos , Imuno-Histoquímica , Linfonodos/metabolismo , Linfonodos/patologia , Macrófagos/patologia , Masculino , Melanoma/metabolismo , Melanoma/patologia , Camundongos , Camundongos Knockout , Pessoa de Meia-Idade , Xantogranuloma Necrobiótico/patologia , Nevo Pigmentado/patologia , Estudos Retrospectivos , Neoplasias Cutâneas/patologia , Xantogranuloma Juvenil/patologiaRESUMO
Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.
Assuntos
Histiocitose de Células não Langerhans/patologia , Leucemia Mieloide Aguda/complicações , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/patologia , Adulto , Biópsia , Diagnóstico Diferencial , Humanos , MasculinoRESUMO
A 50-year-old male presented with fever, joint pain and skin lesions since eight months. Examination showed multiple papules and nodules with periarticular predisposition. Swelling of knees and elbows with flexion deformity of distal interphalangeal joints was present. Investigations revealed anemia and raised ESR. Histopathology was pathognomonic of multicentric reticulohistiocytosis. Patient was treated with bisphosphonates along with systemic steroids and methotrexate to which he responded well.