Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An otherwise healthy 50-year-old woman presented with a 6-month history of having developed more than 100 generalized, non-confluent, reddish-brown, partially yellow-coloured papules. A non-epidemotropic, monomorphous infiltrate of vacuolated mononuclear, and occasionally multinuclear, histiocytes, positive for factor XIIIa and macrophage markers HAM56 and KiM1p, was consistent with the clinical impression of generalized eruptive histiocytomas. However, the additional reactivity for S100 protein, in the absence of features of histiocytosis X, suggested a diagnosis of indeterminate cell histiocytosis (ICH). Further immunohistochemical studies, performed on snap-frozen material, characterized the lesions as being diffusely positive with LN3 (HLA-DR), Leu4 (CD3) and Leu3 (CD4), the infiltrate in the upper dermis as reactive for OKT6 (CD1) and IOT6c (CD1c), and the infiltrate in the lower dermis as reactive for a variety of macrophage markers. Ultrastructural studies showed various non-specific features of histocytic disorders, but no Birbeck granules. Our findings confirm those of previous reports suggesting that ICH is a distinct histiocytic entity, characterized by immunophenotypic features of both X- and non-X histiocytoses. Generalized eruptive histiocytoma seems to be an early indeterminate stage of various non-X histiocytic syndromes including ICH, multicentric reticulohistiocytosis, xanthogranuloma and xanthoma disseminatum. The distribution pattern of the various X/non-X histiocytic markers suggests dermal arrest of antigen-presenting cells during their physiological trafficking from the skin to the lymph nodes.
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PMID:Indeterminate cell histiocytosis--a clinicopathological entity with features of both X- and non-X histiocytosis. 873 82

Generalized eruptive histiocytoma (GEH) is a rare, benign, papular, self-healing histiocytosis characterized by recurrent crops of small, firm, tan to reddish papules that appear in a symmetrical fashion on the face, trunk and arms, affecting mainly adults. Approximately 31 cases have been described, of which 8 were in children. A new concept unifying the confusing potpourri of non-Langerhans cell histiocytosis has recently been described in which GEH has been considered to represent an early undifferentiated stage of various histiocytic disorders. We describe a 9-year-old boy who had all the features of GEH and emphasize the importance of clinical, histologic, immunohistochemical, and ultrastructural examination in the diagnosis of histiocytic disorders. On the basis of the increasing numbers of similar reported cases showing overlap in clinical and histologic features and in accordance with the new unifying concept of non-Langerhans cell histiocytosis, we presume that this group of disorders may represent a continuous spectrum of a single disease rather than a collection of separate disorders.
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PMID:Generalized eruptive histiocytoma: a pediatric case. 1112 77

Generalized eruptive histiocytoma is a rare form of non Langerhan's cell histiocytosis. The disease occurs mainly in adults and its etiology is still unknown. We describe a case of 48-year-old female with multiple, firm, hemispherical, redish brown papules. Lesions were distributed on the face, upper limbs and trunk. Patient's general examination and routine laboratory investigations were normal. Excisional biopsy was taken from one representative lesion. Histopathological examination revealed diffuse dermal histiocytic infiltration that was suggestive of generalized eruptive histiocytoma. Confirmatory immunohistochemical staining for CD68 antibody was done and revealed positive results. Based on clinical and histopathological criteria the diagnosis of generalized eruptive histiocytoma was established.In conclusion, we present a rare case of generalized eruptive histiocytoma which is an uncommon form of non Langerhan's cell histiocytosis. The disease does not require treatment since it is a self-healing disease.
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PMID:Photoletter to the editor: Generalized eruptive histiocytoma. 2218 81