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

Congenital self-healing histiocytosis (CSHH) is a rare primary skin disorder. Of the two cases in newborn infants reported here, one had numerous widespread lesions while the other had a solitary ulcerating scalp nodule. Both neonates were otherwise healthy; neither exhibited either systemic involvement or involvement of mucous membranes. The findings drawn from the skin biopsies, including histology, S-100 positivity in the majority of the cells, and the presence of Birbeck granules, were indistinguishable from those described in infantile Letterer-Siwe disease (histiocytosis X). However, the benign clinical course, with rapid regression of the nodules in both cases, was diagnostic of CSHH.
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PMID:Congenital self-healing histiocytosis. Report of two cases with histochemical and ultrastructural studies. 195 85

Congenital self-healing histiocytosis (CSHH) is a rare primary histiocytic skin disorder. Only a few cases have been studied by ultrastructure and immunohistochemistry. Here we report a new case that was investigated using an electron microscope and a panel of monoclonal (MCA) and polyclonal (PCA) antibodies. CSHH cells were found to bear the immunohistochemical phenotype of normal epidermal Langerhans cells (LC) and histiocytosis X (HX) cells (CD1a/c+, CD1b-, CD4+/-, human leukocyte antigen [HLA]-DR/DQ+, S-100+). However, an electron microscope showed a paucity of Birbeck granule (BG)-containing cells. This contrasted with their immunophenotype. This finding, along with other ultrastructural characteristics of CSHH cells, suggests that histologic differences exist between CSHH and HX. However, because no absolute histologic criterion is known that allows unequivocally the differential diagnosis between the two diseases, this distinction currently has to rely on clinical criteria, mainly the regressive course observed within a few months in CSHH. The precise nosologic position of CSHH among other histiocytic syndromes remains unsettled.
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PMID:Congenital self-healing histiocytosis (Hashimoto-Pritzker). An ultrastructural and immunohistochemical study. 327 80