Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
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PMID:Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? 1069 21

Benign cephalic histiocytosis (BCH) is a rare non-Langerhans cell histiocytosis of unknown etiology. Clinically, lesions are characterized by small, red-to-yellow papules distributed mainly on the head, face, neck, and shoulders of infants and children. Histopathological specimens show massive histiocytic infiltration of the superficial dermis. Immunohistochemically, they are positive for CD68, but negative for CD1a and S-100. Two cases have been reported so far in the relevant work published in Korean literature. Herein, we report on an additional case of BCH.
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PMID:A case of benign cephalic histiocytosis. 2202 63