Normolipemic papuloeruptive xanthomatosis in a child.

Department of Dermatology, Cathay General Hospital, Huinchu, Taipei, Taiwan.
Pediatric Dermatology (Impact Factor: 1.52). 05/2009; 26(3):360-2. DOI: 10.1111/j.1525-1470.2009.00925.x
Source: PubMed

ABSTRACT Normolipemic papuloeruptive xanthomatosis is a very rare skin disorder. We report a child with yellowish papular eruptions on the face that rapidly merged into confluent plaques. Serum lipid profiles showed normolipemia. A diagnosis of normolipemic papuloeruptive xanthomatosis with no associated systemic disorders was made. The skin lesions involuted spontaneously during follow-up.

  • [Show abstract] [Hide abstract]
    ABSTRACT: Papular xanthoma (PX) is one of several clinicopathologic variants of normolipemic cutaneous non-Langerhans cell histiocytoses (n-LCH). PX represents a monomorphous reaction pattern of n-LCH characterized by the presence of predominantly xanthomatized macrophages. The purpose of this study was to identify the clinical, histological and immunohistochemical characteristics of PX. A series of 10 cases of PX was identified and the results compared with the other histologic subtypes, namely the polymorphous and the remaining other monomorphous reaction patterns in n-LCH. In this clinicopathologic study, papular xanthoma presented clinically mainly as solitary papule, with a male to female ratio of4 : 1, in an age range from 13 to 57 years and a biphasic occurrence: in the young adolescence and middle ages. It was predominantly located on the trunk, the extremities, and rarely on the head. Clinically, PX was described as xanthoma, 'cutaneous tumor', but also as atheroma, keloid, histiocytoma, Spitz's nevus or clear cell acanthoma. Histology showed moderately well circumscribed exoendophytic papules with a regular epidermis and a dense infiltration of xanthomatized macrophages interspersed by numerous Touton type giant cells. Immunohistochemically mono- and multinucleated macrophages were consistently positive with KiM1p; while only giant cells were labeled with KP1 (CD68), the reactivity with HAM 56 was much more variable. Up to 50% of the xanthomatized cells labeled positive for the lectin peanut agglutinin. In one case the xanthomatized cells stained positive for CD34. Staining for factor XIIIa and CD1a were negative. This series confirms PX as a rare, but distinguished clinicopathologic entity in the spectrum of n-LCH of the skin.
    Journal of Cutaneous Pathology 05/2002; 29(4):200-6. DOI:10.1034/j.1600-0560.2002.290402.x · 1.56 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Diffuse plane normolipaemic xanthomatosis is a well-defined uncommon condition characterized by yellow-orange plaques involving the eyelids, neck, upper trunk, buttocks and flexural folds. Over half of the reported cases are associated with lymphoproliferative disorders. The condition may arise as a result of perivascular deposition of lipoprotein-immunoglobulin complexes. We present a female patient with clear clinical features of diffuse plane xanthomatosis associated with chronic lymphatic leukaemia and monoclonal gammopathy. In this patient, we found evidence of complement consumption supporting the hypothesis that complexes of lipoprotein and paraprotein are formed. Lipid electrophoresis of fasting serum revealed the presence of a faint precipitate at the application slot, also indicative of the presence of protein-lipid complexes.
    Dermatology 02/2002; 204(4):351-4. DOI:10.1159/000063384 · 1.69 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: We describe a form of diffuse, rapidly self-healing xanthomatosis with the clinical and ultrastructural features of hyperlipemic xanthoma but occurring in a subject with normal lipid metabolism and with no associated systemic disorders.
    Archives of Dermatology 12/1986; 122(11):1294-7. DOI:10.1001/archderm.122.11.1294 · 4.31 Impact Factor
Show more