Cameo Extensive xanthelasma associated with anaplastic large cell lymphoma and hyperimmunoglobulin E syndrome.

A 57-year-old woman presented with a 6-month history of an extensively spreading, yellowish patch on the periorbital areas and cheeks. A diagnosis of hyperimmunoglobulin E syndrome had been made at the age of 22 years on the basis of an eczematous eruption, recurrent furunculosis, and a persistently elevated immunoglobulin E (IgE) level. Her past medical history revealed that she had suffered from numerous recurrent bouts of chronic sinusitis, otitis media, oral candidiasis, orbital cellulitis, acne rosacea, and pneumonia caused by cytomegalovirus since her twenties. In addition, 1 year ago, anaplastic large cell lymphoma of the cervical lymph node (stage IIIb) developed, and she received six cycles of cyclophosphamide–doxorubicin–vincristine–prednisolone (CHOP) chemotherapy with partial remission. None of her family had any of these problems. Cutaneous examination showed extensive, symmetric, noninfiltrated macular areas of distinct yellow discoloration around the eyes and on both cheeks ( ). There were also erythematous papulonodular eruptions on the nose and both cheeks, which were thought to be acne rosacea. Laboratory findings were normal, except for an elevated IgE level (8157 IU/mL). Serum concentrations of IgG, IgA, and IgM were normal. Serum complement levels were normal, as evidenced by normal C3, C4, and CH50. Although she had a previous history of a decreased level (12%) of nitroblue tetrazolium (NBT) test (control, 53%), NBT test at our institute was normal. Neutrophil function tests, including neutrophil chemotaxis, neutrophil phagocytosis, neutrophil respiratory burst, and neutrophil microbial killing test, by flow cytometry, showed normal results. The serum lipid levels, including total cholesterol, triglyceride, low-density lipoprotein-cholesterol, and high-density lipoprotein-cholesterol, were normal. Serum lipoprotein electrophoresis was normal. A biopsy specimen revealed scattered foamy cells throughout the dermis. The larger clusters of foamy cells tended to group around the blood vessels of the dermis ( ). [ABSTRACT FROM AUTHOR]

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