Features:
(1) xanthogranulomas involving the trunk, flexures and periorbital region
(1a) plaques and subcutaneous nodules
(1b) tendency to ulcerate over time
(1c) may be yellow to brown in color
(2) presence of a lymphoproliferative disorder, often a plasma cell dyscrasia
(3) variable ocular symptoms: keratitis , scleritis, episcleritis, uveitis
(4) variable hepatosplenomegaly
(5) variable myocardial involvement
(6) variable mucosal involvement
Histologic features of skin lesions:
(1) palisading granuloma
(2) foreign-body and Touton giant cells
(3) cholesterol clefts
(4) necrobiosis
(5) foam cells
Laboratory findings:
(1) paraproteinemia
(2) variable cryoglobulinemia
(3) elevated erythocyte sedimentation rate (ESR)
(4) variable anemia and leucopenia
(5) low serum complement concentrations
