A 43-year-old African-American male presented with a 35-year history of multiple, slowly enlarging, subcutaneous nodules, which restrict his joint motion and are frequently painful. On examination, multiple ivory colored, firm, but mobile nodules overlie the dorsal aspects of the proximal and distal interphalangeal joints and multiple metacarpal phalangeal joints in the hands (fig. ). Nodules are also noted over the extensor surfaces of the knees (fig. ), Achilles tendons, right elbow (fig. ), sole of the feet, and the helix, scapha, and anti-helix of the ears (fig. ). He has had surgical removal of the nodules in the past, but they recur within a few years. Laboratory tests were remarkable for a total cholesterol of 518 mg/dl (13.4 mmol/l).
A 62-year-old man was admitted to our department due to a gradually evolving disseminated papulonodular eruption on face, flexors, and trunk for 30 years ago. On examination, multiple, well-defined, yellowish brown papules and nodules and tumor-like lesions were seen symmetrically on upper and lower eyelids (). Such papules also were observed on the cheeks and perioral region. On the anterior of the neck the lesions were confluent and formed a diffused plaque ().\nHundreds of red to brown papules were distributed symmetrically on axilla, genitalia, poplitea, and trunk, some of which are confluent together and formed plaque or tumor-like lesions (Figures and ). There were also some papule and nodules on nasal mucosa ().\nHe had no history of polyuria or any previous medical history.\nAnalysis at that point did not reveal any abnormalities in urine osmolality. Laboratory findings revealed a white blood cell count of 10,300 cells/mL containing 74% neutrophils. C-reactive protein was 7 mg/dL (normal values of 0.5 mg/dL).\nFasting cholesterol was 148 (normal values up to 200) and triglyceride was 141 (normal values up to 150).\nChest X-ray was normal.\nMagnetic resonance imaging of the brain and pituitary revealed no abnormalities.\nHistopathologic examination of skin biopsies showed a dense dermal diffuse histiocytic infiltration interspersed with mixed inflammatory cells and giant cells (Figures and ). Abundant foam cells were also seen (). A neural infiltration was not present. Immunohistochemistry was positive for CD68 (KP1) () and negative for S-100 protein.