We report on a 60-year-old overweight white woman who presented with an asymptomatic flat, hard, yellow-brown subcutaneous plaque on her right hip. A total excision was performed. Histopathologic examination showed all the major features of a necrobiotic xanthogranuloma (NX) involving the entire dermis and subcutis, including areas of necrobiosis with cholesterol clefts, granulomatous infiltrate with some bizarre giant cells, numerous Touton cells, foamy cells, lymphoid follicles with germinal centers, foci of plasma cells, and "Touton cell panniculitis." A laboratory investigation revealed only slightly increased titers of cholesterol, strong positivity of anti-Borrelia antibodies, and diffuse skeletal osteoporosis with fractures of seven vertebrae. After 4 years of close follow-up, the osteoporosis was improved, and there were no signs of paraproteinemia, malignancy, or new skin lesions. The authors suggest that this case could represent a solitary clinical variant of NX without paraproteinemia.

A 69-year-old woman presented with a 2-year history of a slowly growing tumour on the nose. The clinical diagnosis was basal cell carcinoma (BCC) and a complete excision was performed. Histologically, the tumour had the general features of a BCC but with sheets and nests of cells with granular cytoplasmic changes in the centre of the lesion. A pathological diagnosis of granular cell BCC was made. On immunohistochemical examination, the tumour cells were Ber-EP4, cytokeratin AE1/AE3 and cytokeratin CAM 5.2 positive but S100 protein negative. Only the granular cells were CD68 antigen (monoclonal antibody KP1) positive.

A 22-year-old female with morphea-like lesions, deep subcutaneous nodules and lipoatrophic areas of the skin on lateral aspects of the upper arms, on the breasts and on the buttocks is described. In 1990 a biopsy specimen obtained from a subcutaneous nodule showed hyaline necrosis of fat tissue; there were no epidermal changes. Direct immunofluorescence revealed granular deposits of IgM at the dermo-epidermal junction of the skin overlying the subcutaneous nodule. In a biopsy specimen taken at the onset of the disease in 1988, hyaline sclerosis of the deep dermis, follicular hyperkeratosis and vacuolar degeneration in the epidermis were described. There was weak positivity for antinuclear antibodies. The diagnosis of lupus erythematosus panniculitis (LEP) was made. Administration of chloroquine resulted in complete clearing of nodules in 3 months. The reported case demonstrates the difficulties in establishing the diagnosis of LEP in patients who present with subcutaneous disease, morphea-like lesions and who do not have other clinical or laboratory evidence of lupus erythematosus. The differential diagnosis of LEP and deep morphea is discussed.

This is a report on a stored lipid atypical ultrastructural pattern in skin samples of Fabry's disease expressed exclusively in the endothelium. The pattern consisted of intersecting short crescentic tightly packed membranes with a periodicity identical to that in classical ultrastructural variants. At low magnification the lysosomal aggregates of the material resembled "sunbursts" or aggregates of densely packed squirming villus-like structures. According to results of ultrastructural, lipid, and lectin histochemical analyses including analysis of the patients' blood groups, it could be concluded that it is just a variant physical state of the otherwise typical Fabry lipid. Its origin could be attributed to impeded formation (or maintenance) of larger lipid lamellae. It was found in great amounts in skin capillaries in 2 cases, and rarely in 5 additional cases. Knowledge of this atypical ultrastructural pattern is of practical significance because it could, if prevalent, cause diagnostic problems.