Primary cutaneous follicle center lymphoma responsive to interferon alfa-2a.

Follicular lymphoma is the most common type of primary cutaneous B-cell lymphomas with a predilection for the scalp, forehead, and trunk. Herein we report a case of primary cutaneous follicle center lymphoma on the scalp of 72-year-old female. The diagnosis was made histopathologically, confirming the presence of centrocytes and centroblasts. Complete resolution was achieved following administration of subcutaneous interferon α-2a at a dose of 4.5 × 106 IU three times weekly for 3 months.

Cutaneous CD4+/CD56+ hematodermic neoplasm.

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK cell lymphoma, is a rare and aggressive neoplasm with a high incidence of cutaneous involvement, risk of leukemic dissemination and poor prognosis. The characteristic features are expression of the T helper inducer cell marker CD4 and the NK-cell marker CD56 in the absence of other T cell or NKcell specific markers. Because of the rarity of this disease, we describe a 48 year old woman suffering from CD4+/CD56+ hematodermic neoplasm on her cheek without leukemic infiltration.

Periumbilical perforating pseudoxanthoma elasticum.

A 58-year-old, gravida 6, obese woman presented with a pruritic yellowish plaque around the umbilicus, which first appeared about 3 years ago. She also had flat yellow papules on the axilla and neck. After a burn from a heating device, a few perforating papules and a violaceous hue occurred on the plaque. Histological examination revealed pathological elastic fibers with a keratotic plug and perforation in the deep dermis, which was consistent with perforating pseudoxanthoma elasticum. This case indicates that perforating pseudoxanthoma elasticum, a variant of hereditary pseudoxanthoma elasticum may perforate due to mechanical factors.

Detection of pemphigus autoantibodies in healthy relatives of Turkish patients with pemphigus.

BACKGROUND: Pemphigus autoantibodies have been reported in healthy relatives of pemphigus patients suggesting a genetic predisposition in the pathogenesis of the disease. AIMS: To test for the presence of pemphigus autoantibodies in healthy relatives of Turkish patients of pemphigus. METHODS: The study group comprised 45 pemphigus patients, 75 unaffected family members and 47 healthy individuals in the control group. Direct and indirect immunofluorescence techniques were performed to determine the presence of pemphigus autoantibodies. RESULTS: By indirect immunofluorescence staining, circulating pemphigus autoantibodies were found in 26.7% of the relatives and in only two of the controls (P value = 0.0001). A direct immunofluorescence technique revealed positive results in three (4%) of the relatives and none of the controls. CONCLUSION: The presence of pemphigus autoantibodies in clinically healthy relatives indicates that genetic predisposition is necessary but not sufficient for the development of clinical disease.

Intertriginous bullous morphea: A clue for the pathogenesis?

Bullae occurring in lesions of morphea are uncommon. The cause of bullae formation in morphea is multifactorial, although lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Bullous morphea may be confused clinically with lichen sclerosus et atrophicus since both diseases may cause bullae in sclerodermatous plaques. A 69-year-old woman presented with a history of generalized morphea diagnosed 9 years earlier; and a 1-month history of pruritic bullae on her inframammary folds, axillary regions, lower abdomen, upper extremities and inguinal folds. Physical examination revealed multiple erythematous erosions, hemorrhagic vesicles and eroded bullae with slight scale or crusts overlying hypopigmented, indurated, shiny plaques. Skin biopsy revealed prominent edema in the papillary dermis, resulting in bulla formation and thickening of collagen fibers within the dermis. Direct immunofluorescence was negative. According to histologic and clinical features, the diagnosis of bullous morphea was established.