Ilana Avinoach

Gamma/delta T-cell lymphoma involving the subcutaneous tissue and associated with a hemophagocytic syndrome.

A case of gamma/delta-positive T-cell lymphoma localized primarily in the subcutaneous adipose tissue and associated with an extensive hemophagocytic syndrome and a rapid, fatal outcome is presented. Previous reports of gamma/delta cutaneous and subcutaneous T-cell lymphomas are reviewed. To define the characteristics of a T-cell subset of neoplastic cells bearing the gamma/delta receptor in a subcutaneous lymphoma, we undertook clinical and laboratory examinations, morphological evaluation of biopsies, electron microscopy, and extensive immunohistochemical studies. Immunohistochemical studies showed that the neoplastic cells expressed the gamma/delta T-cell receptor (CD3+, C gamma M1+) and were CD2+, CD43+, CD45+, CD45RO+, PCNA+, but were beta F1-, CD1-, CD4-, CD8-, CD15-, CD20-, CD25-, CD30-, CD45R-, CD57-, CD68-, Mac 387-, and HLA Dr-. To the best of our knowledge, this is the first report of a subcutaneous T-cell lymphoma of gamma/delta type involving the subcutaneous tissue and associated with a hemophagocytic syndrome.

Leukocytoclastic vasculitis - correlation between different histologic stages and direct immunofluorescence results

Background

Lupus vulgaris complicated by metastatic squamous cell carcinoma

A 47‐year‐old Bedouin man presented with an ulcerated nodule of several months’ duration on the nape of the neck. The nodule developed on an asymptomatic, slowly growing plaque which appeared during childhood. Physical examination revealed two erythematous plaques covering the posterior and right lateral aspects of the neck. The border of the plaques was soft, circinate, with a reddish‐brown color, while the center was slightly erythematous and atrophic. An ulcerated nodule measuring 2 cm was seen on one of the plaques ( Fig. 1 ). Physical examination was unremarkable with no lymphadenopathy. Laboratory tests, including complete blood cell count, erythrocyte sedimentation rate (ESR), and routine chemistry tests, were all within normal limits. Chest X‐ray showed a small calcified perihilar lymph node. The Mantoux test was positive with erythema and induration of 15 mm after 48 h. Biopsy from a plaque showed extensive diffuse granulomatous infiltration throughout the dermis with epithelioid and Langhans giant cells surrounded by mononuclear inflammatory cells. No caseation necrosis was present. Ziehl–Neelsen, periodic acid–Schiff (PAS), and Giemsa stains were negative. Polymerase chain reaction (PCR) for the detection of Mycobacterium tuberculosis and atypical mycobacteria from a skin sample was also negative. Fresh tissue cultures yielded M. tuberculosis after 6 weeks.

Stewart-Treves syndrome in a patient with elephantiasis

Angiosarcoma that develops in areas of chronic lymphedema is also called Stewart-Treves syndrome. It usually appears in areas of lymphedema several years after mastectomy for breast carcinoma. Only 10% of these angiosarcomas occur in areas of chronic lymphedema as a result of another cause. We present a patient with epithelioid angiosarcoma as a rare complication of elephantiasis.

Granulomatous rosacea associated with Demodex folliculorum.

A 25-year-old Israeli woman was examined in the dermatologic out patient clinic of the Soroka Medical Center due to an eruption of the face, which had been present for several months. The patient suffered from mild asthma, but was otherwise well. There was no history of systemic or locally applied medications or cosmetics. Physical examination revealed skin lesions symmetrically distributed on both cheeks, lower eyelids, and nose. The lesions were composed of erythematous papules 2 mm in diameter, which coalesced on the cheeks to form plaques, 2-3 cm in diameter, A few pustules were also present (Fig, 1), On examination of the eyes, blepharitis and an internal chalazion were noted. The rest of the physical examination was within normal limits. Laboratory findings including ESR, routine blood and urine analysis were within normal limits, A culture from the skin lesions for leishmaniasis was negative, as was the PPD test. Chest x-ray was witbin normal limits, Histologic examination obtained from the lesion on the cheek revealed enlarged hair follicles containing structures of Demodex folliculorum (Fig, 2), A dense Infiltrate composed of histiocytes with a few giant cells surrounded by lymphocytes and forming noncaseating foreign body granuloma was observed in tbe dermis (Fig, 3), PAS and acid-fast stains were negative. The patient was treated with minocycline HCI 100 mg/ day and topical clindamycin phosphate. The lesions healed completely, without scarring, after 2 months of treatment. Cessation of medications was not followed by recurrence of tbe skin lesions.

Hypopigmented Mycosis Fungoides in a White Female

A rare case of a young Caucasian female with hypopigmented mycosis fungoides is described. We reviewed and discussed the literature.

Leukocytoclastic vasculitis induced by low‐dose methotrexate: in vitro evidence for an immunologic mechanism

The rare occurrence of methotrexate (MTX)‐induced vasculitis has been associated mainly with high or intermediate MTX doses. We report herein a case of cutaneous leukocytoclastic vasculitis (LCV) following treatment with low‐dose oral MTX (7.5 mg/week) for rheumatoid arthritis. The histological findings of a cutaneous lesion were consistent with drug‐induced vasculitis. The clinical and histological findings, including the temporal relationship between MTX intake and the onset of vasculitis, and the results of withdrawal and rechallenge tests, suggest a causal relationship, and indicate a drug‐induced LCV due to MTX. The role of MTX in the induction of the vasculitis was further supported by a positive mast cell degranulation (MCD) test.

Diffuse lentiginosis in a patient with Werner's syndrome—a possible association with incomplete leopard syndrome

A classical case of Werners syndrome is described. In addition to the numerous skin changes that are typically associated with Werners syndrome, our patients also displayed diffuse lentiginosis, and several of the clinical features of leopard syndrome. Histopathological and ultrastructural findings from a hyperpigmented maculae displayed the typical features of a simple lentigo. A striking feature was the presence of melanosomes in Langerhans cells as has been reported in the leopard syndrome. A possible generalized mesodermal defect has been suggested in Werners syndrome, while the basic defect in the leopard syndrome is thought to be of neuroectodermal origin with pleiotropic changes in the organs derived from the mesoderm. Our patient, with incomplete leopard syndrome and typical Werners syndrome, may be an example of an association of genetic defects affecting both tissues of neuroectodermal and mesodermal origin.

Impotence in Systemic Sclerosis

Excerpt To the editor: Nowlin and associates (1) reported the cases of six patients with scleroderma who were impotent. The pathogenesis of impotence in scleroderma is not known, and the authors ra...

Solitary glomus tumour

A 42‐year‐old woman developed a purplish, tender nodule on her wrist of 6 months duration. Histological examination showed a well‐circumscribed lesion situated in the dermis and subcutis characterized by numerous, dilated thin‐walled vascular spaces surrounded by sheets of glomus cells. The histological findings are characteristic of the diagnosis of an uncommon type of glomus tumour, known as glomangioma.