[Two cases of proliferation of monoclonal and monotypic lymphocytes and plasma cells corresponding to acrodermatitis chronica atrophicans]. / Prolifération cutanée lympho-plasmocytaire monoclonale et monotypique révélant une acrodermatite chronique atrophiante : deux observations.

BACKGROUND: While a dermal proliferation of monotypic monoclonal lymphocytes and plasma cells suggests above all cutaneous marginal zone lymphoma (CMZL) or plasmacytoma, it may also correspond to a Borrelia infection of which the clinic picture is evocative, as demonstrated in the cases presented herein. PATIENTS AND METHODS: The files of two patients were submitted for discussion at the regional multidisciplinary staff meeting on cutaneous lymphomas after review of the skin biopsies led to a diagnosis of plasmacytoma and CMZL on the basis of infiltrate containing abundant plasma cells. The infiltrates of both patients showed a kappa monotypic light chain and cutaneous B-cell clones were detected. However, the clinical features, with monomelic maculopapular rash, were evocative primarily of Borrelia infection. Diagnosis was confirmed by positive serology and clinical cure was achieved after 3 weeks of oral tetracycline, without relapse. COMMENTS: A link between Borrelia infection and cutaneous lymphomas has long been thought to exist. Further, it is recommended that antibiotics be considered in CMZL before undertaking systemic therapy. The classic histological appearance of the tertiary phase of early-stage Lyme's disease shows perivascular and periadnexal infiltrate comprising lymphocytes and plasma cells. At the later stages, epidermal atrophy occurs with thinning of the dermis. The monoclonal and monotypic nature of skin proliferation points above all to CMZL or plasmacytoma. However, clinicopathological correlation is an essential step before such a diagnosis may be made. In the event of monomelic erythema, as in our patients, it is important to screen for Borrelia infection, which responds well to appropriate treatment.

[Pigmented Paget's disease of the male nipple]. / Maladie de Paget pigmentée du mamelon chez l'homme.

BACKGROUND: Mammary Paget's disease unfrequently occurs in males, and may be pigmented in rare instances. Differential diagnosis with malignant melanoma relies on immunohistochemical studies. CASE REPORT: A case of Paget's disease of the nipple in a 76 year-old male is reported, clinically mimicking a malignant melanoma because of massive pigmentation. Histologically, large Paget's clear cells were intermingled with numerous melanin-rich dendritic melanocytes. An underlying ductal carcinoma was found. After differential immunohistochemical staining, diagnosis of Paget's disease could be unequivocally substantiated since Paget's cells stained for epithelial markers, c-erbB-2 and hormonal receptors, whereas protein S100 and HMB45 were negative. DISCUSSION: Pigmentation in mammary Paget's disease occurs preferentially in males. Pigmentation results from numerous melanocytes with abundant melanin in close contact with Paget's cells. An increased number of melanocytes may also be observed in cutaneous metastatic breast carcinomas. It could result from a chemotactic factor produced by neoplastic cells.

Acral erythema in children receiving high-dose methotrexate.

Scant information is available concerning the occurrence and evolution of chemotherapy-induced acral erythema in children receiving intravenous high-dose methotrexate (MTX). Among 50 children with acute lymphoblastic leukemia or lymphoblastic lymphoma receiving a total of 203 courses of high-dose MTX (3-8 g/m2), 3 cases of acral erythema were observed. Painful erythema of finger and toe pads was noted in three children 3 days to 2 weeks after MTX infusion. The lesions resolved completely after blister formation and desquamation. These patients subsequently received high-dose MTX therapy without cutaneous problems. The present work points out that the chemotherapeutic schedule need not be modified in selected patients who develop acral erythema following high-dose MTX infusion.

[Peripheral neuropathies and chronic hepatitis C: a frequent association?]. / Neuropathies périphériques et hépatite chronique virale C: une association fréquente?

OBJECTIVE: The aim of this prospective study was to evaluate the prevalence of the peripheral neuropathies associated with chronic hepatitis C and their clinical, electrophysiological and histological characteristics. PATIENTS AND METHODS: Thirty six patients admitted from December 1994 to January 1996 for chronic hepatitis C were prospectively investigated. Laboratory data included liver blood tests, serum cryoprecipitate immunoelectrophoresis, assays for anti-nuclear antibody, rheumatoid factor, circulating immune complexes, anti-SSA and anti-SSB antibodies. For each patient, a clinical neurological evaluation as well as an electrophysiological evaluation were performed by the same operator. In presence of peripheral neuropathy, a neuromuscular biopsy was performed. RESULTS: In seven patients (19%), a peripheral neuropathy was diagnosed related to hepatitis C virus in 3 patients. In these 3 patients presenting with leg paresthesia, an axonal sensitive neuropathy was evidenced by electromyography. A neuromuscular biopsy performed in two of these patients showed a severe diminution of the myelinated fibers associated with vasculitis lesions in one patient. A skin biopsy was performed in the third patient with leg purpura revealing a leukocytoclasic vasculitis. A positive cryoglobulinemia was found in two of these patients. CONCLUSION: In chronic hepatitis C, the prevalence of peripheral neuropathy is 8% and usually associated with cryoglobulinemia. It is an axonal neuropathy with diminution of the myelinated fibers and vasculitis lesions in the absence of cryoglobulinemia.