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[Bullous localization of a lupus erythematosus induced by radiotherapy].

INTRODUCTION: Connective diseases induced or exacerbated by radiotherapy are not frequent. We report a particular erosive erythematosus lupus with lesions in the precise distribution of radiation therapy given for a breast cancer. OBSERVATION: An 80 year-old woman presented with painful erosive skin lesions of her breast which had been treated by irradiation 9 years before. The patient also had rheumatoid arthritis. A skin biopsy showed keratinocyte necrosis, acantholysis and a dermal lymphocytic infiltrate under the basal cell layer. Direct immunofluorescence showed granular deposition of IgG, IgM and C3 along the basal cell layer. Antinuclear antibodies were positive at a titre of 1: 1,000. Erythematosus lupus diagnosis was established and annular lesions secondarily appeared on her neck, back, and arms. A treatment with hydroxychloroquine and topical corticosteroïds was effective in 3 months. DISCUSSION: The unusual erosive lesions and their localization on a previously irradiated site suggest the role of X-rays in our observation, despite their late appearance after radiotherapy. Radiotherapy and erythematosus lupus affect the same target structures (basal cells and small dermal capillaries) and could have additive effects. The presence of rheumatoid arthritis in the past medical history may have exaggerated this complication.

Adrenal Cortex Hormones↗

Diffuse muscular haemorrhage as presenting sign of juvenile systemic lupus erythematosus and lupus anticoagulant hypoprothrombinaemia syndrome.

Lupus anticoagulants are closely related to systemic lupus erythemathosus (SLE) and to thrombotic events. We describe a 12 year-old girl with a bilateral intramuscular haemorrhage of the gastrocnemius muscles as her main initial presentation of juvenile SLE. Laboratory work-up revealed lupus anticoagulant-hypoprothrombinaemia syndrome (LAHS) with very low levels of factor II due to autoantibodies. She showed a good initial clinical and laboratory response to prednisone therapy, however steroid dependency developed. To the best of our knowledge, this is the first case reported of juvenile SLE presenting with LAHS.

Antiphospholipid Syndrome↗

[Oral lesions in systemic lupus erythematosus. I. The etiopathogenic aspects of lupus erythematosus].

The authors investigate lupus erythematosus systemicus, characterized by the multiform etiology and autoimmune pathogenesis. Cutaneous lesions, large confluent areas of erythema, poikilodermatous lesions, atrophy and necrotizing vasculitis (leukocytoclastic vasculitis) occur in patients with lupus erythematosus (SLE). Ulcerative lesions are an ACR (American College of Rheumatology), (formerly ARA), criterion for the diagnosis of SLE. These lesions are suspected of being caused by a mechanism involving circulating immune complexes and neutrophils. This observation further supports previous evidence that all oral lesions in SLE result from vacuolar degeneration of basal keratinocytes. Immunopathologic study of lupus erythematosus shows the correlation between HLA-dr positive keratinocytes or Langerhans cells and epidermotropic T cells and the immunobiological significance of the antigen-antibody system in LES.

Antibody Formation↗

Discoid lupus erythematosus lesions developed on lupus erythematosus profundus nodules.

We describe this case of LEP for its unusual way of presentation. It first appeared with a LEP pattern, followed by a typical DLE of the skin, overlying the nodules only. Moreover, our histologic findings showed the typical pattern of lymphocytic lobular panniculitis, with hyaline necrosis of fat, the lymphoid nodule, and even the lymphocytic nuclear "dust." The epidermal changes, with the liquefaction degeneration of the basal cell layer, a moderate follicular hyperkeratosis, and a perivascular and periappendeal lymphocytic infiltrate, were also observed in the abdominal lesion that developed last without clinically evident DLE.

Adult↗

Contemporary concepts for the clinical and laboratory evaluation of systemic lupus erythematosus and "lupus-like" syndromes.

Systemic lupus erythematosus (SLE) is a nonorgan-specific autoimmune disease which affects multiple organ systems and is multifactorial in etiology. SLE is the prototypic systemic rheumatic disease with immune dysregulation characterized by (1) polyclonal activation of B-cells and (2) production of a large spectrum of autoantibodies with a marked preference for nuclear and intracellular antigens. The clinical and laboratory manifestations and criteria for classification and diagnosis of systemic lupus erythematosus, lupus-like syndromes, and various subsets of systemic lupus erythematosus, are reviewed. The differential diagnosis of SLE and related diseases is described with correlation of specific intracellular autoantibodies.

Antibodies, Antinuclear↗

Occlusion of small hepatic veins associated with systemic lupus erythematosus with the lupus anticoagulant and anti-cardiolipin antibody.

We report the case of a woman with lupus anticoagulant-positive systemic lupus erythematosus who developed small hepatic vein occlusion. Since the age of 34, she had been known to have hepatomegaly. A definitive diagnosis of systematic lupus erythematosus was made eight years later. Histological evaluation of the liver biopsy specimen was not fully diagnostic of prominent hepatomegaly during this period. Occlusion of the small hepatic veins was confirmed by hepatic venography, but the lumen of the large hepatic veins showed a smooth appearance. The lupus anticoagulant and anti-cardiolipin antibody were both positive. Since a high incidence of thromboembolic diseases in patients with the lupus anticoagulant or anti-cardiolipin antibody has been reported, the presence of this type of anticoagulant may provide an explanation for hypercoagulability and subsequent development of hepatic vein thrombosis in this patient. This is the first report of a patient with systemic lupus erythematosus who developed an occlusion of small hepatic veins attributable to the lupus anticoagulant and anticardiolipin antibody. This case suggested that a systematic search for hepatic vein occlusion should be made in patients with systemic lupus erythematosus who have developed inexplicable hepatomegaly, especially in those with positive tests for the lupus anticoagulant and/or anti-cardiolipin antibody.

Adult↗

Lichen planus and coexisting lupus erythematosus versus lichen planus-like lupus erythematosus. Clinical, histologic, and immunopathologic considerations.

A middle-aged black woman presented initially with painful cutaneous plaques that were located at various sites and that were diagnosed histologically as lichen planus. Standard light microscopic examination showed histopathologic variants of lichen planus. Direct immunofluorescence of a skin lesion had negative results for any of the lupus erythematosus bands but did reveal hyaline bodies in the deep cellular layer of the epidermis and the superficial layer of the dermis. These findings were compatible with either lichen planus or lupus erythematosus. However, both the clinical course of the eruption and the antinuclear antibody tests showed that the immunofluorescent antinuclear antibody pattern of large, speckle-like threads were consistent with lupus erythematosus. Furthermore, the large, speckle-like, thready antinuclear antibody pattern, which has been shown to be a marker for a benign subset of lupus erythematosus, is not seen in lichen planus. Lichen planus--like lupus erythematosus was therefore the more likely diagnosis.

Antibodies, Antinuclear↗