Biomedical subjects
Publications and source records attributed to C Weiller-Merli.
We report 2 patients who had sarcoidosis with skin involvement associated with bilateral anterior uveitis. During the follow-up, they developed chronic myelomonocytic leukemia. This association is of particular interest as sarcoidosis and chronic myelomonocytic leukemia may be considered as disorders of a common monocyte-macrophage cell lineage.
BACKGROUND: Sicca syndrome after Stevens-Johnson syndrome is classically described. However, to our knowledge, authentic Sjögren's syndrome following epidermal necrolysis has not been described previously. CASE REPORT: A 36-year-old woman with Steven-Johnson syndrome developed transient hepatitis and a persistent sicca syndrome. Fourteen years later an authentic Sjögren's syndrome was identified with presence of antinuclear and anti-SSA antibodies and lymphocytes infiltration of the accessory salivary glands, i.e. grade IV disease in the Chisholm classification. DISCUSSION: The initial mucosal destruction observed in our patients may have laid the ground for Sjögren's syndrome via abnormal antigen presentation in a predisposed dysimmune context.
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Polysplenia syndrome includes a group of congenital abnormalities involving the spleen, gastrointestinal tract, liver, and cardiovascular system. A case of severe lower limbs lymphedema occurring in a young woman with polysplenia, azygous continuation of the inferior vena cava, short pancreas, and preduodenal portal vein is reported. Lower limb lymphedema could represent a new clinical manifestation associated with polysplenia syndrome. Lymphedema could be the result of a primary anomaly of the lymphatic system or be induced by high pressure in the venous system or by compression of the lymphatic circulation by the hypertrophic azygous vessels in the posterior mediastinum.
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We report the first case of generalized granuloma annulare occurring in a 65-year-old woman who was chronically infected with hepatitis C virus. Granuloma annulare totally regressed during alpha-interferon treatment. As chronic hepatitis C virus infection is frequent, a serodiagnosis would be of interest in patients who have generalized granuloma annulare. A link between these 2 diseases may be strongly suspected.
We report 2 uncommon cases of skin panniculitis (Pa) occurring during the course of relapsing polychondritis (RP). Skin histological findings were characterized by septal and lobular involvement associated with vasculitis. Chondritis and Pa lesions both regressed with steroid therapy and showed parallel clinical courses. To our knowledge, this type of cutaneous involvement has never been reported in RP.
INTRODUCTION: Anetoderma is a skin disease of unknown etiology sometimes encountered in patients with a lupus syndrome. We report a clinical and pathology analysis of 3 selected cases of anetoderma associated with systemic lupus erythematosus and/or antiphospholipid syndrome. CASE REPORTS: Three patients had skin lesions typical of anetoderma. Antiphospholipid antibodies were present in all three (anticardiolipin in 3, circulating lupus anticoagulants in 3, anti-beta 2GP1 in 2). One patient with systemic lupus erythematosus had signs of antiphospholipid syndrome, the two others had primary antiphospholipid syndrome. DISCUSSION: The analysis of our cases and a review of the literature suggest that anetoderma is a disease which develops in the immunological environment of lupus, and is apparently more related to the presence of antiphospholipids than lupus itself. Thus anetoderma could be another cutaneous sign of the antiphospholipid syndrome.
The antisynthetases syndrome associates lung disease, polymyositis, Raynaud's phenomenon and frequently polyarthritis. Anti-amioacyl-tRNA synthetase antibodies are present. The most common is anti-Jol. We report a case with severe arteritis of the finger with multiple zones of skin necrosis which responded favourably to corticosteroids given in bolus and cyclophosphamid. Finger arteritis, in addition to classical Raynaud's phenomenon, may be a new manifestation of the anti-synthetases syndrome.
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We report a 65-year-old patient who presented a rapid eruption of keratoses on sun-exposed areas and an explosive transformation into several squamous-cell carcinomas, occurring during the accelerated phase of a chronic granulocytic leukemia. Clinical findings resembled those usually described in xeroderma pigmentosum. The role of immunosuppression, chemotherapy and possible genetic predisposition is discussed.
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