[Another case of the rare complications of chronic lymphocytic leukemia: angioedema]. / Encore un cas de complication rare des leucémies lymphoïdes chroniques : l'œdème angioneurotique.

Angioedema is a rare but may be serious (laryngeal edema). This is a recurrent edema, subcutaneous and/or submucosal, whose cause is a hereditary or acquired deficiency in C1 inhibiteur (C1 inhibitor fraction of complement). We present the case of a 56 years old patient who showed recurrent episodes of swelling of the face and hands in association with chronic lymphocytic leukemia stage A. The exploration of the complement pathway has allowed retaining the diagnosis of acquired angioedema type I. The association of angioedema and lymphoproliferative syndrome is rare; we present this interesting case to discuss it from the literature data.

[Idiopathic capillary hyperpermeability syndrome revealing of Waldenström disease]. / Un syndrome d'hyperperméabilité capillaire idiopathique révélant une maladie de Waldenström.

Waldenstrom disease is a rare hematologic disorder characterized by lymphoplasmacytic proliferation associated with the production of monoclonal IgM. Visceral injuries are described but some are rare (lung), others never reported (cardiac). We report for information and discussion a case representing these particular situations, considering that these attacks were revealing. It is a 63 year old man who was admitted to the emergency room in an array of tamponade, with edema at the front and four members. Clinical and radiological examinations were objectified bilateral pleural effusion, ascite and pericarditis. The biological exploration showed pancytopenia, serum proteins 120 g/L and a monoclonal peak migrant beta2 globulin electrophoresis which is made by monoclonal immunoglobulin M (IgM kappa). The bone marrow confirmed the diagnosis of the Waldenström disease. This is a mode of revelation never described before. Considering this case, it would be wise to think of a Waldenström disease before any polyserositis.

[Hypokalemic paralysis revealing Sjögren's syndrome associated with auto-immune thyroiditis]. / Quadriparésie hypokaliémique révélant un syndrome de Gougerot-Sjögren associé à une thyroïdite auto-immune.

We report a case of 36-year-old woman, admitted for hypotonic tetraparesis. Laboratory tests revealed severe hypokalaemia, acidosis, hyperchloremia and alkaline urinary pH allowing the diagnosis of distal tubular acidosis. Additional investigations led to the diagnosis of primary Sjögren's syndrome associated with Hashimoto's thyroïditis. The evolution was favorable under potassium citrate alkalinisation, the corticosteroid therapy and hormonal substitution. Based on this observation, the pathogenesis of distal tubular acidosis during auto-immune diseases (Sjögren's syndrome, monoclonal hypergammaglobulinemia, hypothyroidism) was discussed as well as its consequences and management.

Leflunomide-induced toxic epidermal necrolysis in a patient with rheumatoid arthritis.

INTRODUCTION: Leflunomide is an immunomodulating agent with proven efficacy in rheumatoid arthritis. Although its overall safety profile is good, a few cases of toxic epidermal necrolysis have been reported. CASE REPORT: This 36-year-old woman had rheumatoid arthritis that proved refractory to sulfasalazine and methotrexate, which were used successively in combination with symptomatic drugs. Leflunomide was started. A maculopapular rash and a fever developed 2 weeks later. The skin lesions spread rapidly to most of the body, and ulcers of the ocular and oral mucosa appeared. Leflunomide was stopped. Cholestyramine washout and prednisolone (60 mg/day) were given. The skin lesions healed over the next month. Punctate keratitis with keratinization of the cornea led to complete loss of vision. DISCUSSION: The main adverse effects of leflunomide consist of diarrhea, nausea, liver enzyme elevation, hypertension, alopecia, and allergic skin reactions. A few cases of severe skin reactions such as toxic epidermal necrolysis have been reported. They require immediate discontinuation of the drug and a washout procedure to hasten drug elimination from the body. CONCLUSION: Close monitoring for severe skin reactions is in order when using leflunomide.

Buschke-Ollendorff syndrome.

UNLABELLED: Buschke-Ollendorff syndrome (BOS) is an autosomal dominant disorder characterized by elastin-rich hamartomas and osteopoikilosis. CASE REPORT: In a 21-year-old woman, osteopoikilosis led to the diagnosis of BOS. She had multiple, grouped, buff-colored papules over the thighs and trunk. There was no pain or pruritus associated with the skin lesions. Examination of a biopsy specimen from a papule showed thick uniform collagen fibers and normal numbers of broad interlacing elastic fibers. DISCUSSION: BOS is a rare disease that affects 1/20,000 population. The diagnosis rests on a thorough physical examination and careful examination of radiographs. BOS must be distinguished from other bone abnormalities such as sclerotic bone metastases, particularly when osteopoikilosis is the inaugural manifestation.

Sweet's syndrome in Behçet's disease.

UNLABELLED: Few cases of Sweet's syndrome have been reported in patients with Behçet's disease. CASE REPORT: A 47-year-old woman with five year history of oral and genital ulcers that had not been investigated previously was admitted for an eruption of tender, erythematous, vesicle-like papules over the face, neck, palms, and legs. She reported polyarthralgia and weight loss of 6 kg over the previous month. At admission, she had a fever of 38.5 degrees C and conjunctivitis. Joint tenderness to mobilization without evidence of synovitis was noted. Laboratory tests showed inflammation and leukocytosis (12.5x10(9)/L) with 74% neutrophils. The skin biopsy was typical for Sweet's syndrome, and the pathergy test was positive. Glucocorticoid therapy 40 mg/day and colchicine were effective in alleviating the manifestations. DISCUSSION: Overlap exists between the clinical manifestations of Sweet's syndrome and Behçet's disease. Data from the literature suggest that Behçet's disease may be among the conditions that underlie Sweet's syndrome.