[Venous ulcer on the hand due to a treatment-related arteriovenous fistula]. / Ulcère veineux de la main secondaire à une fistule artérioveineuse thérapeutique.

BACKGROUND: An arteriovenous fistula of the arm for hemodialysis needs to last long and provide easy access for puncture. CASE-REPORT: A 50 year-old woman, with type 2 diabetes complicated by chronic renal failure, presented with an ulcer on the dorsum of the right hand that had developed over the past year. Humeral artery to basilica vein, side-to-side, arteriovenous fistula in the right arm was created in 1996. In 1999, she received a renal transplant. In 2002, she developed a deep ulcer on the dorsum of the right hand that progressed over one year, without improvement good local treatment. Doppler echography and a fistulography revealed proximal stenosis of the basilica vein, and a less restricted distal stenosis before the shunt. A venous ulcer on the dorsum of the hand due excessive venous pressure in the draining area. DISCUSSION: The complications of arteriovenous fistulas can be severe. Arterial stenosis is frequent and is the consequence of intimal hypertrophy. Chronic ischemia symptoms can be observed, but the hemodynamic loss is usually asymptomatic. When clinical signs are noisy emergency surgery is required. Venous stenosis is responsible for ischemia through venous overload, leading to edema of the arm, and rarely to venous-ulcer type trophic disorders as seen in this patient. Screening for stenosis on the vascular vein network must be systematic before creating an arteriovenous fistula.

[Neutrophilic dermatosis associated with propylthiouracil-induced p-ANCA (p-antineutrophil cytoplasmic antibodies)]. / Dermatose neutrophilique associée à des anticorps anticytoplasme des polynucléaires neutrophiles (p-ANCA) induits par le propylthiouracil.

INTRODUCTION: We report on a patient who progressively developed polymorphic expressions of neutrophilic dermatosis (Sneddon-Wilkinson subcorneal pustulosis and pyoderma gangrenosum) associated with p-antineutrophil cytoplasmic antibodies (p-ANCA), while receiving propylthiouracil for hyperthyroidism. To our knowledge, such associations have never been published so far. CASE-REPORT: A 40 year-old woman was treated with propylthiouracil for Graves'disease. After 16 months of therapy, she noted flares of pustular lesions surrounded with erythematous halo mainly localized on the trunk. The lesions became chronic, and were not improved by potent topical corticosteroids. When first seen in our department in February 2003, the eruption was typical of Sneddon-Wilkinson subcorneal pustulosis. This diagnosis was confirmed by the histological examination of a skin biopsy of a pustule. One month later, she developed an inflammatory progressively ulcerative lesion on the right ankle, typical of pyoderma gangrenosum. The diagnosis was confirmed by the histological examination of a skin biopsy taken on the evolving border of the lesion and showed polynuclear neutrophilic infiltration without vasculitis. Direct immunofluorescence was negative. The presence of serum anti-myeloperoxydase p-ANCA was known for this patient since October 2002. No IgA monoclonal gammapathy was revealed on extensive biological check-up. Systemic oral corticosteroid therapy (1 mg/kg/day) dramatically improved skin lesions with complete healing within 8 weeks. DISCUSSION: Propylthiouracil is well known to induce the occurrence of ANCA in 20 to 64p. 100 of patients treated for Graves'disease. The mechanisms involved are badly recognized so far. Cutaneous vasculitis, glomerulonephritis and polychondritis may be clinically associated with those antibodies. Rare observations of neutrophilic dermatosis, mostly Sweet's syndrome, have been described in patients with propylthiouracil-induced ANCA. One case-report described a 44 year-old woman who developed pyoderma gangrenosum associated with propylthiouracil-induced p-ANCA. These manifestations usually appear within 2 years, as our patient. The data in the literature, allows us to report the polymorphic expressions of neutrophilic dermatosis in this patient with p-ANCA which could be related to propylthiouracil. Such association of Sneddon-Wilkinson subcorneal pustulosis and pyoderma gangrenosum with p-ANCA has never been described in this endocrinologic context so far. Furthermore we propose that neutrophilic dermatosis should be inscribed in the list of side effects induced by propylthiouracil therapy.

[Lymphangioma with epithelial hyperplasia included in a Bardet-Biedl syndrome]. / Lymphangiome avec hyperplasie épithéliale associé à un syndrome de Bardet-Biedl.

INTRODUCTION: The Bardet-Biedl syndrome is a rare autosomal recessive disorder, which associates obesity, pigmentary retinopathy, hexadactyly, hypogenitalism, renal dysfunction and mental retardation. Other abnormalities can be observed in the Bardet-Biedl syndrome, but few cutaneous abnormalities have been described. CASE REPORT: A 41 year-old woman, suffering from a Bardet-Biedl syndrome diagnosed when she was 7 Years old, presented with an atypical pseudo verruca-like, dark red lesion of the interbuttock area that had developed over fifteen Years and had become a handicap. The histological examination revealed a double component: epithelial, papillomatous and acanthosic on the one hand and vascular and lymphatic on the other, suggesting a lymphangioma with epidermal hyperplasia. Magnetic resonance imaging of the sacral area revealed a median subcutaneous lesion, extending deeply to the third coccygial vertebra. DISCUSSION: Such a lymphangioma is unusual. Because it occurred during a rare polymalformative syndrome, we suggest that it may represent a new clinical sign that can be observed during the Bardet-Biedl syndrome.