[Alopecia areata during anti-TNF alpha therapy: Nine cases]. / Pelade au cours d'un traitement par anti-TNF alpha : neuf cas.

BACKGROUND: In recent years, a growing number of biological agents have been introduced for the treatment of various diseases, and their principal adverse events are known. We present nine cases of alopecia areata (AA) developed in patients treated with TNF-α blocking agents. PATIENTS AND METHODS: Nine cases are described: five men and four women of mean age 39.2 years (range: 29-54 years). Two patients had a past history of alopecia areata. The anti-TNF given was adalimumab (Humira(®)) in eight cases and etanercept (Enbrel(®)) in one case. The time lapse to development of AA following introduction of the anti-TNF alpha agent was between six weeks and eight months (mean: 4.2 months). There were five cases of patchy AA and four of AA universalis. Anti-TNF alpha treatment was stopped in all patients. Complete regrowth was seen in five patients. Two patients showed no improvement. In two patients, partial hair regrowth (<50%) was seen after systemic corticosteroid therapy and methotrexate. DISCUSSION: Our nine cases of alopecia areata developed in patients treated with TNF-α blockers constitute the largest series reported to our knowledge. 17 cases of AA during anti-TNF-alpha therapy have previously been described in the literature. AA may be a side effect of anti-TNF-alpha drugs. In our patients, no conclusive triggers could be associated with the development of AA, except a context of stress in four patients. Complete regrowth in three patients after discontinuation of the anti-TNF-alpha (without other therapy) is an additional argument in favour of the implication of biotherapies. However, a random coincidence of AA with anti-TNF-alpha cannot be completely ruled out. The role of anti-TNF-alpha therapy in the pathogenesis of AA is poorly understood. Activation of self-reactive T cells by anti-TNF-alpha could lead to the development of AA.

[Dorfman-Chanarin syndrome]. / Le syndrome de Dorfman-Chanarin.

BACKGROUND: Dorfman-Chanarin syndrome is an uncommon condition characterized by non-bullous congenital ichtyosiform erythrodermia and lipid vacuoles in circulating leukocytes. CASE REPORT: We report an unusual presentation in a child who had a dry congenital ichtyosiform erythroderma. Blood smears revealed lipid vacuoles in granulocyte cytoplasm, leading to the diagnosis of Dorfman-Chanarin syndrome. The child also had liver and ophthalmologic involvement. DISCUSSION: Dorfman-Chanarin syndrome is a rare autosomic recessive hereditary disease (27 cases reported in the literature) related to the accumulation of neutral lipids in organ tissues. Clinical manifestations are dry congenital ichtyosiform erythroderma and lipid vacuoles in circulating granulocytes. The syndrome may be expressed more or less severely in several organs. Diagnosis is confirmed on blood smears. The vacuoles can also be observed in smears of heterozygous subjects and can serve as a screening test. The pathogenesis of Dorfman-Chanarin syndrome is poorly understood but appears to be related to perturbed intracellular triglyceride catabolism. Treatment is symptomatic.