[Erythema palmare hereditarium (Lane's disease): Report of a paediatric case]. / Érythème palmaire héréditaire (maladie de Lane) : observation chez un enfant.

BACKGROUND: Palmar erythema may be either congenital or acquired, hereditary or non-hereditary. Its diagnosis and management differs according to whether or not it is acquired, hereditary or associated with symptoms. Herein, we report the case of a child with hereditary palmoplantar erythema (Lane's disease). OBSERVATION: A 2½-year-old girl consulted for palmar erythema, present since birth, predominantly on the thenar and hypothenar eminences and on the palmar aspect of her fingers. She also presented mild diffuse erythema on the soles of her feet. Both her mother and grandmother had similar signs. The girl had neither impairment nor pain linked to this erythema, and she had no associated symptoms. Physical examination was otherwise normal, as were the results of standard laboratory tests. DISCUSSION: We diagnosed Lane's disease based on the girl's hereditary erythema with autosomal dominant transmission, and the congenital and asymptomatic nature of her palmoplantar erythema. Nine publications have reported cases of Lane's disease, but this condition is probably highly under-reported.

[Anti-p200 pemphigoid: Remission under mycophenolate mofetil (Cellcept®)]. / Pemphigoïde anti-p200: rémission sous mycophénolate mofétil (Cellcept®).

BACKGROUND: Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined. PATIENT AND METHODS: A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months. DISCUSSION: The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil. CONCLUSION: Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone.