[Chronic sclerodermiform syndrome disclosing subcutaneous T-cell lymphoma]. / Syndrome sclérodermiforme chronique révélateur d'un lymphome T sous-cutané.

INTRODUCTION: Subcutaneous tissue is an uncommon primary localization for T-cell lymphomas. Panniculitis with recurrent papulonodules are reported in most cases. CASE REPORT: We report a case in which small-cell pleiomorphic non-Hodgkin lymphoma developed initially in subcutaneous tissue followed by secondary systemic extension. The first clinical manifestation was a sclerodermiform syndrome involving the four limbs. DISCUSSION: This type of lymphoma appears to have a polymorphic clinical presentation. Physicians should be aware of this type of lymphoma and use immunohistological techniques for early diagnosis.

[Congenital skin defect and fetus papyraceus. A case]. / Absence cutanée congénitale et foetus papyracé. Une observation.

BACKGROUND: Congenital skin defect is an uncommon condition. The term of "congenital skin aplasia" should be avoided since the origin may not necessarily be congenital malformation. The scalp is involved in 80 p. 100 of the cases. CASE REPORT: We observed a congenital skin defect located exclusively on the trunk. The infant lacked wide areas of skin in symmetrical star-like configurations. DISCUSSION: The notion of a twin expulsed after 4 months gestation suggested the diagnosis of congenital skin defect and fetus papyraceus or group V congenital skin aplasia in the Frieden classification as recalled. This diagnosis must not be overlooked as the prognosis is good. Closure with atrophic skin usually occurs within a few weeks. The etiopathogenesis remains obscure.

[Digestive involvement in dystrophic bullous epidermolysis. Presentation of 2 cases and review of the literature]. / Atteinte digestive au cours de l'épidermolyse bulleuse dystrophique. Présentation de deux cas et revue de la littérature.

Congenital epidermolysis bullosa is an inherited disorder characterized by an anomaly of joint structures between epidermis and dermis. This anomaly involves different malpighian epithelium and particularly in the digestive chorioepithelial junction. Digestive and oropharyngeal manifestations include bullae, erosions and ulcerations that heal by fibrosis, leading to retraction and stenosis. We report two cases of esophageal stenosis, the first one in a young patient with a congenital epidermolysis bullosa of Hallopeau-Siemens and the second one, in a young woman with a Pasini variant. In the first case, the esophageal stenosis was treated by careful dilation. In the second case, the endoscopy induced hemorragic bullae and septicemia, which were treated with antibiotics and total parenteral nutrition.

Sarcoid reactions in cystic duct carcinoma.

A diagnosis of sarcoidosis was evoked in a 61-year-old man on clinical and histologic bases. Nevertheless, a bile duct carcinoma was disclosed in association with the discovery of generalized sarcoid-like granulomas. This is only the third time that such an association has been described. HLA-B8, DR3, and DRw52 antigens were found, suggesting that altered immunologic mechanisms could play a role in the pathogenesis of this sarcoid-like reaction.