Iloprost administration in acrodermatitis of Hallopeau complicated by acquired toes syndactyly: a case report and review of the literature.

OBJECTIVE: Acrodermatitis Continua of Hallopeau (ACH) is a variant of pustular psoriasis often very difficult to treat. Secondary syndactyly, also called "pseudosyndactyly", is rare and can be a complication of burns, dystrophic epidermolysis bullosa or trauma. If left untreated, joint complications and definitive functional impairments may occur. CASE REPORT: We report a case of a 74-year-old man with acrodermatitis continua of Hallopeau involving the toes and complicated by syndactyly. ACH regression following Iloprost administration was also observed. DISCUSSION: Published studies are mainly limited to case reports only, due to the rarity of the disease. Therefore, there are no clear-cut therapeutic management guidelines available for this chronic and sometimes debilitating disease. ACH is often recalcitrant to the available therapies. Topical and systemic treatments have been described in literature with no long-lasting results. CONCLUSIONS: To our knowledge, this is the first report of foot syndactyly associated to ACH. In our patient, ACH symptoms regressed with Iloprost administration: this finding has never been previously described in literature. If confirmed by other clinical experiences, Iloprost could be a further therapeutic option in ACH.

Effect of ursodeoxycholic acid on inflammatory infiltrate in gallbladder muscle of cholesterol gallstone patients.

BACKGROUND: Reduced gallbladder (GB) contractility and chronic inflammatory changes in the mucosa have been reported in patients with cholesterol gallstones (GS). Ursodeoxycholic acid (UDCA) restores GB contractility and antagonises liver macrophage activation. In the colon, hydrophobic bile acid, not hydrophilic UDCA, induces mast cell degranulation. We studied the presence of monocyte/macrophage infiltrate, cyclooxygenase (COX)-2, inducible nitric oxide synthase (iNOS) expression, the number of total and degranulated mast cells in the GB muscle layer of cholesterol GS patients, and the effect of UDCA administration. METHODS: Gallbladder tissue was obtained from cholesterol GS patients, either treated or untreated with UDCA (10 mg kg(-1) day(-1)) for 30 days prior to surgery. Gallbladders removed for neoplastic diseases, not involving GB, were evaluated for control purposes. The presence of monocytes/macrophages (CD68 positive), granulocytes, and mast cells, and the COX-2 and iNOS expression, was determined immunohistochemically. KEY RESULTS: The number of CD68, granulocytes, mast cells, COX-2 and iNOS positive cells was significantly higher in the muscle layer of GS patients than in controls. Compared to untreated patients, those treated with UDCA showed significantly lower levels of CD68, COX-2 positive cells and degranulated mast cells and a lesser number of iNOS positive cells and granulocytes. CONCLUSIONS & INFERENCES: An inflammatory monocyte/macrophage, mast cell and granulocyte infiltrate is present in the GB muscle layer of GS patients. Ursodeoxycholic acid decreases macrophages, degranulated mast cells and COX-2 expression. These results suggest that monocytes/macrophages and degranulating mast cells contribute to muscle cell dysfunction in cholesterol GS patients and support the anti-inflammatory effect of UDCA.