Pemphigus vulgaris presenting as an isolated crusted plaque of the cheek.

A healthy 45-year-old man presented with a three-month history of an isolated enlarging eroded plaque on the left cheek. Pemphigus vulgaris was diagnosed by biopsy and positive direct immunofluorescence. The patient was lost to follow up, but returned two months later with generalized cutaneous involvement without mucosal involvement.

New mutations of Darier disease in Tunisian patients.

Darier's disease (DD, MIM 124200) also known as Darier-White disease and keratosis follicularis, is a rare autosomal dominant skin disorder characterized by warty papules and plaques in the seborrheic area (central trunk, flexures, scalp, and forehead). Pathogenic mutations in the ATP2A2 gene encoding the sarcoplasmic/endoplasmic reticulum Ca(2+) ATPase (SERCA) 2 gene underlie the disease. In the present study, we performed genetic investigation of three unrelated Tunisian families affected by DD.

Erythematous nodule of the eyelid.

A 68-year-old woman had a 15-day history of a well-circumscribed, erythematous nodule of the left eyebrow/eyelid area. After excision a primary cutaneous large B-cell lymphoma was diagnosed. The tumor was excised with clear margins and the patient has shown no recurrence over two years.

[Sneddon-Wilkinson's subcorneal dermatosis: an atypical case pustular].

Background: Sneddon-Wilkinson disease (SW) is chronic and benign disease belonging to the heterogeneous spectrum of neutrophilic diseases. It is characterised by flask, superficial, aseptic pustules, mainly involving the trunk, axilla and inguinal folds, favourably responsive to sulfones.

Aim: We report an original case with an atypical clinical presentation and course.

[Plexiform fibrohistiocytic tumor: a low grade malignancy lesion].

The plexiform fibrohistiocytic tumor is rare. It affects mainly children and adolescent. This tumor occurs at the dermo-subcutaneous region of the upper extremities.

[Sweet's syndrome: a propos of 8 cases].

Background: Sweet's syndrome is a dermatosis classically determined by painful erythematous plaques or nodules associated with fever, neutrophilia and dense neutrophilic dermal infiltrate. In most cases, Sweet's syndrome may occur in the absence of other diseases. However, it can be associated with an inflammatory disease and essentially with malignant conditions in 20% of cases requiring a careful investigation.

[Prospective study of 420 biopsies realised in patients with duodenal ulcer with positive Helicobacter pylori].

It's a prospective study leaded between September 1997 and july 1999 (23 months ) in 75 patients with duodenal ulcer and positif for Helicobacter pylori. All patients had a first endoscopy with antral, fundic and duodenal biopsies, followed one month later by a second control fibroscopy with biopsies of the same sites. A total of 420 biopsies was realised.

[Chondroid syringoma].

This is a retrospective monocentric stydy of all the cases diagnosed at our department over a period of 10 years. The study included seven male patients. All patients presented with a single painless skin lesion of about 1,5 cm on the face, lasting for 14 months on average.

[Gastrointestinal stromal tumors. A study of 5 cases].

GI stromal tumors are mesenchymatal tumors arising primarily from the digestive tract or from the omentum. Despite their rarity (less than 1% of digestive tumors). These tumors have become of current interest since the discovery of a treatment for metastatic and locally advanced tumors, the imatinib (Glivec).

[Genital lichen sclerosus].

Lichen sclerosus is a chronic inflammatory mucocutaneous disease which origin remains unknown. Its prevalence ranges from one in 300 to one in 1000 of all patients referred to a dermatology clinic in the seventeenth. Through the analysis of a hospital survey, we outline the epidemio-clinical aspects of this dermatosis.

[Angiomatoid fibrous histiocytoma: rare tumor of intermediate malignancy].

Angiomatoid fibrous histiocytoma is a rare tumour affecting young adults. Unlike conventional malignant fibrous histiocytoma, its extension is only local thus giving a good prognosis. We report the cases of a 9 year-old girl and a 16 year-old boy presenting respectively, with an axillary tumour 5 cm of diameter and a paravertebral subcutaneous tumour 1.

[Multicentric extrahepatic bile ducts carcinoma. Clinical presentation, pathologic features and management].

Extrahepatic bile ducts carcinoma (EHBC) arises usually in the upper third of the biliary tract. They are rarely multicentric associated or not to a gallbladder carcinoma. We report the case of a 53 years man operated for a head pancreas.