Squamous cell carcinoma complicating recessive dystrophic epidermolysis bullosa-Hallopeau-Siemens: a report of four cases.

BACKGROUND: Recessive dystrophic epidermolysis bullosa (RDEB), an autosomal-recessive genodermatosis, is one of the more severe forms of the epidermolysis bullosa dystrophica group, and is characterized by generalized blistering of the skin and mucous membranes. Cutaneous squamous cell carcinoma is one of the most serious complications of this disease. METHODS: We report four patients (three females and one male), two of whom were under 20 years of age, suffering from RDEB-Hallopeau-Siemens. RESULTS: All patients developed well-differentiated squamous cell carcinoma. No metastases were detected. All cases were treated surgically. Fatal evolution was noted in one patient. A second tumor was detected in another patient during the follow-up period. No further tumors or metastases were observed in the other patients. CONCLUSIONS: Regular clinical and histologic examination of any nodular lesions or non healing ulcers of all patients suffering from RDEB-Hallopeau-Siemens to detect an early malignancy is recommended.

[Clinical and histological study of lupoid cutaneous leishmaniasis (16 cases)]. / Etude anatomoclinique de la forme lupoïde de la leishmaniose cutanée (16 cas).

INTRODUCTION: Zoonotic cutaneous leishmaniasis (CL) is characterised by a major clinical polymorphism, especially the lupoid type. The aim of our study was to precise the anatomic and clinical particularities of this clinical form of CL. MATERIALS AND METHODS: The present work was a prospective 1-year study. In all patients, the epidemiologic and clinical characteristics were identified, together with those regarding disease progression. Standard cutaneous biopsies were carried out for all study patients. RESULTS: The lupoid form was observed in 15% of the patients. It was clinically characterised by a lupoid aspect with papular and squamous placard surrounded by a satellite papule, a short duration of disease progression (28 months in average) and a preferential localisation at the level of the face and the elbow, statistically proven. At the histological level, epidermic hyperplasia was found in 93.8% of the cases; an inflammatory polymorphic dermic infiltrate consisting of lymphocytes and plasmocytes was found in all patients; granulomas were noted in 50% of the cases, and isolated epitheloid cells were observed in the derm of 3 patients. Amastigotes were rarely observed. DISCUSSION: In our series, the lupoid form was characterised with a short and non chronic evolution and two preferential sites for the affection: the face and the elbow. At the histological level, the lupoid type of CL appeared characterized by a high frequency of granuloma, usually organized, and rare amastigotes. However, the histology of authentic lupoid forms can be non granolomatosic.

[Epidemio-clinical profile of skin cancer in southern Tunisia]. / Profil epidemio-clinique des cancers cutanes dans le sud Tunisien.

BACKGROUND: Skin cancer (SK) frequency is increasing all over the world. AIM: We report a clinical and epidemiological study of SK in the south of Tunisia through a 1476 cases series. METHOD: On the basis of a retrospective study, we report the clinical and epidemiological data collected from files of patients with skin cancers seen during a 24-year-period (1979-2002). RESULTS: Our series was composed of 1476 patients, with an incidence of 615 cases/year. The mean age was 55 years. Epithelial skin carcinomas were the most common (1288 cases, 87.3%). Basal cell carcinomas were found in 890 patients (69%) and squamous cell carcinomas in 398 (31%). The mean age was 60 years. The phototype III was the most common (52.8%) among these patients. Skin melanomas were found in 71 cases (4.8%). The mean age was 54 years with a female predominance (60.6 %). Kaposi sarcoma was found in 4.5% of patients. The mean age was 64.3% and a male predominance was found (80% of cases). Two patients with Kaposi sarcoma (33%) were HIV positive. Dermatofibrosarcoma protuberans was reported in 2% of patients and skin lymphoma in 1.7%. CONCLUSION: The mean age is relatively low in our series. The increase in the incidence of skin cancers is probably related to the climatic conditions, the ageing of the population and to the changes in the social, economic and cultural aspects in the country. The male predominance is changing toward an equal distribution between both sexes probably in relation to the present similarity in the life style and work conditions.

[Cutaneous manifestations of chronic hemodialysis. Prospective study of 363 cases]. / Manifestations cutanées chez les hémodialysés chroniques. Etude prospective de 363 cas.

OBJECTIVE: Chronic hemodialysis patients experience frequent and varied cutaneous manifestations, of often hypothetical pathogenesis. The aim of this work is to assess the prevalence and nature of these cutaneous lesions and discuss some pathogenic mechanisms. METHODS: This prospective study was conducted from 1 February through 30 April 1996. It included 363 hemodialysis patients in seven centers. Each patient was interviewed and examined. RESULTS: There were 210 men and 153 women. Their mean age was 50.6 years. The total duration of hemodialysis ranged from 1 to 192 months, with a mean of 52.4 months. 88% of the patients had cutaneous manifestations. Pruritus was reported by 44.8%. It appeared after hemodialysis began for 82.1% of them. Cutaneous xerosis was observed in 69%. Changes in pigmentation were observed in 17% of cases, primarily involving hyperpigmentation of photo-exposed areas. In 6 patients, skin, hair, and exoskeleton began to turn lighter 10 months to 8 years after hemodialysis. Follicular hyperkeratosis was observed in 15%. One patient had a confirmed case of perforating follicular dermatosis. Disorders of the exoskeleton (18.5% of cases) were represented mainly by thin nails and subungual hemorrhages. Other cutaneous manifestations included petechiae and ecchymoses (66%), folliculitis-type infections (9%), subcutaneous calcifications (2 cases), cutaneous pseudoporphyria (2 cases), and eczema around the fistula (11.5%), due essentially to locally-applied products. CONCLUSION: Cutaneous manifestations in chronic hemodialysis patients are frequent and polymorphous. While most have long been known, lightening of skin, in particular, others have been discovered more recently. Their pathogenesis is most often poorly elucidated. Treatment is often symptomatic. Dialysis and renal transplantation constitute the best treatment.