Sweets syndrome: about of 8 cases

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. We report herein, the epidemiological, clinical, therapeutic and evolutive features of a hospital serie. We retrospectively reviewed all the files of patients conforming with the diagnosis of SS, seen at the dermatology departement of Habib Thameur hospital during a 7-year period [from 1997 to 2003]. All patients fulfilled at least two major criteria and two minor criteria of Su et Liu's diagnostic criteria modified by Von Den Driesch. 8 cases of .Sweet's syndrome were diagnosed. All patients were females. The mean age was 51.62 years. The lesions occurred on the upper limbs in 7/8 cases. SS was isolated in 6 cases and para-inflammatory in one case corresponding to an associated Sjogren's syndrome. Besides the st and ard therapy using oral corticosteroids [3 patients], non steroidal inflammatory agents has been efficient in 4 cases. In our serie, we report an exclusive female involvement and a lower frequency of associated diseases compared with the relevant literature. Association of SS and Sjogren's syndrome is exceptional. To our knowledge, only 6 cases have already been reported

[Sneddon wilkinson's subcorneal pustular dermatosis: an atypical case]

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. We report an original case with an atypical clinical presentation and course. A 25-year old women presented with an erythematous pustular eruption appeared one week ago. Similar flares were reported since 3 years. No drug intake history nor recent infection were reported. Physical examination showed superficial pustules mainly located on the face and limbs, lying on an erythematous skin and sometimes grouped in larges plaques with a serpiginous disposition having a characteristic aspect of hypopion pustule. A specimen taken from the pustules did not show bacterial or fungal infection. Histological examination of a biopsy specimen showed subcorneal pustules with a dermal peri-capillar neutrophilic infiltrate. Direct immunofluoresence was negative. No associated diseases were found. The patient was resistant to a 1 month treatment with dapsone [100mg/day]. Improvement was obtained with acitretin [25mg/day] within 3 months. Our case has some specificities: cephalic involvement, resistance to dapsone and favourable outcome to a low dose of acitretin

[Prospective study of 420 biopsies realised in patients with duodenal ulcer with positive Helcobacter 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 months later by a second control fibroscopy with biopsies of the same sites. A total of 420 biopsies was realized. Chronic gastritis was evaluated according to sydney system. Patients was divided by randomization in 4 groups. Every group was received a different therapeutic association. The results was conform to liberation concerning activity 80%, intestinal metaplasia 12%, inflammation 100%. Atrophy was observed in 56% of cases, this percentage is variable in literature; chronic gastritis was predominant in antre relatively to fundus [p< 0.005]. After treatment, a significative fall of Helicobacter pylori and activity and atrophy was established, contrariety to intestinal metaplasia and chronic inflammation witch are persisted. The prevalence of follicular gastritis was 57%. The better rate of ulcer cicatrisation and Helicobacter pylori eradication was respectively of 79% and 66% in group 1 treated by omeprazol, amoxicillin, metronidazol by comparison with the others 3 groups [p< 0.005]

Lichen sclereux genital

Lichen sclerosis 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 seventeeth. Through the analysis of a hospital survey, we outline the epidemio-clinical aspects of this dermatosis. Over a 19-year period [1984-2002], we have conducted a retrospective and monocentric study of all patients with genital lichen sclerosis were examined at the dermatology department of Habib Thameur hospital. Thirty-four patients suffered from lichen sclerosis. There were 33 female and only one male [sex-ratio: 0.03]. All patients underwent topical corticosteroid therapy [level I, II or IV]. The recovery rate of lichen sc1erosus was about 20% [7/34]. An epidermoid carcinoma occurred in three patients. The frequency of lichen sc1erosus in our study is estimated at 1,8 new cases per year. This frequency is probably under-estimated because of some patients' reluctance to seek help. A relatively low recovery rate of genital lichen sclerosis was found in our study. This may be related to an inadequate follow up added to an insufficient treatment adherence

[Gastrointhstinal stromal tumors. a study of 5 cases]

G1 stromal tumors are mesenchymatal tumors arising primarily from the digestive tract or from the omentum. Despite their rarity [less than I% of digestive tumors]. These tumors have become of current interest since the discovery of a treatment for metastatic and locally advanced tumors, the imatinib [Glivecr]. In this study we report 5 cases of stromal tumors. Patients were 42 years old an average. Abdominal mass was the revealing signal in 80% of cases, localisation was obtained by endoscopy and ultrasonography in 60% cases. Total resection of the tumor was performed in all patients. Immuno-histochemical examination confirmed the diagnosis of stromal tumors. Based on these cases and on a review of literature, we insist on the diagnostic difficulties of this rare pathology, while trying to determine the histological prognostic parameters and the latest therapeutic methods

[Chondroid syringoma]

This is a retrospective monocentric study 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. In all cases, treatment was surgical and the outcome satisfactory. Chondroid syringoma is a benign sweat gland tumor. This lesion is electively located in the craniofacial area, made of a subcutaneous painless tumor measuring from 0.5 to 3 cm. Diagnosis can always be confirmed by histology which shows tubuloalveolar and gland like structures with two or more cuboidal cell lines in a fibroadipoid or chondroid stroma that is positively stained for Alcian blue. Mixed tumors have a slow course; their progress to malignancy is rare but possible. The best treatment remains surgical allowing a histological diagnosis with a control of the lesion edges

Histiocytome fibreux angiomatoide: Une tumeur rare a malignite intermediaire

Angiomatoid fibrous histiocytoma is a rare humour affecting young adults. Unlike conventional malignant fibrous histiocytoma, it's extension is only local thus giving a good prognosis. We report the cases of a 9 years-old girl and a 1 6 years-old boy presenting respectively, with an axiliary tumour 5cm of diameter and a paravertebral subcutaneous tumour 1,5cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection