[Mycetomas in central Tunisia]. / Les mycétomes en Tunisie (région du centre).

INTRODUCTION: Mycetomas are inflammatory pseudo-tumors containing fungal or actinomycosic-type grains. They are frequent in tropical and subtropical countries and unknown in Tunisia. PATIENTS AND METHODS: We conducted a retrospective study of 12 cases of mycetoma registered in the Dermatological department of the university hospital in Sousse (central Tunisia) over a period of 27 years, from 1974 to 2001. The diagnosis was confirmed by anatomopathological and/or mycological examination. RESULTS: The mean age at the onset was of 49 years and the sex ratio of 1. A notion of a traumatism was reported in two cases and eight patients had various agricultural activities. The mean duration of progression was of eight years. The localization was the foot in 10 cases. The mycetoma was of actinomycosic origin in 10 cases, due to Actinomadura madurae in nine cases, to Nocardia spp in one case and of fungal origin in 2 cases:Pseudoallescheria boydii in one case and Madurella mycetomi in the other. Antibiotic therapy was associated with surgical exeresis in nine cases and amputation in the other two cases. COMMENTS: Confrontation of our results with those of Tunisian series and a review of the literature, helped to specify the clinico-epidemiological characteristics and progression of mycetoma in Tunisia. These characteristics are: the rareness of the infection, the relative frequency of affection in women, the proximal involvement of the foot, the frequency of agricultural activity and the rareness of traumatic past history, the predominance of the actinomycosic origin due to Actinomadura madurae, and the need to associate surgical exeresis with the medical treatment or amputation in order to stop the progress of the disease.

[Intense hemosiderin deposits in a case of self-healing congenital histiocytosis]. / Dépôts hémosidériniques importants au cours d'une histiocytose congénitale auto-involutive.

BACKGROUND: Congenital self healing histiocytosis, described for the first time in 1973 by Hashimoto and Pritzker, is characterized by multiple violaceous brown cutaneous papulonodules present at birth and disappearing spontaneously in 2 to 4 months. There are classically no systemic manifestations and the prognosis is always good. We present a case, particular by the intensity of hemosiderinic deposits. CASE-REPORT: The patient was a 2-month-old female infant. Physical examination at birth revealed a healthy-appearing full-term newborn, with five skin lesions: firm violaceous-reddish pigmented papules and nodules distributed over the head (scalp and forehead), right ear, right forearm and the flexural aspect of the left thigh. There was no hepatosplenomegaly or lymphadenopathy, and the patient's general condition was excellent. Histologic examination confirm the diagnosis by showing a dense polymorphous infiltrate composed of histiocytes associated with giant cells and erythrocytes extravasation. Perls stain was strongly positive. Staining with S100 Protein was positive. Outcome was good with involution of all lesions with atrophic and pigmented scars. DISCUSSION: The patient's excellent general condition, the absence of visceral locations and the spontaneous self healing allows us to classify this form as a benign self healing congenital histiocytosis. This patient presented intensely hemosiderinic deposits giving the lesions a strongly pigmented aspect.

[Comparative epidemiology of pemphigus in Tunisia and France. Incidence of foliaceus pemphigus in young Tunisian women]. / Epidémiologie comparative du pemphigus en Tunisie et en France fréquence du pemphigus superficiel parmi les jeunes femmes Tunisiennes.

INTRODUCTION: Recent studies have suggested that pemphigus foliaceus is quite frequent in young Tunisian women. In order to confirm this hypothesis, we compared the incidence of pemphigus in general in Tunisia with that in the Ile-de-France region. METHOD: All new cases of pemphigus diagnosed during a 6-year period were reviewed in our dermatology and pathology laboratories. These cases were classed as pemphigus foliaceus or pemphigus vulgaris on the basis of histology reports. RESULTS: In France, the incidence was 1-7 new cases per million per year (95 p. 100 confidence interval 1.4-2.1). Pemphigus vulgaris was diagnosed in 73 p. 100 of the cases with an incidence increasing with age. Sex ratio (F/M) was 1.2. The incidence observed in Tunisia was significantly higher than that observed in France with 6.7 new cases per million per year (95 p. 100 confidence interval 5.8-7.7). Pemphigus foliaceus was more frequent (61 p. 100), the sex ratio (F/M) was 4.1. Incidence was higher in young women, with 20 new cases of pemphigus foliaceus per million per year among women from 25 to 34 years of age. These levels were higher in rural desert areas. No familial cases were observed and only one case occurred in a child. DISCUSSION: These findings confirm the specific epidemiology of pemphigus in Tunisia, which appears to be similar and also different from that in Brazilian pemphigus. As in Brazil, there was a predominance of pemphigus foliaceus in young adults living in rural areas in poor socioeconomic conditions. However in Tunisia the disease predominates significantly in women and there are no familial and rare juvenile cases.

Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women.

Recent studies have suggested that in Tunisia, pemphigus foliaceus is more frequent in young women than expected. To confirm these findings, we compared the incidence rates of pemphigus in Tunisia as a whole and in a large area of France. New cases of pemphigus were detected retrospectively from dermatology departments and pathology laboratories over a 6-year period and classified as pemphigus vulgaris or foliaceus according to the pathology. In France, the incidence rate was 1.7 cases per million per year (95% confidence interval 1.4 to 2.1). Pemphigus vulgaris accounted for 73% of all cases, incidence increased with age, and the female-to-male sex ratio was 1.2. The incidence rate was significantly higher in Tunisia than in France: 6.7 cases per million per year (95% confidence interval 5.8 to 7.7); pemphigus foliaceus was more frequent (61%), the female-to-male sex ratio was 4.1, and the incidence rate was higher in young women. The incidence rate was 15.5 cases per million per year for pemphigus foliaceus among women aged 25 to 34 years and was even higher in some rural areas. No case was observed among household members or in neonates, and only one case occurred in childhood. Thus, we confirmed that the epidemiology of pemphigus in Tunisia is unusual. High rates of pemphigus foliaceus among young people living in rural areas are reminiscent of Brazilian pemphigus. However, the absence of cases among genetically related household members and during childhood, and the large predominance of women, contrast with Brazilian pemphigus.

[Cutaneopulmonary blastomycosis]. / Blastomycose cutaneo-pulmonaire.

We observed a new case of blastomycosis in a patient with haemoptysis. The chest X-ray revealed a lesion confined to the upper right lobe of the lung and there was an ulcerating vegetation at the medial angle of the right eye. Skin biopsy and bronchial aspiration led to the identification of typical Blastomyces dermatitidis. Outcome was favourable after oral therapy with ketoconazole. Cutaneo-pulmonary blastomycosis is weakly endemic in Tunisia. Pulmonary lesions are more frequent because of airborne transmission. Cutaneous lesions may be clinically misleading. This disease is sensitive to antifungal imidazole derivatives.

[Iga linear bullous dermatosis in children. A series of 12 Tunisian patients]. / La dermatose bulleuse à IgA linéaire de l'enfant. Une série de 12 malades tunisiens.

BACKGROUND: Linear IgA bullous dermatosis (LABD) of childhood is one of the chronic, non-hereditary blistering diseases of childhood in which clinical, histologic and therapeutic findings are similar to those associated with bullous pemphigoid (BP) and dermatitis herpetiformis (DH). LABD, however, can be distinguished from BP of DH by direct immunofluorescence (IF) demonstration of linear IgA deposits along the basement membrane zone (BMZ). RESULTS: During the period 1984-1993, 12 children with LABD were studied. Their ages ranged from 2 years to 15 years with a mean of 8.5 years. There were 7 males and 5 females. All children had a generalized eruption consisting of large tense blisters arising on normal skin. The blisters were more profuse on the lower trunk, pelvic region and limbs. Face and scalp were also affected. Occasionally, annular blister formation producing a "rosette" or "cluster of jewels" was found. Pruritus was frequent. Histological features of BP and DH were seen. Direct IF showed linear deposits of IgA at the BMZ in all cases. IgM, IgG, and complement were also seen in 8 cases. Four patients showed IgA BMZ antibodies by indirect IF. There were no symptoms of malabsorption and 3 patients had a mild bowel lesions. HLA studies showed the B8DR3 antigen in 7 of the 10 patients studied. Nine patients were treated with dapsone associated in 3 patients with prednisone. Three patients were controlled on oxacillin. CONCLUSION: LABD of childhood is a definite clinical entity. It is the most frequent chronic, non hereditary bullous disease of childhood in Tunisia. It is characterized by a self limiting blistering eruption which resembles BP or DH histologically and has a characteristic linear deposits of IgA at the BMZ of the skin. The treatment consisted on dapsone therapy, but 3 patients in our study were well controlled on oxacillin.

[Apropos of a case of perforating, leprous plantar ulcer occuring at 15 years of age]. / A propos d'un cas de mal perforant plantaire lépreux survenu à l'âge de 15 ans.

Perforating ulcers of the foot occurring in leprosy are frequent, chronic, often giving mutilations. They usually affect the adult. Plantar ulcer occurred in a 15 year-old young patient affected by tuberculoid leprosy has incited us to report it.

[Actinic lichen planus. An anatomoclinical study of 10 Tunisian cases]. / Le lichen plan actinique. Etude anatomo-clinique de dix observations tunisiennes.

BACKGROUND: Actinic lichen planus is a distinct variant of lichen planus involving mainly children and teenagers with racial predilection to orientals. The clinical and histopathologic features of actinic lichen planus in ten tunisian patients were studied. RESULTS: The mean age was 14 years. The eruption was distributed over sun-exposed areas, with particular predilection for the face. In most cases the lesions consisted of erythematous brownish plaques with an annular configuration. Less commonly, hypermelanotic patches, sometimes assuming a melasma-like appearance, were present. The histopathologic feature consisted of a lichenoid dermatitis. CONCLUSION: The cause of actinic lichen planus is unknown. Sunlight appears to be the major precipitating factor and therefore the disease would best be included among the photodermatoses.

Pemphigus foliaceus in young women. An endemic focus in the Sousse area of Tunisia.

BACKGROUND AND DESIGN: With the exception of some areas in Brazil, pemphigus is an exceptional disease. Several previous observations suggested that pemphigus foliaceus occurred more frequently than expected in Tunisia. To confirm these observations, a 15-month prospective survey was undertaken at the University Hospital of Sousse in Tunisia. RESULTS: During the survey, 23 patients with pemphigus were observed. Twenty of 23 cases were of pemphigus foliaceus that affected only young women. Seven patients had the clinical features of pemphigus herpetiformis. The estimated incidence of pemphigus foliaceus in the Sousse area was four new cases per million inhabitants per year, far above European or North American incidence but lower than the incidence in the most severely affected areas in Brazil. The high prevalence of the herpetiform clinical variant, the young age, the feminine exclusivity, and the absence of familial cases differentiated this Tunisian pemphigus both from pemphigus foliaceus observed in Europe and North America and from the Brazilian fogo selvagem. No etiologic factor was identified. CONCLUSIONS: This study suggests the existence in Tunisia (and possibly in all North Africa) of an endemic form of pemphigus foliaceus restricted to young women.

[Bone anomalies in von Recklinghausen disease]. / Anomalies osseuses dans la maladie de Von Recklinghausen.

28 cases of neurofibromatosis are reported. Principals bone anomalies are reviewed. The skeletal manifestations are numerous and varied. These anomalies interest specially cranial vault, spheno-orbital area and vertebral spine. It is imperative to keep in mind skeletal manifestations because neurologic and cutaneous signs can be absent.