Transient symptomatic zinc deficiency in a breast-fed infant: relevance of a genetic study.

OBJECTIVE: We present a case of acrodermatitis enteropathica in a full-term, breast-fed, 7-mo-old infant born from consanguineous parents with a family history of acrodermatitis enteropathica. METHODS: The patient presented with periorificial and symmetric acral lesions, which prompted us to review the clinical features of acrodermatitis enteropathica and its pathogenesis. Laboratory investigations showed low zinc levels in the infant's and mother's sera and in the mothers' milk. RESULTS: A diagnosis of acrodermatitis enteropathica was made. A mutation screening of the SLC39A4 gene in the patient and his mother showed heterozygosity for the deletion c.1223_1227delCCGGG. The diagnosis of transient symptomatic zinc deficiency was then established. CONCLUSION: Transient symptomatic zinc deficiency is generally reported in premature infants but should also be considered in full-term, breast-fed infants, as in the present case.

[Pyoderma gangrenosum: 6 cases]. / Le pyoderma gangrenosum: à propos de 6 observations.

Pyoderma gangrenosum (PG) is an uncommon extensive cutaneous ulceration belonging to the neutrophilic disease spectrum. It is associated to a systemic disease in almost 50% of cases. The diagnosis primarily relies on clinico-pathological features and the treatment is empirical. We report a retrospective series of 6 observations of PG (4 women and 2 men, median age = 43 years) enrolled over 15 years. The diagnosis was established based on the presence of 2 major criteria and 2 minor criteria of the disease. In 3 patients, PG was associated to an already known ulcerative colitis. The treatment consisted in general corticotherapy.

[Fixed drug eruption : about 13 cases]. / Erytheme pigmente fixe. A travers une serie hospitaliere de 13 cas.

AIM: To discuss, through a retrospective study, the epidemiological and clinical aspects and the causative agents of fixed drug eruption. METHODS: Thirteen cases were collected retrospectively during 11 years. There were 10 females and 3 males with a mean age of 44 years. RESULTS: The lesions correspond to erythematous plaques which fade to leave slate-brawn macules in all cases. The most frequent localizations were limbs (12 cases), trunk (6 cases), face (3 cases) ans external genitals (3 cases). Sulfonamides were the most frequent responsible drugs in our series (7 cases). CONCLUSION: Fixed drug eruption is characterized by one or more erythematous plaques which recur in the same places after challenge. Sulfonamides are actually the most frequent causative drugs in the different series.

Michelin tire syndrome: a report of two siblings.

Michelin tire syndrome is a rare syndrome characterized by excessive folding of the skin. The diagnosis is mainly clinical. It has been found to be associated with noncutaneous anomalies, and probably reflects multiple underlying disorders. We report two siblings with Michelin tire syndrome, a 5-year-old boy and his sister both of whom had marked skin folds and facial anomalies. Histologic study found an increase in smooth muscle fibers of the dermis. Electron microscopy showed details of smooth muscle cells. We also review reported cases in the literature to contribute to a better understanding of this syndrome.

[Mycosis fungoides. Study of a series of 11 Tunisian cases]. / Le mycosis fongoïde. Etude d'une série Tunisienne de 11 cas.

Mycosis fungoides is an epidermotropic cutaneous T lymphoma. It's a non Hodgkinian lymphoma. We report the results of a retrospective review of 11 mycosis fungoide seen during 22 years. The frequency of MF was about 39.3% among all cutaneous lymphoma. Six patients were male and 5 were female; the mean age was about 56 years. Mean delay between diagnostic and the first manifestation was about 25 months. All patients had the progressive form: 4 had infiltrate plaques and 7 were at the tumoral phase. Lymph nodes and medullar metastases were noted respectively in 1 and 2 cases. Treatment was mono or polychemotherapy associated in 6 cases with topical drug. Three patients died of their diseases According to our experience and after reviewed the literature, we notice that our patients are slightly younger without male predominance. The diagnostic was done tardily and this may explain the pejorative prognostic.

Possible relation of Tunisian pemphigus with traditional cosmetics: a multicenter case-control study.

Pemphigus is a severe, autoimmune, blistering disorder with a high incidence among young women in rural Tunisia. The authors investigated explanatory environmental factors. A multicenter case-control study was conducted prospectively from 1992 to 1996 in Tunisia. Sixty-eight incident female cases of pemphigus and 166 controls matched on age, hospital, and geographic area were included. Data collected concerned socioeconomic status, medical history, drug intakes, lifestyle, and environment. Several factors were significantly associated with pemphigus in multivariate logistic regression analyses: traditional cosmetics (odds ratio (OR) = 4.0, 95% confidence interval (CI): 1.1, 14.8); Turkish baths (OR = 3.2, 95% CI: 1.4, 7.3); cutting up raw poultry (OR = 5.1, 95% CI: 1.3, 19.4); contact with ruminants (OR = 2.7, 95% CI: 1.3, 5.8); and wasp, bee, and spider stings (OR = 3.1, 95% CI: 1.5, 6.4). A dose-dependent relation was observed for traditional cosmetics. All risks except insect bites were higher when analysis was restricted to younger women, the demographic group with higher incidence. The strength of the associations, the dose-dependent relation for traditional cosmetics, and the increase of risk estimates for younger women support a causal relation. Traditional cosmetics widely used by Tunisian women could play a major role in excess of cases of pemphigus.

[Pityriasis rotunda: second Tunisian case]. / Pityriasis rotunda: 2ème cas Tunisien.

Pityriasis Rotunda is a dermatosis well known in Japan and Black Africa. Its unusual in Europe and North Africa. Several authors consider that Pityriasis Rotunda is an acquired ichtyose and that it represents a real paraneoplastic syndrome. We report the case of Tunisian men suffering from a Pityriasis Rotunda associated to a dilatation of branch.