Cutaneous sarcoidosis in Tunisia.

AIM: Sarcoidosis is a multisystem disease of unknown etiology characterized by granulomatous infiltration. As clinical features vary among patients from different ethnic groups and geographic areas, local reports are of great interest. The aim of this study was to make an inventory of the clinical presentation of cutaneous sarcoidosis in Tunisia. METHODS: The study was conducted in an academic teaching hospital, inpatient and outpatient department. The authors retrospectively reviewed the medical records of all patients diagnosed with sarcoidosis at the Dermatological Departement of Charles Nicolle teaching Hospital, Tunis, within a 32-year period. RESULTS: One hundred and eighteen patients were diagnosed to have sarcoidosis, corresponding to 25 males and 93 females. Mean age at presentation was 39.5 years. Childhood sarcoidosis represented 14.4% of cases. Cutaneous involvement was dominated by micronodular lesions. Chest X-rays abnormalities were present in 28 patient/93 (30.1%). Most of them were at stage 1 or 2. Peripheral lymph node enlargement was reported in 15 patients (12.7%). Other internal organ involvements corresponded to: eyes (15 cases); bones (12 cases), joints (5 cases), gasto-intestinal tract (5 cases), upper airways (5 cases), liver (4 cases), kidney ( 1 case) and muscles (1 case). Systemic involvement was noticed in 88% patients with lupus pernio. Treatment modalities included topical and oral corticosteroids, hydroxychloroquine, allopurinol and surgical excision with variables outcomes. CONCLUSION: The data of this study suggest that sarcoidosis manifests with a wide range of cutaneous lesions in Tunisian patients and that extracutaneous involvement is not rare. Remarkable clinico-epidemiological characteristics are the clear predominance in women, the high frequency of childhood cases and the predominance of micronodular skin lesions.

[Sweet's syndrome: report of 5 cases]. / Le syndrome de Sweet: a propos de 5 observations.

Sweet's syndrome is an acute febrile neutrophilic dermatoses. Cutaneous signs are the main manifestations. The objective of this study is to deal with the epidemiological and clinical aspects of the disease and its treatment. It is a retrospective study over a period of 5 years (2000- 2004). We have included all the patients with characteristic clinical, biological and histological features. Five patients had been identified. They were all females. Their mean age was 42, 2 years. All patients had typical clinical, histological and biological features. Crohn disease was associated in one case. Sweet's syndrome responds rapidly to systemic therapy with corticosteroids and/or nonsteridal anti-inflammatory agents. Sweet's syndrome is a rare disease which occurs mainly in middle-aged women. This was found in our study. Corticosteroids are the standard therapy. Several diseases, particularly neoplasms and inflammatory diseases, can bee associated with Sweet's syndrome and condition the prognosis.

Dyskeratosis follicularis disease: case reports and review of the literature.

BACKGROUND: Dyskeratosis follicularis (Darier's disease) is rare autosomal dominant disease characterized by the loss of adhesion between epidermal cells and by abnormal keratinization. METHODS: We performed a retrospective study of all the patients diagnosed with Darier's disease at the Department of Dermatology of Charles Nicolle Hospital of Tunis, between 1971 and 2002. RESULTS: During the observation period, we identified 12 patients with Darier's disease; five males and seven females with a mean age of 17.36 years. No family history was found in eight patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the face (nine patients), chest and scalp. Seven patients had nail lesions. UV light exposure exacerbated the disease symptoms in seven cases. The patients were treated with topical and systemic retinoids (six cases). CONCLUSION: Although Darier's disease has a chronic course, most patients manage to lead a relatively normal life. Treatment is usually unsatisfactory despite much progress in understanding of the underlying abnormalities in Darier's disease.

Intramuscular bipenicillin vs. intravenous penicillin in the treatment of erysipelas in adults: randomized controlled study.

The objective of the study was to evaluate the efficacy of intramuscular penicillin: mixture of benzyl penicillin and procain penicillin (2 MU x 2 times daily) and intravenous benzyl penicillin (4 MU x 6 times daily) in the treatment of hospitalized adult patients with erysipelas. A prospective randomized unicentric trial was conducted. In total, 112 patients entered the study; 57 in the intramuscular group and 55 patients in the intravenous group completed the trial. The failure rate was 14% for intramuscular group and 20% for the intravenous group (P = 0.40). Local complications such as of the leg abscesses were observed in the two groups (intravenous 9.1%, intramuscular 7%; P = 0477). Of the patients treated with intravenous benzyl penicillin, 25.5% presented complications related to the route (venitis). Intramuscular penicillin should be considered an effective and well-tolerated treatment of erysipelas in adult patients.

[Melanoma in xeroderma pigmentosum: 12 cases]. / Mélanome et xeroderma pigmentosum: 12 cas.

BACKGROUND: Xeroderma pigmentosum is a rare genodermatosis, with a defect affecting recovery of ultraviolet-induced damages and characterized by a high rate of malignancies of the exposed skin areas. We studied melanoma features of patients with xeroderma pigmentosum. PATIENTS AND METHODS: A retrospective study of xeroderma pigmentosum patients admitted to the Charles Nicolle Hospital of Tunis between 1973 and 1998. RESULTS: Two hundred sixteen patients with xeroderma pigmentosum were registered. Melanoma was present in 12 patients, 7 females and 5 males. Two patients were sisters. Cutaneous melanoma was found in 8 patients. Four patients presented with metastatic melanoma. The median age for development of the first melanoma was 17.5 years. All of the cutaneous melanomas were found on the face. Lentigo malignant melanoma was reported in 3 cases. The tumors were treated with surgical excision. Except for a melanoma affecting the orbit, characterized by a fatal outcome, no metastases were detected at the different investigations. DISCUSSION: Melanoma occurs frequently in patients with xeroderma pigmentosum, it has been reported in 5.5 p. 100 of cases and 11.3 p. 100 of patients with cutaneous carcinoma. The age of onset was low: 17.5 years. It appeared later than the carcinoma. The location of cutaneous melanoma in face in xeroderma pigmentosum patients indicates that they were caused mainly by sunlight exposure. Lentigo malignant melanoma was the most frequent type. Prognosis is difficult to define owing to the large number of other cutaneous malignancies. Apart from one case of rapidly fatal orbital melanoma, we recorded long survivals even in cases of melanoma revealed by metastases.

[Comparative study of the efficacy and tolerance of 12% glycolic acid cream and 0.05% retinoic acid cream for polymorphic acne]. / Etude comparative de l'efficacité et de la tolérance d'une crème a 12% d'acide glycolyque et d'une crème a 0.05% d'acide retinoique dans l'acné polymorphe.

UNLABELLED: Topical retinoic acid has demonstrated its efficacy in acne. Glycolic acid has also keratolytic action and could be interesting to treat acne. OBJECTIVE: To evaluate the efficacy and safety of topical glycolic acid (Glyco A 12%) and retinoin acid (Kefrane 0.05%) on facial acne lesions. METHOD: It was a randomised double blind study of 40 patients, divided in 2 groups, 20 receiving GlycoA cream (Group A) and 20 receiving Kefrane cream (group B). Medical exam was realised by the same physician at day 0, 21 and 45. The improvement of acne as well as side effects were assessed by both the physician and the patients themselves. RESULTS: 2 patients developed severe eczema of the face and were excluded (one from group A and one from group B). 22 patients followed totally the study. 90% of group A and 83.3% of group B patients improved significantly their lesions. A comparative tolerability was observed for both groups. CONCLUSION: Glyco A cream could be a good treatment for acne even in dark skin patients.

[GAPO syndrome]. / Le syndrome GAPO.

INTRODUCTION: The GAPO syndrome is a rare but distinct genetic disorder. GAPO is an acronym for the manifestation of Growth retardation, Alopecia, Pseudoanodontia and Optic atrophy. The syndrome was first reported in 1947; to date, 24 cases have been reported. We report the first Tunisian case. OBSERVATION: We studied a 12 year-old boy with GAPO syndrome which was associated with peculiar facial appearance, umbilical hernia, hemangiomatous plaques of the neck, depigmented maculae arranged in a splashed pattern located in the trunk and the right upper limb. He had a pulsated mass in the right mastoid area and a bruit was audible, he had a second flaccid mass of the vertex. These tumefactions correspond to very developed commissure veins. DISCUSSION: In addition to the classical manifestations of the GAPO syndrome, the patients have a strikingly characteristic facial appearance and may also have umbilical hernia, skin redundance and prominent dilatation of scalp veins. Our case had depigmented maculae suggestive of incontinentia pigmenti achromians. This has never been reported previously. The pathogenesis of this syndrome is unknown and inheritance is considered to be autosomal recessive.