Hailey-Hailey disease in Tunisia.

SUMMARY BACKGROUND: Most of the published reports on Hailey-Hailey disease (HHD) come from European and Asian countries. We report here the clinical and genetic investigation of 20 patients affected with HHD in Tunisia. METHODS: Affected individuals from three large teaching hospitals in Tunis were recruited for the study over a 25-year period. Nine patients were identified through the active files and examined together with their family members that were visited in their respective regions. We have clinically examined in total 65 individuals and then identified 11 new cases. Patients were included on the basis of evocative skin lesions, biopsy proven HHD and negative immunofluorescence. Investigations to rule out fungal, bacterial and viral infections were done according to clinical symptoms. RESULTS: Twenty patients (12 males and 8 females) from 8 families were included in the present study with more than 55% that were undiagnosed before this investigation. Four patients had mild disease, eight had moderate disease and another eight had severe disease, among whom seven were females. Parental consanguinity was found in 7 cases out of 20 cases (35%). The neck region was first affected in half (4/8) of the male patients. Groins were first affected in 42% (5/12) of the female patients. Depression complicated the course of the disease in two female patients with severe HHD. We report an original association of supernumerary nipples with HHD in two sisters from the north of Tunisia. In 10 patients, the disease has become less troublesome with aging. CONCLUSION: HHD is underestimated. Physicians must be aware of this disease in case of resistant intertriginous dermatosis especially with a positive family history as nine out of 20 patients were misdiagnosed.

[Leg ulcers are rare in Tunisian women]. / Les ulcères de jambes sont rares chez la femme tunisienne.

Numerous reports from Tunisia and Morocco have underlined the low frequency of leg ulcers in women from the Maghreb region in comparison with European women. This retrospective study describes patients hospitalized for leg ulcers in the Dermatology Department of Charles Nicolle Hospital in Tunis between January 1995 and December 2005. A total of 134 patients including 107 men and 27 women (M/F sex-ratio: 3.96) were hospitalized for leg ulcers. Venous leg ulcer was the most common accounting for 54.5% of cases overall, 57% of cases in men and 33.3% of cases in women (p = 0.028).

[Evaluation of the role of dietary intake in the occurrence of alopecia]. / Evaluation du rôle des apports alimentaires dans la survenue d'une alopécie diffuse.

Transversal case-control study was conducted among 42 patients aged between 20 and 35 years who had diffuse alopecia. Alimentary inquiry according to the 3-day method was achieved in each case. The following nutriments were evaluated: total proteins, calcium, copper, iron, iodine, magnesium, manganese, phosphorus, potassium, selenium, zinc, omega 3 and omega 6. A control group (composed of 230 individuals), matched for age, gender and metabolic profile was established. These persons did not suffer from hair and nail disorder. The nutriments were codified according to the data of the software Food processor 8.3 version. The data were analyzed using "SPSS" 11.5 version. Comparisons of the means were performed using the Student's t test. ROC graphics allowed to determine the statistically significative limits for the comparison of both groups. On multivariate analysis, only a protein intake was directly associated to alopecia, odds ratio of 1,5 (1,06 - 2,3) p=0,02.

Efficacy of tacrolimus 0.03% ointment as second-line treatment for children with moderate-to-severe atopic dermatitis: evidence from a randomized, double-blind non-inferiority trial vs. fluticasone 0.005% ointment.

Tacrolimus 0.03% ointment is licensed for second-line treatment of children with atopic dermatitis (AD). Although data are available from clinical trials, no study has enrolled only second-line patients. This double-blind, non-inferiority study compared tacrolimus 0.03% and fluticasone 0.005% ointments in children with moderate-to-severe AD, who had responded insufficiently to conventional therapies. Children (aged 2-15 yr) were randomized to tacrolimus ointment (n = 240) or fluticasone ointment (n = 239), twice daily until clearance or for a maximum of 3 wk and, if lesions remained, once daily for up to 3 wk further. Primary end-point was week 3 response rate (improvement of >or=60% in modified Eczema Area and Severity Index and not withdrawn for lack of efficacy). Secondary end-points included pruritus and sleep quality, global assessment of clinical response, incidence of new flares and safety. Response rates were 86.3% with tacrolimus ointment and 91.5% with fluticasone. Lower limit of the 95% confidence interval was -11.8%, exceeding the non-inferiority limit of -15% and meeting the primary end-point. Moderate or better improvement on the physicians' global assessment occurred in 93.6% and 92.4% of patients in the tacrolimus ointment and fluticasone arms, respectively, while median pruritus scores improved by 84.0% and 91.5%. Sleep quality improved by approximately 92% in both treatment arms. After day 21, new flare-up occurred in 5.5% and 11.3% of patients receiving tacrolimus ointment and fluticasone, respectively; mean times to new flares were 6.5 +/- 5.0 and 8.6 +/- 5.2 days. Adverse events were similar between the two arms, with the exception of application-site skin burning sensation in the tacrolimus ointment group. In conclusion, efficacy of tacrolimus 0.03% ointment as second-line treatment was not inferior to that of fluticasone 0.005% ointment, with similar benefits on global disease improvement and quality of sleep.

Acquired erythroderma in adults: a clinical and prognostic study.

BACKGROUND: Erythroderma is a severe syndrome and prognostic studies are rare in the literature. OBJECTIVES: Through a retrospective study of erythroderma in adults, we have analysed epidemiological and clinical data and precised the relevant aetiologies and survival in our patients. METHODS: This study was performed at the Department of Dermatology of Charles Nicolle Hospital of Tunis (1995-2007) including 82 cases of acquired erythroderma (>16 years). We have recorded epidemio-clinical, biological and histological data, treatment and outcome. Clinical-histological correlation was analysed [kappa coefficient (kappa)]. Follow-up time and disease-free survival time were calculated as were Kaplan-Meier estimates of overall survival and relapse-free survival for some aetiologies. RESULTS: Erythroderma represented 0.44 per thousand of all dermatoses with an age of 55.13 +/- 18.16 and no sex predilection. Psoriasis was the predominant aetiology (32.9%) with a median duration of 6.75 years and previous one or more episodes of erythroderma. Psoriasis was significantly associated with pruritus (P = 0.0001), pachyonychia (P = 0.00001), palmoplantar keratoderma (P = 0.0001) and hypereosinophilia (P = 0.008). The latter is then not specific for drug induced erythroderma (P = 0.004). Carbamazepine (27.8%) and penicillin (22.2%) were the most implicated drugs. Positive Clinical-histological correlation was found in 77% of cases (kappa = 0.753). Relapse was seen in all aetiologies, but drug reactions and had occurred in the first 3 years in 90% of them. Mortality rate was 11.3 per 1000 patients-years. CONCLUSIONS: Our study illustrates the severity of erythroderma. It alters heavily the quality of life of patients which is initially altered by the pre-existent dermatosis. It may be life threatening as mortality rate is high.

[Temporary tatooing: black henna or harkous?]. / Tatouage éphémère: henné noir ou harkous?

Contact sensitization to natural henna (Lawsonia inermis) long used as a skin dye in powder or paste form has rarely, if ever, been observed. Recently a number of anaphylactoid reactions to PPD contained in paints used for temporary tattoos have been described. The purpose of this article is to present 8 cases involving 4 children and 4 adults who developed contact dermatitis after temporary tattoo using "harkous" that contains no henna. The authors review the literature about temporary tattooing. "Harkous" is a mixture of nut gall and clove to which PPD is sometimes added. Many reports have described cases of contact dermatitis due to "black henna". Most of these cases have involved tourists returning from trips.

Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects.

According to the WHO-EORTC classification of cutaneous lymphomas, primary cutaneous marginal zone B-cell lymphoma are now well characterized. We report here a case of primary cutaneous marginal zone B-cell lymphoma in a 51 year-old man in which the diagnosis was made using both histology and immunopathology. The patient had no remarkable medical history, no history of either acute inflammation or insect bite, and presented with a 5 cm solitary asymptomatic erythematous firm, multinodular and infiltrated plaque on the back for 12 months. Histological examination and immunohistochemical study of a cutaneous biopsy provided a differential diagnosis between B cell lymphoma and lymphocytoma cutis. Full body work up revealed no signs of extracutaneous dissemination. The patient underwent surgical excision of the nodule. Histological examination showed a histological and immunophenotyping profile typical of primary cutaneous marginal zone B-cell lymphoma. The lesion was completely excised with clear margins and no recurrence occurred after a 12 month-follow-up period. Primary cutaneous marginal zone B-cell lymphoma are low-grade lymphomas that have an indolent course and a high tendency to recur. They should be differentiated from lymphocytoma cutis and from the other types of cutaneous B cell lymphomas that have a different course and prognosis.

[Tuberculous lupus]. / Le lupus tuberculeux.

UNLABELLED: Cutaneous tuberculosis accounts for 2% of all extrapulmonary tuberculosis and is one of the multiple clinical presentations of extrapulmonary tuberculosis. We report a case of cutaneous tuberculosis with unusual clinical and histological presentations. CASE REPORT: We report the case of a 29-year-old woman with plurifocal tuberculous lupus. Histological examination of cutaneous lesions revealed the presence of a granuloma with caseum necrosis. Chest X-ray showed a severe pulmonary tuberculosis lesion. The Mantoux test performed by intradermal injection was positive. Skin lesion PCR and sputum culture were negative. Both skin and pulmonary lesions responded to antituberculous treatment. COMMENTARY: Lupus vulgaris is commonly presents on the head and neck. The diagnosis may be difficult when lupus vulgaris occurs at unexpected sites or in unusual clinical presentations. Caseum necrosis observed in our patient was unusual.

Superiority of tacrolimus 0.1% ointment compared with fluticasone 0.005% in adults with moderate to severe atopic dermatitis of the face: results from a randomized, double-blind trial.

BACKGROUND: No specific data are available on tacrolimus ointment as a second-line treatment in adults with facial eczema. OBJECTIVES: To compare tacrolimus 0.1% and fluticasone 0.005% ointments in adults with moderate to severe atopic dermatitis (AD) of the face in whom conventional treatment was ineffective or poorly tolerated. METHODS: Patients were randomized to double-blind treatment of facial AD with twice-daily tacrolimus ointment (n = 288) or fluticasone ointment (n = 280) for 3 weeks or until clearance. After day 21, patients could continue without the study treatment, apply the same ointment once daily, or switch to the other medication twice daily, depending on lesion clearance and patient/physician satisfaction. The primary endpoint was the day-21 response [> or = 60% reduction in the modified Local Eczema and Severity Index (mLEASI) score]. Secondary endpoints included facial erythema and pruritus, global clinical response, treatment switching at day 21 and safety. RESULTS Response with tacrolimus ointment (93%) was superior to that with fluticasone (88%; P = 0.026). Improvements in mLEASI components were also greater with tacrolimus ointment. Facial erythema and pruritus improved in both groups. Global clinical response was rated 'marked improvement' or better in 88% and 79% of patients in the tacrolimus ointment and fluticasone groups, respectively. At day 21, 9% of patients switched from fluticasone to tacrolimus ointment, while 4.5% switched from tacrolimus ointment to fluticasone. Adverse events were more frequent with tacrolimus ointment as a result of the higher incidence of application-site skin burning sensation. Safety of both drugs was in line with their respective summary of product characteristics. CONCLUSIONS: Tacrolimus 0.1% ointment has superior efficacy to fluticasone 0.005% ointment for twice-daily treatment of adults with moderate to severe facial AD in whom conventional therapy was inadequately effective or not tolerated. Tacrolimus 0.1% ointment is a safe and effective second-line treatment for the control of moderate to severe AD of the face.

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.