Samy Fenniche

Pyoderma gangrenosum: a report of 21 cases.

Background Pyoderma gangrenosum (PG) is an uncommon, destructive, cutaneous ulceration, belonging to the neutrophilic disease spectrum. It is associated with systemic disease in 50% of cases.

Acebutolol-induced subacute cutaneous lupus erythematosus.

Beta-blocking medications are rarely associated with drug-induced lupus erythematosus syndrome and have never been incriminated as a cause of subacute lupus erythematosus (SCLE). We present herein the first case of SCLE induced by acebutolol. A 57-year-old woman presented with a 1-month history of a cutaneous eruption of the photoexposed areas. One month ago, the patient had started a treatment with oral acebutolol to cure a hypertension of 1-year evolution. Physical examination revealed erythematous scaly annular plaques, involving the face, arms and trunk. Immunologic serology findings revealed a positive titer of antinuclear antibodies up to 1/1,280 with positivity of antihistone and Ro/SSA antibodies. Acebutolol was stopped, and the lesions cleared completely 4 months later. Literature data, along with our case, suggest a link between acebutolol therapy and the onset of a lupus syndrome. Although this is the first report of acebutolol-induced SCLE, we should be aware of this occurrence, and avoidance of acebutolol is recommended in patients with stigmata of lupus erythematosus

Leishmaniose endonasale primitive à Leishmania infantum MON-80 en Tunisie

BACKGROUND Mucocutaneous leishmaniasis is endemic in Central and South America. It causes massive mutilating and disfiguring lesions and can lead to destruction of facial structures. In Tunisia, leishmaniasis of the mucous membranes is rare, usually developing as a complication of cutaneous leishmaniasis via direct extension. We report the first case in Tunisia of isolated and primary nasal leishmaniasis. CASE REPORT A 70-year-old man with a history of nephrectomy for renal lithiasis was seen with a painless nodule that had been present for one month. The latter was erythematous, polypoid and firm, with a diameter of 2 cm, and was situated in the right endonasal mucosa. The diagnosis of leishmaniasis was confirmed by histological and direct examinations revealing high numbers of amastigotes of Leishmania. Culture of the offending organism in NNN medium and isoenzymatic characterization resulted in identification of MON-80 Leishmania infantum leishmaniasis. The outcome was good with treatment, and the nodule was deflated after six months. DISCUSSION There have been few reports of similar cases of primary and isolated mucosal leishmaniasis caused by Leishmania infantum. Our case is also unusual in that zymodeme MON-80 is only rarely a cause of Mediterranean leishmaniasis.

Radiotherapy‐induced pemphigus vulgaris with autoantibodies targeting a 110 kDa epidermal antigen

Background  Pemphigus is an autoimmune intraepidermal blistering disease mediated by autoantibodies targeting desmosomes. It can be induced by many triggers, such as ionizing radiation.

New mutations of Darier disease in Tunisian patients

Darier’s disease (DD, MIM 124200) also known as Darier-White disease and keratosis follicularis, is a rare autosomal dominant skin disorder characterized by warty papules and plaques in the seborrheic area (central trunk, flexures, scalp, and forehead). Pathogenic mutations in the ATP2A2 gene encoding the sarcoplasmic/endoplasmic reticulum Ca2+ ATPase (SERCA) 2 gene underlie the disease. In the present study, we performed genetic investigation of three unrelated Tunisian families affected by DD. Mutation screening was performed by direct sequencing of the coding region and exon/intron boundaries of the ATP2A2 gene. Patients in the 3 studied families exhibited classical DD phenotype. DD was associated with neurological and cardiac disorders in one family. Two novel mutations were identified: a missense mutation (R559Q) and a frameshift mutation (1713-1714 del 2A). Both pathogenic mutations are located in exon 13 of the ATP2A2 gene and affected the ATP-binding site of the SERCA2 protein. In one family, no mutation was found within the coding region and exon/intron boundaries of the ATP2A2 gene. Our findings provide further evidence for the genetic heterogeneity of DD in Tunisia and that most mutations involved in this disease are family specific.

Hémangioendothéliome épithélioïde cutané

Resume Introduction L’hemangioendotheliome epithelioide est une tumeur des tissus mous prenant naissance a partir de l’endothelium vasculaire. Elle est consideree comme une tumeur de bas grade de malignite. La localisation a la peau est tres rare et fait souvent partie d’une atteinte multisystemique. Observation Une femme de 34 ans, consultait pour un nodule cutane erythemato-violace, douloureux, siegeant a l’avant-bras droit et evoluant depuis 6 mois. Une echographie abdominale avait ete pratiquee 1 mois avant l’apparition du nodule cutane, du fait de douleurs epigastriques et etait normale. Les examens histologiques et immunohistochimiques cutanes permettaient de poser le diagnostic d’hemangioendotheliome epithelioide. Le bilan d’extension, comprenant des examens radiologiques, echographiques, un scanner thoraco-abdominal et une imagerie par resonance magnetique, revelait la presence de nodules hepatiques. Le traitement consistait en une exerese large et complete de la tumeur. Apres un an d’evolution, aucune recidive locale n’etait observee et les localisations hepatiques etaient stables. Discussion L’hemangioendotheliome epithelioide appartient au groupe des tumeurs vasculaires epithelioides ayant en commun un aspect morphologique epithelioide des cellules tumorales endotheliales. La localisation a la peau est rare et a notre connaissance, seulement 20 cas d’hemangioendotheliome epithelioide avec atteinte cutanee ont ete rapportes dans la litterature. L’hemangioendotheliome epithelioide cutane peut rester isole ou rentrer dans le cadre d’une maladie multifocale avec des atteintes viscerales associees. Ainsi, la decouverte de lesions cutanees impose la pratique d’un bilan d’extension a la recherche de localisations profondes. L’hemangioendotheliome epithelioide etant considere comme une tumeur de bas grade de malignite, l’integrite du foie chez cette malade, observee un mois avant l’apparition de la maladie, est en faveur d’une maladie multicentrique plutot que de localisations metastatiques hepatiques d’une tumeur cutanee primitive.

Hailey‐Hailey disease in Tunisia

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.

Tongue hyperpigmentation during PEG-interferon-alfa/ribavirin therapy in a non-Caucasian patient with chronic hepatitis C: a case report and review of the literature

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L’apport de la cryochirurgie dans la prise en charge du xeroderma pigmentosum

BACKGROUND Basal cell carcinoma (BCC) occurs frequently in young patients with xeroderma pigmentosum (XP). Because tumours are multiple and usually found on the face, alternatives to surgery are proposed. The purpose of this study was to evaluate the efficacy of cryosurgery for BCC in XP patients. PATIENTS AND METHODS This was a retrospective study that included all instances of BCC occurring in XP patients treated by cryosurgery between January 2002 and October 2008. RESULTS Forty-five cases of primary facial BCC and one case of relapsing BCC (16 on the cheeks, 11 on the nose, six on the face and five on the inner edge of the eye area) were treated by cryosurgery in 18 XP patients (average age: 18.7 years; sex ratio: 0.5). Mean carcinoma size was 10mm. Thirty-six tumours were nodular and 10 were nodular and ulcerated. Follow-up ranged from 16 to 60 months (mean: 30 months). Complications were rare. Cosmetic results were satisfactory. We noted 10 cases of hypopigmentation (21.7%) and one notch on the nose following treatment for recurrent BCC on a radiotherapy scar. Relapse occurred in one case (2.17%). CONCLUSION Because of its simplicity, its good safety, its sparing of skin and its reliability of oncological outcome, cryosurgery is the treatment of choice for CBC in XP patients.

Atopic Dermatitis in Tunisia

Our aim was to investigate the epidemiology and clinical features of atopic dermatitis in Tunisia. To reach this object, a retrospective multicentre study was carried out at the 7 Tunisian departments of dermatology covering a period of 5 years. We observed 1,032 cases of atopic dermatitis during the studied period. These cases represent 0.52% of all new diagnoses in the same period. The mean age at onset was 3.5 years. Atopic dermatitis began after the age of 5 years in 14.05% of patients and was assessed to be severe in 5% of cases. Atopic dermatitis seems to be less frequent in Tunisia than in western countries. Its age at onset is higher, and this may be correlated with the less severe forms of the disease seen.