I. Zaraa

Functional defects of peripheral regulatory T lymphocytes in patients with progressive vitiligo.

Auto‐reactive cytotoxic T lymphocytes play a key role in the progressive loss or destruction of melanocytes in vitiligo but the mechanism underlying the loss of self‐tolerance is unknown. A deregulation of regulatory T‐cell biology has recently been suggested. The analysis of the suppressive effects of peripheral T regulatory cells in vitiligo patients revealed a functional defect in seven of 15 cases. This defect was strongly correlated with disease activity. The evaluation of the percentage of peripheral regulatory T lymphocytes did not reveal any intrinsic quantitative defect. Yet, a decrease in the percentage of such cells was noted in patients with progressive forms, suggesting a recruitment of regulatory T cells from the peripheral blood to the site of injury. This was further corroborated by the significant increase of Forkhead box P3 expression in the vitiliginous skin of patients. Our data support the involvement of a functional defect of peripheral regulatory T cells in the pathogenesis of vitiligo and open new possibilities to advance therapeutic approaches.

Genome‐wide linkage scan for psoriasis susceptibility loci in multiplex Tunisian families

Background  Psoriasis is a relapsing chronic inflammatory skin disease affecting all population groups, with a peak prevalence of 3% in northern European and Scandinavian caucasians. Epidemiological studies have implicated a genetic component to psoriasis. In the past 12 years multiple genome‐wide linkage analyses have identified putative susceptibility loci on several chromosomes, with a major locus in the major histocompatibility complex region.

CARD14 alterations in Tunisian patients with psoriasis and further characterization in European cohorts

Rare highly penetrant gain‐of‐function mutations in caspase recruitment domain family, member 14 (CARD14) can lead to psoriasis, a chronic inflammatory disease of the skin and other organs.

Localized pemphigus: a report of three cases

Localized pemphigus: a report of three cases Dear Sir, Pemphigus is an acquired autoimmune blistering disease. It is characterized by skin and mucous membrane lesions usually spread out and rarely localized. The pathophysiology of localized pemphigus (LP) is not clearly established even though several hypotheses have been suggested. A certain number of LP cases that have developed on burns, electrocoagulation, or surgical scars, have been reported in the literature suggesting a Koebner-like phenomenon. Other authors incriminated handling of pesticides and chemicals. Recently, two cases of localized contact pemphigus had been observed following the application of ketoprofene gel and imiquimod. We describe herein three cases of LP: two cases of pemphigus foliaceous (PF) located, respectively, on the scalp and face, and one case of pemphigus vulgaris (PV) on the scalp. Clinical and laboratory findings of our patients are reported in Table 1; Fig. 1. In the course of pemphigus, the lesions may remain localized before spreading over the whole integument but it is exceptional that the condition remains localized over 5 months. Reviewing the literature, we registered only 33 cases of LP, namely 21 PV and 12 PF. In our department, between 1980 and 2006, only four cases of LP were registered among 151 cases of pemphigus (one PV of the temple already published and the remaining three cases are reported here). These findings suggest the rarity of this entity. In pemphigus, according to the extension of the skin lesions, a spectrum of forms has been individualized ranging from a localized minor form to an extensive one. Localized PV prevails on the oral mucous membranes (eight cases), the nose (five cases), and the scalp (five cases), whereas the PF involved essentially the face (nine cases) (nose: six cases). This high frequency of LP involving the face can be explained by the role of the ultraviolet (UV) radiation. It has been reported experimentally that UV radiation induced an acantholysis in patients affected with pemphigus; clinical exacerbation of the disease after sun exposure is sometimes seen in practice. Both localized PV and PF were mainly observed in adults [mean age about 46.4 years (21–76 years) and 49.9 years (27–81 years)], with no sex preponderance. The clinical distribution of lesions in pemphigus is directly

Maladie de Dowling-Degos étendue après PUVA thérapie prolongée

BACKGROUND Dowling-Degos disease is a rare and benign inherited dermatosis. PATIENTS AND METHODS A 53-year-old woman presented with generalized histologically confirmed Dowling-Degos disease revealed 8 years after psoralen photochemotherapy (PUVA) for psoriasis. This presentation was special in terms of its considerable spread as well as the absence of comedone-like and punctate scars. DISCUSSION Dowling-Degos disease is a reticulate pigmentary disorder of the flexures associating prominent comedone-like lesions and pitted scars. Diagnosis is based on clinical and histopathological examination, which allows this entity to be differentiated from other reticulate pigmentary disorders. A literature review failed to provide any indication that PUVA therapy either aggravates or reveals Dowling-Degos disease, a finding which we feel merits mention.

Dermatofibrosarcome protubérant de l’enfant

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare skin neoplasm. Usually affecting adults, the incidence in children is even less frequent. Through a report of three pediatric cases of DFSP, we describe the particularities of this tumor in children. Three boys aged 8, 9, and 15 years presented with a firm nodular skin lesion of the trunk, varying in size from 1 to 5 cm. No previous trauma event had occurred. Diagnosis was confirmed in all cases by immunohistological study. Surgical excision was performed in all cases. No recurrence was evident during the follow-up period of, respectively, 15, 36, and 49 months. The DFSP is an infiltrative tumor of intermediate malignancy, with a limited potential for metastasis (<5%) but a high rate of local recurrence (≥ 50%). The incidence in children is even less frequent. In children, its seemingly benign clinical appearance may explain delays in diagnosis; the majority of lesions affect the extremities, suggesting a potential role-played by injury. In our observations, however, as in adults, the trunk was the site of occurrence. Despite the uncertain pathogenesis of this tumor, the finding of certain characteristic histopathological features helps establish an accurate diagnosis. As in adults, surgical treatment with large surgical margins remains the best practice for children with DFSP, directly affecting the prognosis.

CO 38 : Pemphigus herpétiforme : à travers une série hospitalière

Introduction Le pemphigus herpetiforme (PH) est une forme rare de pemphigus. Le but de notre travail est d’etudier les aspects anatomo-cliniques et evolutifs du PH. Materiel et Methodes Etude retrospective incluant les patients suivis pour PH dans notre service de dermatologie entre 1989 et 2014. Resultats Nous avons colliges 10 cas de PH dont 8 femmes et 2 hommes. L’âge moyen etait de 44,4 ans (24-82 ans). Le prurit etait present chez 9 patients. Des vesiculo-bulles, a disposition herpe- tiforme, reposant sur peau erythemateuse etaient observees chez tous les patients, Un seul patient avait une atteinte muqueuse (erosion buccale). L’histologie montrait un clivage intra-epider- mique chez 5 patients associee a une acantholyse dans 4 cas et a une spongiose a eosinophile chez 3 patients. L’immunofluorescence directe montrait des depots inter-keratinocytaires d’IgG et C3 dans tous les cas. La dapsone en monotherapie a ete prescrite chez 9 patients et associee a la prednisone (0,5 mg/kg/j) dans un cas. Une remission complete a ete obtenue dans 7 cas. Une rechute de la maladie etait observee chez un seul patient. La duree moyenne du suivi etait de 13,1 mois.

Acute generalized exanthematous pustulosis induced by the essential oil of Pistacia lentiscus

Acute generalized exanthematous pustulosis (AGEP) is an uncommon pustular eruption characterized by small nonfollicular pustules on an erythematous background, sometimes associated with fever and neutrophilia. Over 90% of cases are drug‐induced; however, it can be caused in rare cases by other agents. We report two cases of AGEP secondary to ingestion of Pistacia lentiscus essential oil, the first two such cases to our knowledge. The cutaneous morphology, disease course and histological findings were consistent with a definite diagnosis of AGEP, based on the criteria of the EuroSCAR study group. These two cases highlight the need to consider herbal extracts as a potential rare cause of AGEP and to ensure the safety of herbal medicines.

P 05 : Une tuberculose qui fait parler d’elle

Introduction La tuberculose oro-pharyngee est une variante rare de la tuberculose cutanee. Nous rapportons un cas de tuberculose oro-laryngee mimant une pyostomatite vegetante. Observations Une femme âgee de 27 ans nous a ete adressee pour une gingivite erosive evoluant depuis 1 an. A l‘interrogatoire elle rapportait une dysphonie evoluant depuis 3 ans ainsi qu’une perte de poids estimee a 5 kg en 6 mois. L’examen physique trouvait de multiples pustules friables sur base erythemateuse et infiltree, siegeant sur la muqueuse labiale et gingivale. Le palais presentait un aspect franchement papillomateux voire vegetant. Le reste de l’examen etait sans particularites. Le diagnostic de pyostomatite vegetante semblait le plus probable. Un examen oto-rhino-laryn- gologique (ORL) sous anesthesie generale a ete pratique et avait note un aspect infiltre de la glotte, du nasopharynx et des cordes vocales ainsi qu’une hypertrophie du mur posterieur du pharynx. Un scanner du massif facial avait note la presence d’une infiltration du larynx, du pharynx, se rehaussant apres injection de produit de contraste. Des biopsies de la muqueuse buccale et laryngee ont revele une acanthose avec des ulcerations focales ainsi qu’un granulome tuberculoide sans necrose caseeuse au niveau du chorion. L’intra-dermo-reaction a la tuberculine etait fortement positive. La radiographie du thorax et le scanner thoracique trouvait des signes suggestifs de primo-infection tuberculeuse. La recherche du bacille de Koch etait negative dans les crachats. La patiente a beneficie de 6 mois de traitement anti-tuberculeux associant 2 mois de HRZE et 4 mois de HR. Une amelioration etait notee a partir du 3 e mois avec regression de la dysphonie et amelioration de l’etat local buccal avec une guerison complete a 6 mois. Conclusion La tuberculose oro-pharyngee reste de diagnostic difficile etant donne sa rarete et son grand polymorphisme lesionnel, pouvant etre deroutant pour le clinicien. Toute lesion chronique de la bouche devrait inciter a la pratique de biopsies au mieux guidees par une endoscopie ORL.

FOXP3 transcription is enhanced in lesional and perilesional skin of patients with focal Alopecia areata.

References 1 Wo zniak K, Kazama T, Kowalewski C. A practical technique for differentiation of subepidermal bullous diseases: localization of in vivo-bound IgG by laser scanning confocal microscopy. Arch Dermatol 2003; 139: 1007–1011. 2 Wolff K, Rappersberger K, Steiner A, et al. Vegetating cicatricial pemphigoid. A new subset of the cicatricial pemphigoid spectrum. Arch Dermatol Res 1987; 279 (Suppl.): S30–S37. 3 Wo zniak K, G orkiewicz A, Olszewska M, et al. Cicatricial pemphigoid vegetans. Int J Dermatol 2007; 46: 299–302.