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Resume Introduction Les epidermolyses bulleuses dystrophiques recessives representent un groupe de maladies genetiques, caracterisees par une fragilite cutanee et muqueuse, causees par des mutations dans le gene COL7A1. L’amylose AA constitue une complication rare de ces genodermatoses. Observations Deux patients, porteurs d’une epidermolyse bulleuse dystrophique recessive, generalisee severe dans le premier cas et generalisee intermediaire dans le deuxieme cas, ont developpe a l’âge de 38 et 28 ans, respectivement, un syndrome nephrotique. Le diagnostic d’amylose renale secondaire etait confirme par une biopsie renale dans le premier cas et des glandes salivaires dans le deuxieme cas. Le deces est survenu deux mois apres dans les deux cas. Conclusion L’amylose AA au cours des epidermolyses bulleuses dystrophiques recessives est souvent revelee par une atteinte renale caracterisee par un syndrome nephrotique et une insuffisance renale d’aggravation rapide. Le pronostic est sombre au vu de la fragilite du terrain et l’absence d’un traitement etiologique.

A 21-year-old woman presented with a 13-year history of a linear lesion on the lip. She experienced no pain and only had an esthetic complaint. Her personal and family history was otherwise unremarkable. She had no history of photosensitivity, Raynaud's phenomenon, arthralgias, dry eyes, fever, trauma, or exposure to irradiation. (

Background Hydroxychloroquine (HCQ)-induced acute generalized exanthematous pustulosis (AGEP) is poorly described in the literature. The aim of our study was to characterize the clinical, laboratory, allergological, and genetic features of HCQ-induced AGEP. Methods We conducted a retrospective study of patients with HCQ-induced AGEP diagnosed between 2011 and 2019. We performed molecular analysis to identify variations in the IL36RN gene. We also reviewed similar cases reported between 1991 and March 2020. Results Seven female patients were included. The mean age was 47 years old, and the average time from HCQ start to onset of symptoms was 40 days. All patients received topical steroids with a full resolution of the rash within an average of 39 days after HCQ withdrawal. Patch tests were performed for three patients with positive results in one case. Genetic analyses were performed for three patients, and no mutation in the IL36RN gene was identified. Conclusion The latent period and the duration for resolution of HCQ-induced AGEP may be longer than with other drugs due to the metabolic characteristics of HCQ. Mutations in the IL36RN gene were not identified in our patients.

Erysipeloid cutaneous leishmaniasis (ECL) is known as the chronic form of cutaneous leishmaniasis (CL). However, keeping its clinical presentation in view, there is a need to revisit this form of the disease.To describe ECL in view of clinical features and treatment modalities.We include a case series seen in Sfax (Southern Tunisia) from January 2017 to January 2021. All patients clinically suggestive and laboratory confirmed with a diagnosis of CL were registered. Patients of all age groups and of either gender having cutaneous lesions resembling erysipela on the face were included in the study. Different demographic features of the patients and clinical aspects were identified. Descriptive statistics were used for analysis.Of 1300 registered patients with CL, 40 (3%) were diagnosed as ECL. Ages ranged from 15 to 65 years, and duration of lesions varied from 15 to 180 days. All patients had lesions over the face. Clinically, a painful infiltrated inflammatory placard of the central facial area with a butterfly shape was observed in 14 cases, as well as zones of the cheekbone (11 cases), cheekbone and nose (5 cases), cheekbone and eyelid (8 cases), and cheekbone with ear (2 cases). Several therapeutic methods were prescribed with a sufficient result with no recurrence.ECL is a rare presentation that typically occurs on the face, looking like erysipelas, in patients who are native from an endemic region of CL.

Therapies - Sous presse. Epreuves corrigees par l'auteur. Disponible en ligne depuis le jeudi 30 mai 2019

Introduction La papillomatose confluente et reticulee de Gougerot–Carteaud (PCRGC) est un trouble de keratinisation acquis benin et rare du sujet jeune. Le but de ce travail est d’en etudier ses caracteristiques epidemio-cliniques et therapeutiques. Materiel et methodes Il s’agit d’une etude retrospective des cas de PCRGC suivis dans notre service de dermatologie entre 2016 et 2020. Nous avons recueilli les donnees epidemiologiques, cliniques et evolutives. Resultats Douze patients ont ete inclus avec un sex-ratio de 0,42. L’âge median etait 23,6 ans [15–36 ans]. L’examen montrait dans tous les cas des papules d’emblee brunâtres hyperkeratosiques et confluentes realisant des plaques reticulees. La duree moyenne d’evolution etait 10,3 mois. Un prurit leger etait observe dans 40 % des cas. Le siege des lesions etait : region inter- et sous-mammaire (90 %), cou (50 %), region interscapulaire (40 %), decollete (40 %), bras (30 %), abdomen (20 %), region axillaire (10 %) et plis du coude (10 %). Une pigmentation du menton existait chez 3 patientes amelioree sous traitement. Un patient avait un diabete recent et un autre souffrait d’une obesite. Le reste de l’examen trouvait une acne (3 cas) et un acanthosis nigricans (1 cas). Le scotch test realise chez 4 patients etait negatif. Un traitement par ketoconazole local recu par 3 patients etait inefficace. Neuf patients etaient traites par doxycycline 100 mg/jour associee a un traitement local : erythromycine (4 cas), adapalene (2 cas), ketoconazole (1 cas). La duree moyenne de traitement etait 2,2 mois. La guerison etait observee dans tous les cas. Deux recidives etaient notees a l’arret des cyclines et 1 an apres. Ces deux patients avaient recu un mois de cyclines. Discussion La PCRGC est une dermatose rare. Elle debute chez l’adolescent et l’adulte jeune. Une predominance feminine a ete notee dans notre serie. Les facteurs suggeres etiologiques sont genetiques, endocriniens, bacteriens et fongiques a Malassezia furfur. Un autre facteur important est la secretion sebacee expliquant la survenue chez des adolescents et la predilection dans les zones seborrheiques. Dans notre serie, nous n’avons pas trouve le Malassezia et le ketoconazole etait inefficace. L’association a une acne, un AN, un diabete et une obesite renforce l’hypothese endocrinienne. Le diagnostic est principalement clinique. L’atteinte du menton n’a pas ete rapportee auparavant. Les cyclines, surtout la doxycycline, sont efficaces et bien tolerees. La duree de traitement est de deux a quatre mois. Un traitement court peut expliquer les recidives dans notre serie. L’efficacite des cyclines peut etre alors consideree comme un test therapeutique et renforce la theorie bacterienne (Dietzia papillomatosis). Le pronostic est bon.

Recessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membrane, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of these genodermatosis.Two patients with recessive dystrophic epidermolysis bullosa, generalized severe in the first case and generalized intermediate in the second case, developed at the age of 38 and 28, respectively, nephrotic syndrome. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case and by minor salivary gland biopsy in the second case. Death occurred 2 months after diagnosis in both cases.Renal involvement is quite common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function renal are characteristic features. The prognosis is poor due to underlying conditions and the lack of an etiological treatment.

Background: Bacillus Calmette-Guerin (BCG) vaccine is a widely used vaccine. Management of local BCG complications differs between clinicians, and the optimal approach remains unclear. Aims: We aim to describe the epidemiological, clinical and therapeutic aspects of the BCG vaccine side effects in Sfax. Patients and Methods: This was a retrospective study of all the cases of BCG vaccine adverse reactions recorded in the Dermatology and Paediatrics Departments of Hedi Chaker University Hospital of Sfax over a period of 10 years (2005–2015). Results: Twenty cases of BCG adverse reactions were notified during the study period. Actually, 80% of the patients presented local adverse reactions. The outcome was good in all the followed patients. The rate of disseminated BCG disease was 20%. Biological tests of immunity showed a primary immunodeficiency in three cases, whereas the outcome was fatal in two cases. Conclusion: BCG vaccine adverse reactions range from mild to severe. However, the management of benign local reactions remains unclear. Disseminated BCG disease must alert clinicians to the possibility of a primary immunodeficiency.

Introduction Le lichen plan pigmentogene (LPP) est une variante rare du lichen plan, caracterisee cliniquement par une hyperpigmentation maculeuse surtout des zones photo-exposees ou des plis, et histologiquement par une dermatite lichenoide d’interface avec incontinence pigmentaire. Materiel et methodes Nous etudions retrospectivement les caracteristiques epidemio-cliniques et histologiques du LPP, a travers une serie hospitaliere sur une periode de 6 ans (2015–2020). Resultats Trente-quatre cas etaient recenses dont 25 femmes (72 %) et 9 hommes, de phototype fonce (Fitzpatrick III–V). L’âge moyen etait de 55,2 ans (12–79 ans). La duree moyenne d’evolution etait de 17,2 mois. L’aspect clinique revelait dans tous les cas des macules brun-gris a gris violace. Un prurit etait retrouve dans 32,3 %. Le schema de pigmentation le plus commun etait tachete (76,7 %) suivi de diffus (11,7 %) puis lineaire et reticulee (5,8 % chacun). Les sites les plus touches sont le visage (21 cas), surtout le front, la region periorale et periorbitaire, et le cou (12 cas). Les patients avec une atteinte du visage a disposition a type de melasma avaient une extension ailleurs. Une hypopigmentation en confettis etait trouvee chez une patiente sans utilisation de depigmentant. Neuf cas de LPP inverse concernaient plusieurs plis a la fois. Un patient avait des macules pigmentees buccales. La biopsie montrait un infiltrat lymphocytaire du derme et une incontinence pigmentaire avec des melanophages (100 %), une atrophie epidermique (64,7 %), une orthokeratose (38,2 %), une hypergranulose (17,6 %) ou une vacuolisation de la basale (61,7 %). Les associations sont dominees par les dysthyroidies (4 cas), l’alopecie frontale fibrosante (AFF) (4 cas), une cheilite lichenienne (2 cas), le lichen buccal et le lichen pilaire chacun dans un cas. La serologie des hepatites B et C, realisee dans 15 cas, etait positive dans un cas. Cinq patients avaient des anticorps antinucleaires (AAN) positifs sans signe de maladie auto-immune. Discussion Le LPP est frequent au sud asiatique mais n’est pas rare dans notre region. La maladie touchait majoritairement les femmes ce qui peut etre attribue a l’exposition solaire et a l’utilisation de cosmetiques. Notre serie est particuliere par la frequence de survenue chez des patients âges (47 % apres 50 ans). La principale plainte est esthetique avec parfois un prurit. Le LPP se caracterise par des schemas morphologiques differents : la forme tachetee est dominante dans notre serie. La forme blaschkolineaire et l’atteinte de la muqueuse buccale, exceptionnelles, etaient notees. Le LPP peut preceder l’AFF. La prevalence de l’hypothyroidie dans notre serie (16 %) est plus frequente qu’une serie Indienne (11 %). Les maladies auto-immunes n’etaient pas retrouvees malgre des AAN parfois positifs et des associations a d’autres formes de lichen qu’il faut rechercher confirmant leur appartenance au meme spectre.

Introduction Le pemphigus paraneoplasique (PPN) est une dermatose bulleuse (DB) rare pouvant toucher plusieurs organes d’ou le concept de « syndrome multi-organes auto-immun paraneoplasique » ou « PAMS ». Nous en rapportons une serie. Materiel et methodes Il s’agit d’une etude retrospective de tous les cas de PNP hospitalises au service de dermatologie du CHU Hedi Chaker de Sfax en 14 ans (2007–2020). Resultats Neuf patients (4 hommes et 5 femmes) ont ete colliges. L’âge median etait de 54,3 ans (43–80 ans). L’association d’atteinte cutanee et muqueuse constante. L’atteinte cutanee etait polymorphe a type de bulles flasques avec erosions (6 cas), de bulles tendues (1 cas), de lesions en cocarde type erytheme polymorphe (2 cas), de lesions lichenoides (3 cas), et de cheilite erosive (5 cas). Les lesions siegeaient au niveau du tronc (5 cas), des membres (5 cas), des plis (4 cas) ou du cuir chevelu (2 cas). L’atteinte muqueuse etait variable : buccale (n = 9), genitale (n = 3) et oculaire (n = 2). L’atteinte muqueuse etait inaugurale dans 7 cas. La biopsie montrait une acantholyse (n = 7), des necroses keratinocytaires (n = 3) ou une dermite d’interface (n = 5). L’IFD etait positive avec depot lineaire continu (6 cas) et/ou en maille de filet (4 cas) de C3 et/ou d’IgG de la jonction dermo-epidermique et/ou inter-keratinocytaire. L’IFI montrait des anticorps anti substance intercellulaire (3 patients). L’immunoblot montrait des autoanticorps anti-envoplakine et periplakine (2 patients). Le bilan montrait : leucemie lymphoide chronique (2 cas), lymphome non hodgkinien (1 cas), maladie de Castleman (MC) (2 cas), cancer du poumon (2 cas), de l’ovaire (1 cas) et du pancreas (1 cas). Leur decouverte precedait (3 cas), etait concomitante (1 cas) ou suivait (5 cas) le pemphigus. Les traitements prescrits etaient : corticotherapie orale (9 patients), azathioprine (2 patients), plasmapherese (1 patiente) et rituximab (RTX) (3 patients). L’exerese de la tumeur dans le cadre de la MC etait faite dans les deux cas. Cinq patients etaient decedes dans le mois suivant l’apparition des lesions par defaillance respiratoire (DR), trois avaient en une cicatrisation des lesions (dont 2 avec MC) et un gardait des erosions buccales et developpait une bronchiolite obliterante. Discussion Le PPN est une affection rare touchant dans notre serie plus de femmes. Il existe certes des ressemblances entre le PPN et d’autres DB mais le caractere polymorphe et rapidement evolutif des lesions a permis d’evoquer le diagnostic. L’atteinte muqueuse est constante et inaugurale dans 50 % des cas. Les neoplasies associees sont variees avec les hemopathies au 1er plan, conformement aux donnees de la litterature. Le PPN avec MC a un meilleur pronostic. La mortalite chez nos patients est tres elevee (55,5 %). Le RTX est une bonne alternative ameliorant l’evolution des patients. Comme dans les anciennes series, le deces est majoritairement secondaire a la DR dans le cadre du PAMS.

Introduction Le pilomatricome, est une tumeur cutanee annexielle benigne issue de la matrice pilaire. Elle est le plus souvent rencontree pendant les deux premieres decennies de la vie et localisee au niveau cervico-facial. Materiel et methodes On rapporte une serie descriptive retrospective de tous les cas de pilomatricome confirmes histologiquement pendant une periode de 10 ans (2011-2020) pour en definir ses principales caracteristiques. Resultats (si adapte) Nous avons collige 55 cas. L’âge moyen au diagnostic etait de 39,9 ans (6-76 ans). Les patients âges de moins de 20 ans representaient uniquement 20 %. Le sex-ratio etait de 0,77. Le delai moyen d’evolution etait de 32,1 mois (1 mois-60 ans). Le diagnostic de pilomatricome etait rarement evoque. Les principaux diagnostics differentiels etaient les kystes suivis par la calcinose et le dermatofibrome. La peau en regard etait normale dans 70,9 %. Dans les autres cas, elle etait erythemateuse (9 %), pigmentee (5,4 %), bleutee (3,6 %), blanchâtre (1,8 %), anetodermique (1,8 %) ou perforante/ulceree (3,6%) simulant un keratoacanthome (KA). Les principales localisations par ordre de frequence decroissant etaient les membres superieurs (47,2 %), l’extremite cervico-faciale (34,5 %), les membres inferieurs (12,7 %), le pubis (1 cas), le dos (1 cas) et les fesses (1 cas). La taille des tumeurs variait entre 0,4 et 4 cm. 7,3 % des lesions etaient geantes (> 3 cm). Une douleur etait notee chez 20% des patients. Les lesions etaient toujours solitaires. Aucune association clinique morbide n’etait trouvee. La survenue sur le site d’injection d’un vaccin au niveau du bras etait rapportee dans un cas. Quatre patients avaient beneficie d’une echographie ne montrant pas de signes specifiques. Une excision chirurgicale etait faite pour tous les patients. L’aspect histologique montrait dans tous les cas la presence de cellules basophiles et de cellules momifiees avec des calcifications (41,8 %) et une reaction inflammatoire a cellules geantes (67,2 %). Apres la chirurgie, une seule recidive etait observee 3 mois apres et un seul patient avait developpe un autre pilomatricome ailleurs. Discussion Le diagnostic du pilomatricome, souvent meconnu du fait de son polymorphisme clinique, est histologique. Conformement aux donnees de la litterature, il existe dans notre serie une predominance feminine et de la forme commune qui se presente sous forme de nodule sous-cutane, solitaire, dur, parfois douloureux et de taille inferieure a 3 cm. Les formes perforantes, erythemateuses, pigmentees, bleutees, anetodermiques et geantes sont decrites et sont presentes chez 1/3 de nos patients. La guerison sans recidive est la regle apres exerese chirurgicale. L’originalite de notre etude reside dans la predominance chez l’adulte, la predominance de l’atteinte des membres superieurs, la presence de formes perforantes et la survenue de recidive.

Background Sweet's syndrome (SS) is a neutrophilic dermatosis characterized by the abrupt onset of cutaneous, systemic and histopathological alterations in response to different stimuli. Objectives The aim of this study was to assess the epidemioclinical, histological, and therapeutic features and outcomes of SS. Methods A retrospective study of all patients diagnosed with SS over a 20-year period (1993–2012) was conducted. Data were analyzed using a level of significance of 5%. Results Ninety patients (mean age: 46.5 years) fulfilled the inclusion criteria. The ratio of women to men was 5. Significant associations emerged between dermohypodermic nodes and location on the lower limbs (P = 0.042), and vesiculobullous lesions and location on the legs (P = 0.030), dorsum of the hand (P = 0.015), and forearms (P = 0.003), and paraneoplastic forms (P = 0.012). The upper extremities were involved in the majority of patients (83.3%). Correlations were found between edema of the superficial dermis and vesiculobullous lesions and between leukocytoclastic vasculitis and atypical targetoid lesions. Sweet's syndrome was associated with cytomegalovirus infection (n = 1), inflammatory bowel disease (n = 4), neoplasm (n = 6), and pregnancy (n = 3). First-line treatment consisted of colchicine. Conclusions To the best of the present authors' knowledge, this is the largest series of SS to be reported. Clinical presentations are similar to those described in the literature. Colchicine was efficient and facilitates the reduced use of corticosteroids. The association between SS and neoplasms should be considered in the context of vesiculobullous lesions.

Background Aplasia cutis congenita (ACC) is a rare malformation characterized by absent or scarred areas of skin at birth. Although most commonly found on the scalp, ACC can also involve other locations. Its etiology and pathogenesis remain unclear. Objective To describe the epidemiologic, clinical, therapeutic, and evolutionary aspects of ACC through a hospital series. Methods We conducted a retrospective study from 1995 to 2012 and reported all cases of ACC. Results We enrolled 22 cases (14 girls and eight boys) of ACC during 18 years. The mean age at diagnosis was 5.7 years. Sixteen ACC involved the scalp, five the trunk, and one the left buttock. ACC was oval-shaped in 20 cases, triangular in one case, and linear in one case. The mean size was 4 cm. ACC was associated with bone defects in two cases, various malformations in eight (37.1%), and with syndromic malformation in three (Adams–Olivier syndrome: two cases; Goltz syndrome: one case). Conservative treatment consisting of wound dressing with vaseline was indicated in six cases. Bone reconstruction was performed in two cases. Regular follow-up and no treatment was recommended in 14 cases. Conclusion Our study emphasizes the frequent association of ACC with malformations (37.1%) and bone defects (9%).

Congenital hemangiomas (CHs) are fully developed hemangiomas that are present at birth. There are 2 possible types: rapidly involuting CHs (RICHs) and noninvoluting CHs (NICHs). We conducted a retrospective study (2008-2012) of 6 patients (2 females, 4 males) with CHs (mean age, 16 days). We analyzed the epidemiology, clinical characteristics, and clinical outcome of CHs over this 5-year period.

Background Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by dermal or subcutaneous red or brown papules or nodules, most commonly on the head and neck. Objective The aim of this study was to review the epidemiological and clinical characteristics of ALHE, focusing particularly on the histological and therapeutic features. Methods We performed a retrospective study of all cases of ALHE diagnosed in our dermatology and pathology departments between 2004 and 2015. Results Over 12 years, we collected nine cases of ALHE (0.75 case/year). There were four men and five women. The mean age was 43 years. Lesions presented as erythematous or violaceous papules or nodules in all cases, multiple in five cases, and localized on the head in eight cases or other sites in four cases. The diagnosis of ALHE was clinically suspected in only two cases. The histopathological findings showed an ill-circumscribed, intradermal slightly lobular proliferation of capillary-sized vessels around several central vessels. In all cases, the blood vessels were lined by large endothelial cells. An inflammatory infiltrate around the vessels was formed mainly of lymphocytes and eosinophils with isolated plasma cells and histiocytes. Surgery was the most common treatment in our series. Other local or general treatment has also been used with varying responses. Conclusion Angiolymphoid hyperplasia with eosinophilia is a rare epithelioid vascular tumor with a challenging clinical and histological diagnosis. Despite its benign nature, ALHE causes a therapeutic dilemma.

La Presse Medicale - In Press.Proof corrected by the author Available online since vendredi 26 septembre 2014

Few prospective studies are available on the incidence and analysis of the characteristics of adverse cutaneous drug reactions.To describe the adverse cutaneous reactions, their epidemiologic characteristics as well as the different causative drugs through a prospective hospital study.A 12-month prospective study was managed in our department of dermatology of the teaching hospital Hedi Chaker of Sfax. Requested information included patient characteristics (associated disorders), drug intake (list and chronology of the drug intake during the 3 weeks preceding the adverse reaction) and characteristics of the skin reaction (type, course). The diagnosis was based on a beam of clinical and anamnestic arguments. The drug imputability was evaluated according to the Begaud's French method.One hundred eighteen cases were collected. A prevalence of 1.08/100 among patients consulting in dermatology department was estimated. The macular and papular exanthema represented the most frequent clinical aspects (42 cases) followed by acute urticaria (23 cases), photosensitivity (19 cases) and fixed drug eruption (15 cases). Principal imputable drugs were antibiotics, mainly penicillins followed by analgesics and non-steroidal anti-inflammatory.Although it was monocentric, this study revealed a high frequency of drug-induced dermatitis with different clinical presentation. The high incidence of drug-induced dermatitis induced by antibiotics, analgesics and anti-inflammatory is due to their widespread use, often in self-medication.

Author(s): Charfi, Slim; Makni, Saloua; Amouri, Meriem; Ellouze, Sameh; Turki, Hamida; Boudawara, Tahya Sellami

Lymphangiomas are rare benign proliferations of the lymphatic system. Acquired lymphangioma circumscriptum of the vulva is induced by impaired lymph flow. We describe a new case of lymphangioma circumscriptum resulting from tuberculosis involving a lymph node. A 45-year-old female presented with swelling of the vulva for the past 8 years. She had a history of multiple left inguinal swellings successfully treated with four antituberculosis drugs. Her vulval swelling did not respond and was diagnosed as acquired lymphangioma circumscriptum. She becomes the fifth reported case of acquired lymphangioma circumscriptum of the vulva with tubercular lymphadenitis. The single antituberculosis treatment is insufficient to control lymphangioma. Ablative methods seem encouraging.

La Presse Medicale - In Press.Proof corrected by the author Available online since jeudi 24 janvier 2013