Co-amoxiclav-induced Stevens Johnson Syndrome in a child

Stevens-Johnson Syndrome is an uncommon life threatening disease generally induced by drugs. Antibiotics, mainly sulphonamides, are the most involved drugs in Stevens-Johnson Syndrome in children. Co-amoxiclav is a well tolerated antibiotic. It has never been reported to cause, lonely this syndrome in children. Herein, we report a co-amoxiclav-induced Stevens-Johnson Syndrome occurring in an 18-month-old child. The diagnosis of SJS is often challenging in children and other possible diseases should be ruled out. The etiology of this syndrome is not yet fully understood. It is thought to be mediated by an immunologic mechanism. Management involves early identification, withdrawal of the culprit drug and rapid initiation of supportive therapies.Pan African Medical Journal 2013; 14:3

Complete form pachydermoperiostosis in Tunisia – A case series and literature review

Introduction: Pachydermoperiostosis (PDP) or hypertrophic osteoarthropathy (HOA) is a rare disease that has genetic predisposition and defined clinical features. Aim of the work: To study and analyse the clinical features of Tunisian PDP patients and review the literature. Patients and methods: The PDP cases attending the Rheumatology Department of the University Hospital Farhat Hached of Sousse in Tunisia were retrospectively studied over a period of 17 years (2000–2017). Secondary causes were excluded. Results: The PDP cases had a primary and complete form of the disease and included 6 men at a frequency of 0.03% (6/20,000) of the total number of rheumatic diseases cases attending the rheumatology clinic. The mean age was 27 ± 12.3 years [18–46 years]. Five patients had arthralgia and one polyarthritis. Thickening of the skin of the head and distal extremities (pachyderma) and deep folds and furrows of the skin of the forehead was observed in all cases. Digital clubbing of the fingers and toes and spade-like enlargement of the hands and feet was noted in 5 cases. Hyperhidrosis of the hands and feet was observed in 4 cases and seborrhea in 2. Elevated acute phase reactants were found in 4 cases. Hypergammaglobulinemia was present in one case. Periostosis of the long bones was observed in all cases. Treatment consisted of analgesics in all cases, non-steroidal anti-inflammatory drugs in 2 cases and tamoxifen in 2 cases. Conclusions: Complete form of primary PDP is rarely present among rheumatic diseases patients in Tunisia. A multicenter larger number longitudinal study is recommended. Keywords: Pachydermoperiostosis, Clubbing, Hypertrophic osteoarthropathy, Periostosi

Acetaminophen-induced cellulitis-like fixed drug eruption

Acetaminophen is a widely used analgesic drug. Its adverse reactions are rare but severe. An 89-year-old man developed an indurated edematous and erythematous plaque on his left arm 1 day after acetaminophen ingestion. Cellulitis was suspected and antibiotictherapy was started but there was no improvement of the rash; there was a spectacular extension of the lesion with occurrence of flaccid vesicles and blisters in the affected sites. The diagnosis of generalized-bullous-fixed drug eruption induced by acetaminophen was considered especially with a reported history of a previous milder reaction occurring in the same site. Acetaminophen was withdrawn and the rash improved significantly. According to the Naranjo probability scale, the eruption experienced by the patient was probably due to acetaminophen. Clinicians should be aware of the ability of acetaminophen to induce fixed drug eruption that may clinically take several aspects and may be misdiagnosed

Pemphigoid gestationis: Report of 44 cases

Introduction: Pemphigoid gestationis (PG) is a rare autoimmune bullous dermatosis occurring during pregnancy and post partum. The aim of our study was to analyse epidemio-clinical, immunopathological, therapeutic and prognostic features of PG through a hospital study of 44 cases. Methods: It is a retrospective study, collecting all cases diagnosed at the dermatological department of Farhat Hached Soussa (Tunisia) from January 1990 to December 2015. Results: The disease began in 40,9% of cases at the second trimester of pregnancy and 48% at the third trimester of pregnancy, one case in post abortum. It was a polymorphic eruption, localized in the chest, the back and limbs in all cases. The face was affected in 18,2% of cases and the mucosa in one case. The skin biopsy showed a subepidermal blister in 25 cases. The direct immunofluoresence found linear C3 deposits along the basement membrane in all cases, associated to IgG deposits in 25% of cases. The treatment was mainly based on local or general corticotherapy with favourable evolution. We noted exacerbation in post partum in 17 cases and relapses during the ulterior pregnancies in 7 cases. Fetal damage was present in PG in 8 cases. Conclusion: Our study is conformed to literature data; nevertheless, it is distinguished by its appearance on the face the less frequency of palmo-plantar manifestations and the exceptional case of PG postabortum

A novel nonsense ATP2C1 mutation causes Hailey-Hailey disease in a Tunisian family

Background: Hailey-Hailey disease (HHD) is an autosomal dominant blistering skin disorder that manifests in the third to fourth decade of life. The ATP2C1 has been identified as the pathogenic gene of this disease since 2000. Materials and Methods: We report here a three generations Tunisian pedigree, where almost all males are severely affected and present with complete penetrance of HHD, while only one female shows a mild disease’s phenotype in her fourth decade. A molecular study using Whole exome sequencing and direct sequencing was performed to this family. Results: By whole exome sequencing and direct DNA sequencing, a novel nonsense mutation in ATP2C1 (c.2698A>T; p.Lys900Ter) was identified in all patients, supporting that alterations in ATP2C1 are causative of HHD. Unexpectedly, this mutation was found in one female who was initially not diagnosed for HHD. Our observations would be in line with incomplete penetrance and variable expressivity between male and female of this disease, or evidence for genetic modifiers. Conclusion: We report here a novel nonsense heterozygous mutation in ATP2C1 gene in 5 patients with HHD. Interestingly, one woman carries the nonsense ATP2C1 mutation but displays a mild phenotype of HHD. This could indicate a variation in pattern and expressivity between male and female developing HHD phenotype which should be considered when providing genetic counselling to family members carrying such mutations

Erysipèle du membre inférieur: étude de 400 cas [Erysipelas of the lower limb: study of 400 cases]

Introduction: Erysipelas is an acute, non-necrotizing dermo-hypodermitis of predominantly streptococcal origin. Objective: To clarify the epidemiological and evolutionary features of the lower limb erysipelas through a hospital series. Materiels and Methods: We retrospectively analyzed all cases of lower limb erysipelas hospitalized in the Department of Dermatology of the Farhat Hached Hospital of Sousse between January 2000 and December 2015 (10 years). Results: Four hundred cases of erysipelas of the lower limb were recorded. The mean age of the patients was 55.82 years. The sex ratio was 1.51. The main predisposing factors were sedentarity, diabetes and obesity. In 96.75% of cases, entry lesion, like mycoses or traumatic injuries was noted. Clinically, a classic presentation of erysipelas was described in all cases. Erysipela was unilateral in 96% of cases. Treatment was based on intravenous penicillin G in 86.5% of cases, on an average of 9.75 days. The evolution was favorable in 83.25% of cases. Antibioprophylaxis was prescribed in 38% of cases. Loco-regional and general complications were noted in 10.25% of cases. Three patients died. Late complications were dominated by relapses and persistence of sequelled lymphedema. RÉSUMÉ Introduction: L’érysipèle est une dermo-hypodermite aigue, non nécrosante, d’origine principalement streptococcique. Objectif: préciser les particularités épidémio-cliniques et évolutives de l’Erysipèle du membre inferieur à travers une série hospitalière. Matériel et Méthodes: Nous avons rétrospectivement analysé tous les cas d’érysipèle du membre inférieur hospitalisés dans le service de dermatologie de l’hôpital Farhat Hached de Sousse entre janvier 2000 et décembre 2015 (10 ans). Résultats: Quatre cents cas d’érysipèle du membre inférieur ont été recensés. L’âge moyen des patients était de 55.82 ans. Le sexe ratio était de 1.51. Les principaux facteurs favorisants étaient : la sédentarité, le diabète et la surcharge pondérale. Une porte d’entrée à type d’intertrigo interorteil ou de plaie traumatique était notée dans 96.75% des cas. Cliniquement, un tableau typique d’érysipèle était décrit dans tous les cas. L’atteinte était unilatérale dans 96% des cas. Le traitement était à base de pénicilline G par voie intraveineuse dans 86,5% des cas pendant en moyenne 9.75 jours. L’évolution était favorable dans 83.25% des cas. Une antibioprophylaxie était prescrite chez 38% des cas. Les complications loco-régionales et générales précoces étaient notées dans 10.25% des cas. Trois patients étaient décedés. Les complications tardives étaient dominées par les récidives et la persistance d’un lymphoedème séquellaire