Stevens Johnson syndrome-Toxic Epidermal Necrolysis Overlap induced by sulfasalazine treatment: a case report.

INTRODUCTION: Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are life-threatening and severe adverse cutaneous drug reactions characterized by epidermal detachment presenting as blisters and areas of denuded skin. SJS, SJS-TEN overlap and TEN differ only by their extent of skin detachment. CASE PRESENTATION: We report here the case of a young woman (33- year old) admitted to the dermatological unit for epidermal detachment (at 18% of the body surface area), blisters, red macular and papular lesions, developed 15 days after administration of sulfasalazine. Prior to this, she complained of fever and discomfort upon swallowing. Skin biopsy had shown epidermal necrosis compatible with Stevens Johnson Syndrome and Toxic Epidermal Necrolysis. As the epidermal detachment was between 10% and 30%, she was diagnosed as a Stevens Johnson Syndrome/Toxic Epidermal Necrolysis overlap. The course was favorable 17 days after stopping the drug and starting a symptomatic treatment. CONCLUSION: Practitioners and patients need to be aware of the initial clinical signs of severe cutaneous adverse drug reactions such as fever, influenza-like symptoms, dysphagia or burning eyes. Early discontinuation of medication remains the best way to improve prognosis of patients with Stevens Johnson's Syndrome and Toxic Epidermal Necrolysis.

Dyggve-Melchiore-Clausen dysplasia (DMC): syndrome associated with a micropenis.

Dyggve-Melchiore-Clausen (DMC) syndrome is a are autosomal recessive spondyloepimetaphyseal dysplasia associated with mental retardation resulting from mutations in the Dymeclin (DYM) gene mapped in the 18q12-12.1 chromosomal region. We report a case of a consanguineous Moroccan boy with this disease confirmed by the presence of homozygous mutation at c.1878delA of DYM gene. Our patient additionally has a micropenis. We discuss the clinical severity, difficult management of this syndrome and its association with micropenis never described before in the literature.