Epidermolytic Ichthyosis without Keratin 1 or 10 Mutations: A Case Report.

In this paper, the authors report a case of an 11-year-old boy with epidermolytic ichthyosis who presented with multiple scattered erosions and typical hyperkeratotic plaques over the face, upper and lower extremities, the trunk, palms and soles. Family history revealed an affected older male sibling and an affected first-degree female relative. In addition, there was a positive history of generations of consanguinity in the patient's family pedigree, increasing the probability of an autosomal recessive inheritance. The clinical diagnosis was confirmed by histopathology; however, mutations in the keratin 1 and 10 genes were absent. This case report addresses the importance of establishing correct diagnosis and mode of inheritance, with literature review of genetic mutations, possible differential diagnosis and the most common and successful treatment modalities for epidermolytic ichthyosis.

Saudi practical guidelines on biologic treatment of psoriasis.

The current treatment of psoriasis patients with biologic agents in the Kingdom of Saudi Arabia (KSA) is mainly based on clinical experience. Although there are published international guidelines for treatment with biologics, such as the European S3 guidelines (a joint project of the European Dermatology Forum, the European Academy of Dermatology and Venereology, and the International Psoriasis Council), many nations have found it beneficial to develop country-based guidelines that incorporate specific regional aspects of therapy (legal and practical). With the expanded role of biologic agents in the treatment of psoriasis in Saudi Arabia, a need for local Saudi guidelines has become evident. Here we present a practical approach to the evidence-based clinical administration of biologics for professionals who treat patients with psoriasis.

Ecthyma gangrenosum in Saudi Arabia.

We report a diagnosis of ecthyma gangrenosum EG in 4 females of mean age 54.8 range 43-64 years, within 10 months. Severe drug reaction treated with high dose systemic corticosteroids in patient one, acute myelocytic leukemia treated with high dose dexamethasone, and multiple broad-spectrum antibiotics in patient 2 preceded the onset of EG. Patients 3 and 4 had vasculitic purpura and hemodialysis. In addition, patient 3 was receiving multiple broad-spectrum antibiotics plus anti-tuberculosis TB drugs for gastric TB, while patient 4 was on melphalan and high dose systemic corticosteroids. Pseudomonas aeruginosa was isolated from blood culture of the first 3 patients, and skin culture of patient one. Blister aspirate from patient 4 yielded Candida albicans. Factors enhancing skin invasion by pathogenic organisms in our patients were breached skin integrity, therapy with high dose corticosteroids and multiple broad-spectrum antibiotics, hematologic malignancies and chemotherapy with severe neutropenia.