Successful interferon-alpha 2b therapy for unremitting warts in a patient with DOCK8 deficiency.

The autosomal recessive form of the Hyper IgE syndrome (AR-HIES) with dedicator of cytokinesis 8 (DOCK8) deficiency is associated with difficult to treat persistent viral skin infections, including papilloma virus infection. Type I interferons play an important role in the defense against viruses. We examined the effect of therapy with IFN-α 2b in an 11-year old boy with DOCK8 deficiency due to a homozygous splice donor site mutation in DOCK8 intron 40. His unremitting warts showed dramatic response to IFN-α 2b therapy. Immunological studies revealed decreased circulating plasmacytoid dendritic cells (pDCs) and profound deficiency of IFN-α production by his peripheral blood mononuclear cells in response to treatment with CpG oligonucleotides. These findings indicate that underlying pDC deficiency and impaired IFN-α production may predispose to chronic viral infections in DOCK8 deficiency. IFN-α 2b therapy maybe useful in controlling recalcitrant viral infections in these patients.

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.

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.

Peeling skin syndrome: 11 cases from Saudi Arabia.

BACKGROUND: Peeling skin syndrome (PSS) is a rare genodermatoses of probable autosomal recessive inheritance. In Saudi Arabia, consanguinity of parents is common and consequently the occurrence of familial disease, including that of the skin, is not uncommon. METHODS: To characterize the clinical and pathological features of PSS in Saudi Arabia, we reviewed the medical records and clinical photographs of patients with recurring blistering diseases and conducted a histopathologic evaluation of skin biopsies to identify the site of cleavage. RESULTS: Eleven patients with PSS were seen at King Khalid National Guard Hospital in Jeddah between the years 1986 and 2005. Ages ranged between 2 and 15 years and there were 9 males (81.8%) and 2 females (18.2%). The most common presentation in the majority of patients was localized spontaneous peeling of the skin. Eight patients (72.7%) had a history of vesicles that were small, dry and peeled away. Trauma did not play a role in blister formation. All patients were local from Bedouin tribes where a family history of a similar complaint was documented in 8 cases (72.7%) and consanguinity of marriage was evident in 6 patients (54.5%). Histological examination of skin biopsies showed either intracorneal or superficial subcorneal cleavage above the granular layer in all biopsied patients. CONCLUSION: Although rare, PSS occurs in Saudi Arabia and is most likely related to consanguinity of marriages. This disease is generally mild and is characterized by intracorneal cleavage within the superficial epidermis. The disease should be recognized and not confused with other vesicobullous disease.

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.