Dermoscopic approach for differential diagnosing of autoimmune bullous disease: pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus.

INTRODUCTION: Dermoscopy is a noninvasive technique for the evaluation of different pigments and microstructures of the epidermis, dermoepidermal junction, and papillary dermis that are not apparent to the naked eye, which therefore improves diagnostic accuracy. AIM OF THE STUDY: This study aims to describe the characteristic dermoscopic features of bullous diseases and analyze the characteristic dermoscopic features of bullous diseases of the skin and hair. PATIENTS AND METHODS: A descriptive study was conducted to describe and analyze the characteristic dermoscopic features of bullous diseases in the Zagazig University Hospitals. RESULTS: This study enrolled 22 patients. Dermoscopy revealed yellow hemorrhagic crusts in all patients and white yellow structure with red halo in 90.9% of patients. Pemphigus vulgaris patients were identified by the presence of dermoscopic clues such as bluish deep discoloration, tubular scaling, black dots, hair casts, hair tufts, yellow dots with whitish halos (fried egg sign) and yellow follicular pustules that are not seen in pemphigus foliaceus and IgA pemphigus. DISCUSSION: Dermoscopy is an important tool that serves as a link between clinical and histopathological diagnoses, and it can easily be used in daily practice. Several suggestive dermoscopic features can help in the differential diagnosis of autoimmune bullous disease but only after making a provisional clinical diagnosis. Dermoscopy is a very useful tool in the differentiation of pemphigus subtypes.

Treatment of striae distensae with needling therapy versus microdermabrasion with sonophoresis.

UNLABELLED: Striae distensae (SD) is a challenging cosmetic problem for which various treatment modalities have been applied. Our aim was to evaluate the efficacy and tolerability of needling therapy versus microdermabrasion with sonophoresis in the treatment of SD. MATERIALS AND METHODS: Forty female patients with SD (mean duration 2.98 ± 2.66) were enrolled in this study. Patients were assigned to two groups, Group 1 treated with needling therapy and Group 2 treated by microdermabrasion with sonophoresis. In Group 1, three sessions of needling therapy were performed for each patient with 4-week interval between the sessions, while in Group 2, ten sessions of combined microdermabrasion and sonophoresis were performed for each patient. Skin biopsies were taken from the most atrophic site stained with hematoxylin and eosin stain and Masson trichrome stains to study the histopathological changes and efficacy of treatment, respectively. RESULTS: There was a significant clinical improvement in SD in Group 1 compared with Group 2. Amount of collagen, number of fibroblasts, and epidermal thickness increased in the dermis at the end of treatment sessions (90% in Group 1 compared to 50% in Group 2). CONCLUSION: Needling therapy is an easy, safe, and economic method and considered as a suitable modality in management of striae.

Association between genes encoding components of the IL-4/IL-4 receptor pathway and dermatitis in children.

OBJECTIVE: To determine whether IL-4, IL-4Rα and STAT6 polymorphisms are associated with susceptibility to dermatitis in Egyptian children. METHODS: We genotyped three groups of children, consisting of 106 atopic dermatitis (AD) children, 95 non-AD children, and 100 of healthy controls, for IL-4 (-590 C/T), (-33 C/T), IL-4Rα (I50V), (Q576R) and STAT6 (2964 G/A), (2892 C/T) gene polymorphisms using PCR-RFLP assay. Total serum IgE and serum IL-4 levels were detected by ELISA. RESULTS: There was a non-significant association of IL-4 -590 C/T, -33 C/T polymorphisms in the children with non-AD or those with AD when compared with the controls. We identified a significant association between IL-4Rα I50V, Q576R polymorphisms and dermatitis susceptibility in AD (p=0.002, <0.001 respectively), whereas no such association was observed in non-AD group (p=0.52, 0.99 respectively). A significant association between STAT6 polymorphisms and both types of dermatitis was found. Patients who were carriers of IL4 -590C, IL-4Rα I50V G, STAT6 2964 A and STAT6 2892 T had an increased risk of AD [OR and 95% CI: 3.2 (2.5-4.2), p=0.005]. Furthermore, there was no relation between each polymorphism and serum IL-4 level (p>0.05 for each) while homozygosity for the risk alleles of IL-4, IL-4Rα and STAT6 SNPs were significantly associated with increased total IgE levels in all subjects. CONCLUSION: In Egyptian children, the IL-4Rα and the STAT6 polymorphism may play a role in susceptibility to AD. In addition, gene-gene interaction between the IL-4, the IL-4Rα and the STAT6 significantly increases an individual's susceptibility to AD.

Nonmutilating palmoplantar and periorificial kertoderma: a variant of Olmsted syndrome or a distinct entity?

BACKGROUND: Olmsted syndrome is a rare keratinization disorder characterized by mutilating palmoplantar and periorificial keratoderma as the two major diagnostic features. Some authors believe that atypical cases without this standard combination may not really belong to Olmsted syndrome. Herein, we describe two familial cases with congenital nonmutilating palmoplantar and periorificial keratoderma, and discuss their similarities and differences with Olmsted syndrome. PATIENTS: The study included two sisters who presented with focal and punctate nonmutilating palmoplantar keratoderma (PPK), periorificial hyperkeratotic plaques, and widely distributed keratotic lesions. Fragile denuded areas of the skin were found in sites exposed to trauma. Fingernails showed a characteristic form of leukonychia. RESULTS: Histopathology of plantar keratoderma showed psoriasiform hyperplasia with marked compact hyperkeratosis, while vicinity of denuded skin revealed thin parakeratotic zone and dissolution of the granular cell layer. Immunohistochemistry demonstrated suprabasal staining pattern for acidic keratin (AE1) and uniform positivity, starting four to six layers above the basal layer, for cytokeratin 10. Electron microscopy showed defective keratinization. Cytogenetic studies revealed normal karyotype and no chromosomal breakage. CONCLUSION: Our cases share Olmsted syndrome in the early onset, and the presence of symmetrical PPK, periorificial keratoderma and keratotic lesions. However, the striking nonmutilating nature of PPK and the presence of unique features in our patients suggest a newly described keratinization disorder.

Juvenile hyaline fibromatosis and infantile systemic hyalinosis: a unifying term and a proposed grading system.

BACKGROUND: It has been suggested that juvenile hyaline fibromatosis and infantile systemic hyalinosis represent different severities of the same disease. OBJECTIVE: We sought to redefine these disorders clearly to establish a common inclusive terminology. PATIENTS: The study included two children with early onset of similar pink papulonodular skin lesions and marked gingival hyperplasia. The first case was characterized by flexion contractures of the large joints, fractures, persistent diarrhea, recurrent chest infections, and retarded physical growth. The second patient had large swellings on the scalp and knees without systemic involvement. RESULTS: Radiologic examination revealed fractures and osteolytic bone lesions in the first case, and soft tissue masses in the second case. Laboratory tests showed anemia in both cases, and hypogammaglobulinemia, hypoalbuminemia, and electrolyte imbalance in the first case. Histopathological and ultrastructural evaluation demonstrated hyalinized fibrous tissue in the dermis in both cases. LIMITATIONS: Genetic studies were unavailable. CONCLUSION: Juvenile hyaline fibromatosis and infantile systemic hyalinosis share many common features that strongly support consideration of these conditions as different expressions of the same disorder. We propose a common term, "hyaline fibromatosis syndrome," which can be divided into mild, moderate, and severe subtypes.