Mohammad Ali El-Darouti

Basaloid follicular hamartoma

Basaloid follicular hamartoma (BFH) is a unique benign follicular hamartoma characterized by variable clinical presentations, identical histologic features and possible associations with numerous disorders.

Muckle–Wells syndrome: Report of six cases with hyperpigmented sclerodermoid skin lesions

Muckle–Wells syndrome (MWS) is a rare syndrome, characterized by chronic recurrent urticaria, often combined with fever, chills, rigors, malaise, and arthralgia. Progressive sensorineural deafness, and, in approximately one third of the patients, amyloidosis of the kidneys as well as of other organs may occur. It was first described in 1962 by Muckle and Wells.

Treatment of dystrophic epidermolysis bullosa with bone marrow non‐hematopoeitic stem cells: a randomized controlled trial

Patients with dystrophic epidermolysis bullosa (DEB) have mutations in type VII collagen gene. Type VII collagen is synthesized by keratinocytes and fibroblasts. Based on the ability of bone marrow non‐hematopoeitic stem cells (NHBMSC) to develop into fibroblasts, we decided to investigate the use of NHBMSC in the treatment of recessive DEB (RDEB). This study included fourteen patients with RDEB; the first seven of them were given cyclosporine after the infusion of NHBMSC. As cyclosporine has been used for the treatment of RDEB we decided not to use cyclosporine for the second group of seven patients. Skin biopsies from the lesions were studied by electron microscopy before and after treatment. The number of new blisters decreased significantly after treatment in both groups (p = 0.003 and 0.004 respectively) and the rate of healing of new blisters became significantly faster after treatment in both groups (p < 0.001) with no significant difference between the two groups. Electron microscopic examination revealed increased number of anchoring fibrils after treatment in both groups. No major side effects were reported during the 1‐year follow‐up period. Our findings highlight the efficacy as well as the safety of NHBMSC in the treatment of RDEB.

Leukocytoclastic vasculitis and necrolytic acral erythema in patients with hepatitis C infection: Do viral load and viral genotype play a role?

BACKGROUND Leukocytoclastic vasculitis (LCV) and necrolytic acral erythema (NAE) are skin disorders associated with hepatitis C virus (HCV) infection. However, they have not been found to occur simultaneously in the same patient. OBJECTIVE We sought to analyze the role of serum HCV-RNA levels and HCV genotype in the pathogenesis of both LCV and NAE in an attempt to assess whether these two parameters play a role in mutual exclusivity of LCV and NAE in the same patient. METHODS The study included 11 patients with LCV and 13 with NAE, all of whom were infected with HCV. All 24 patients were evaluated for the quantitative levels of HCV-RNA, using real-time polymerase chain reaction. HCV genotyping was performed on 10 patients in each group (N = 20). RESULTS Patients with LCV had a higher prevalence of moderate and high levels of HCV-RNA viremia (P = .038) than those with NAE. However, there was no significant difference in HCV genotype between LCV and NAE groups (P = .211). LIMITATIONS Small number of cases is a limitation. CONCLUSION Viral load seems to play a role in determining the response of the skin to HCV infection. High levels of HCV viremia were found to be significantly associated with LCV but not with NAE. HCV viremia may play a role in the development of LCV in HCV-infected patients.

Psoriasis: Highlights on Pathogenesis, Adjuvant Therapy and Treatment of Resistant and Problematic Cases (Part I)

Psoriasis is a common inflammatory disease of the skin and joints. Its etiology has been linked to complex interactions between predisposing genes and the environment. The pathophysiology of psoriasis is characterized by epidermal hyperproliferation, enhanced antigen presentation, T-helper1 cytokine production, T-cell expansion, and angiogenesis. Tremendous advances in our understanding of this disorder have led to the development of novel therapeutics. In this review, we focus on specific advances in our understanding of the pathogenesis and the unrecognized severe effects of psoriasis, and the systemic treatment of resistant and problematic cases that are of major clinical relevance to the clinician.

Pachyonychia congenita: treatment of the thickened nails and palmoplantar circumscribed callosities with urea 40% paste

JEADV 2006, 18, 602–634

Histopathological study of apparently normal skin of patients with leprosy

Background  Several clinical and laboratory observations point to the possible microscopical affection of normal‐looking skin in leprosy.

Mycophenolate mofetil: a novel immunosuppressant in the treatment of dystrophic epidermolysis bullosa, a randomized controlled trial

Background: No effective treatment has been found for epidermolysis bullosa dystrophica (EBD). Objective: To evaluate the efficacy and safety mycophenolate mofetil (MMF) in treating EBD. Methods: This randomized controlled double-blinded study included 35 patients with severe generalized EBD. Patients were randomly divided into two groups: group I (18 patients) received cyclosporine therapy (5 mg/kg/day) and group II (17 patients) received MMF therapy (500–1500 mg/day). Clinical assessment was made weekly for 3 months from the start of the treatment. Patients were assessed by measuring the extent of the disease, the % of improvement, assessing the number of new blister formation and the time of complete healing of new blisters. Side effects were recorded when detected. Results: The % of improvement in the disease extent was statistically significantly higher (p = 0.009) in group I (mean ± SD: 59.21 ± 22.676) than in group II (mean ± SD: 44.03 ± 25.71). As regards the number of new blisters and the rate of healing of blisters, there was no statistically significant difference between both groups (p = 0.693 and 0.404, respectively). No serious side effects were reported. Conclusion: MMF seems to be a good therapeutic option for the long-term treatment of EBD, it can be a good alternative for patients who cannot tolerate cyclosporine.

Comparative study of the effect of a daily steroid regimen versus a weekly oral pulse steroid regimen on morphological changes, blood sugar, bone mineral density and suprarenal gland activity.

Abstract Background: The most serious side effects of systemic steroids include osteoporosis and suprarenal suppression. Many steroid regimens have been suggested to minimize these side effects; one of them is oral steroid pulse therapy. Objective: To compare the side effects of a daily oral steroid regimen versus a weekly oral steroid pulse regimen on bone mineral density and suprarenal suppression. Methods: Thirty patients with different skin diseases were divided into two groups: 15 for oral daily steroids (ODS) (group 1) and 15 for weekly oral pulse steroids (WOPS) (group 2). They were evaluated for bone mineral density (measured by DEXA) and suprarenal suppression (measured by serum cortisol level), morphological changes and blood sugar. Treatment was continued for 6 months to 3 years. Results: Cushingoid features in group 1 were observed in 73%, yet they were not detectable in group 2. Disturbed blood sugar in group 1 was 33% and 0% in group 2. The serum cortisol level was lower in patients on ODS than those on WOPS. The effect of WOPS on bone mineral density was very limited in comparison with the ODS. Conclusion: Weekly oral steroid pulse therapy induces no significant bone loss and no suprarenal suppression and can be an alternative option in the treatment of chronic disorders requiring long-term oral steroid therapy.

Hyperpigmented, hypertrichotic, and sclerodermoid plaques: An unusual variant of Muckle―Wells syndrome

To the Editor: We read with great interest the article by Molho-Pessach et al in the July 2008 issue of the Journal entitled ‘‘The H syndrome: A genodermatosis characterized by indurated, hyperpigmented, and hypertrichotic skin with systemic manifestations,’’ in which the authors described 11 patients with unique features including progressive sclerodermatous thickening, mainly of the skin of the middle and lower parts of the body, with overlying hyperpigmentation and hypertrichosis. Short stature, sensorineural deafness, and hepatosplenomegally were important additional features. All of the patients had markedly elevated erythrocyte sedimentation rates (ESRs). All cutaneous histopathologic examinations showed a thickened hyperpigmented epidermis, dermal and subcutaneous fibrosis, and plasma cell and mast cell infiltration. The authors suggested the title ‘‘H syndrome’’ to highlight the characteristic features of the skin changes—namely, hyperpigmentation and hypertrichosis. They stated that such a constellation of clinical and histopathologic findings were not reported before except in three recently published reports.