Evaluation of desmoglein 1 and 3 autoantibodies in pemphigus vulgaris : correlation with disease severity

Pemphigus is an autoimmune blistering disease of the skin and mucous membranes caused by autoantibodies against desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). Pemphigus vulgaris (PV) is the most common form of pemphigus. The aim of this study was to assess the correlation between the levels of anti-desmoglein 1 and 3 autoantibodies and the severity of PV disease. Nineteen newly diagnosed patients with pemphigus vulgaris were enrolled in this study. The titers of Dsg in subjects by using enzyme-linked immunosorbent assay (ELISA) were done at diagnosis time-point, 4th and 8th weeks after the initiation of treatment, and the correlation of antibodies with the oral and skin disease severity was evaluated. The severity of cutaneous lesions was significantly correlated with anti-Dsg1 titer in all visits and the severity of mucosal lesions was correlated with the titer of Dsg3 in the third visit (<0.001, 0.001, 0.016 and 0.015 P value, respectively). Anti-Dsg-1 autoantibodies titers seem to be more useful in showing the extent of the disease and activity in pemphigus with mucocutaneous lesions

Survey on Etiology of Stevens-Johnson syndrome and Toxic Epidermal Necrolysis in Pediatric Patients: A Six-Year Study from Iran

Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are among the most severe dermatologic reactions to the drugs. Data about SJS and TEN among pediatric population especially in Iran is limited. This study aimed to investigate the clinical and para-clinical characteristics of pediatric patients with SJS/TEN. Materials and Methods From 2010 to 2016, all SJS and TEN children from three teaching hospitals in Mashhad-Iran with age less than 15 years were included in the study. Patients’ catechistic, history, physical examinations, progress notes, laboratory findings, medical consults, treatments taken and the final outcome were extracted from medical records by researcher. Data were further analyzed by SPSS (version 17.0). Results Among 165 records, 48 children (58.3% male; mean age of 9.1 years) were among the SJS and TEN spectrum. Anticonvulsants (50%; including lamotrigine, phenobarbital, phenytoin, carbamazepine, valproate and clobazam) were the most common drugs followed by antibiotics (38.1%; including cefixime, penicillin, azithromycin, co-amoxiclav, cephalexin, co-trimoxazole and ceftriaxone), and analgesics (9.5%; including acetaminophen, ibuprofen and naproxen). Infectious agents were the possible cause of SJS/TEN in two patients. WBC counts, liver function tests, renal and electrolyte tests were significantly different in SJS and TEN groups. Conclusion The main suspected medications found in this study were anticonvulsants and antibiotics and the mortality rate was 12.5%. The main suspected medications found in this study were anticonvulsants and antibiotics and the mortality rate was 12.5%

A Case Report of a Successful Allergen Immunotherapy with Candida Albicans in Patient with Sever Atopic Dermatitis Sensitive to Candida Albicans

Introduction: Atopic dermatitis (AD) is an inflammatory, chronic, relapsing, itchy skin disease with an immunologic basis. This disease is associated with itchy skin lesions (pruritus), dry skin (xerosis) and plaques of eczema. The role of aeroallergens, such as house dust mite (HDM) and food allergens has been proven to exacerbate skin eczema lesions. Alongside drugs such as corticosteroids, topical emollients, antihistamines, tacrolimus, and immune suppressants, phototherapy and subcutaneous immunotherapy (SCIT) also done. SCIT is mostly using for sensitization to mite allergens.Case Presentation: We present a 30 y/o Iranian woman with severe atopic dermatitis and sensitization to Candida allergens. We initiated SCIT with candida allergen and the patient had obvious improvements in her signs and symptoms 3 months after starting SCIT.Conclusion: Although subcutaneous immunotherapy was only approved for mite sensitization in atopic dermatitis, it should be considered in other aeroallergen sensitizations

A Preliminary Study on the Therapeutic Effects of Hydroxychloroquine on Generalized Vitiligo

Vitiligo is a recalcitrant depigmentary autoimmune skin disorder. Hydroxychloroquine (HCQ) is an effective immunomodulatory drug which is widely used in treatment of autoimmune disorders. HCQ-induced pigmenta- tion has been previously found in patients taking HCQ due to other auto- immune diseases. The present study aimed to determine whether HCQ im- proves re-pigmentation of generalized vitiligo. HCQ was orally administered 400 mg daily (6.5 mg/Kg of body weight) by 15 patients with generalized vitiligo (more than 10% involvement of body surface area) for three months. Patients were evaluated monthly and skin re-pigmentation was assessed us- ing the Vitiligo Area Scoring Index (VASI). Laboratory data were obtained and repeated monthly. Fifteen patients (12 women and 3 men) with a mean age of 30.13±12.75 years were studied. After 3 months, the extent of re-pigmen- tation on all the body regions, including the upper extremities, hands, trunk, lower extremities, feet, and head and neck was significantly higher than the baseline (P value <0.001, 0.016, 0.029, <0.001, 0.006, 0.006, respectively). Patients with concomitant autoimmune diseases had significantly more re- pigmentation compared with others (P=0.020). No irregular laboratory data were observed during the study. HCQ could be an effective treatment for generalized vitiligo. The benefits are likely to be more evident in case of con- comitant autoimmune disease. The authors recommend additional large- scale controlled studies to draw further conclusions

Efficacy of Topical Liposomal Amphotericin B versus Intralesional Meglumine Antimoniate (Glucantime) in the Treatment of Cutaneous Leishmaniasis

Background. Topical treatment of cutaneous leishmaniasis is an attractive alternative avoiding toxicities of parenteral therapy while being administered through a simple painless route. Recently liposomal formulations of amphotericin B have been increasingly used in the treatment of several types of leishmaniasis. Aims. The efficacy of a topical liposomal amphotericin B formulation was compared with intralesional glucantime in the treatment of cutaneous leishmaniasis. Methods. From 110 patients, the randomly selected 50 received a topical liposomal formulation of amphotericin B into each lesion, 3–7 drops twice daily, according to the lesion's size and for 8 weeks. The other group of 60 patients received intralesional glucantime injection of 1-2 mL once a week for the same period. The clinical responses and side effects of both groups were evaluated weekly during the treatment course. Results. Per-protocol analysis showed no statistically significant difference between the two groups (P = 0.317, 95% confidence interval (CI) = 1.610 (0.632–4.101)). Moreover, after intention-to-treat analysis, the same results were seen (P = 0.650, 95% CI = 0.1.91 (0.560–2.530)). Serious post treatment side effects were not observed in either group. Conclusions. Topical liposomal amphotericin B has the same efficacy as intralesional glucantime in the treatment of cutaneous leishmaniasis

H Syndrome Masquerade as Rheumatologic Disease

Background  H syndrome is an autosomal recessive genodermatosis with a low prevalence which is caused by a mutation in SLC29A3 gene. This disorder is characterized by sclerotic, hyperpigmented, hypertrichotic cutaneous plaques with systemic involvement including: hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, low height, and hyperglycemia. Case Presentation  Here we have presented two cases of H syndrome that have been misdiagnosed and mismanaged as rheumatologic disease. The first case had been represented with sclerotic skin lesions and diagnosed as morphea, and second one with chronic and recalcitrant to treatment arthritis as juvenile idiopathic arthritis. Conclusion  H syndrome is an autosomal recessive genodermatosis that has been recently recognized with a variety of manifestations and overlapping features with other diseases. Increase the knowledge of physicians for wide spectrum manifestations of this syndrome along with reporting the misdiagnosis of this condition can increase the accuracy of physicians for its better identification. This time our cases masquerade as rheumatologic diseases