Desmoglein ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris

Anti-desmoglein 3 and 1 autoantibodies are involved in the pathogenesis of pemphigus diseases. Our objective was to assess the value of ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris. Based on clinical presentation and histopathologic confirmation for the diagnosis of the pemphigus, 38 patients took part in the study. Sera of the patients were tested by desmoglein 1 and desmoglein 3 ELISA. Also, direct immunofluorescence was performed for all patients which revealed positive results in 36 patients [94.7%]. ELISA was positive in 37 of 38 pemphigus patients [Sensitivity: 97.3%]. The relationship between desmoglein 1 index values and skin severity was statistically significant [p<0.05]. Desmoglein 3 index values increased with oral severity although this was not statistically significant. Iranian patients similar to Indian patients had higher positive anti-desmoglein 1 autoantibodies. Desmoglein-ELISA test is appropriate in the diagnosis of pemphigus. Desmoglein 1 index value is statistically correlated with the severity of pemphigus vulgaris

Microscopic nikolsky's sign: Is it useful for diagnosis of pemphigus vulgaris?

Pemphigus vulgaris [PV] is an autoimmune blistering disease, caused by autoantibodies against desmoglein [Dsg] 3 and/or Dsg 1 which induce the loss of adhesion between keratinocytes. Nikolsky's sign is the ability to induce peripheral extension of a blister as a consequence of applying lateral pressure to the border of on intact blister. If the weakening of the intercellular adhesion is present but not marked, then the damage may be demonstrated only microscopically [microscopic Nikolsky's sign and can increase the sensitivity of the histopathological studies. We studied 40 patients and divided them randomly into two groups [A, B]. Group A were subjected to the tangential pressure over the perilesional skin before a biopsy specimen was taken from that site; group B patients were subjected to a biopsy without the tangential pressure technique. Histopathological changes of pemphigus vulgaris were present in 30% of the patients in group A and 5% of the patients in group B. They were not statistically different. The presence of microscopic Nikolsky's sign was significantly higher in patients with generalized disease. Microscopic Nikolsky sign can increase the sensitivity of histologic diagnosis of PV

[ relation between thiopurine methyl transferase activity and efficacy and side effects of azathioprine in patients with pemphigus vulgaris]

Azathioprine is the most widely used immunosuppressive agent as an adjunct to corticosteroids in the treatment of pemphigus vulgaris [PV]. Thiopurine methyl transferase [TPMT] is a key enzyme in azathioprine metabolism and a genetic polymorphism controls its activity in human tissue. TPMT activity can provide a rational basis to determine suitable dose of azathioprine, theoretically. The aim of this study was to evaluate the clinical relevancy of this hypothesis in PV patients. In this cross sectional study in Razi Hospital, the activity of TPMT in the red blood cells of 52 PV patients who received azathioprine for at least 12 months and 29 PV patients who did not receive this drug was measured and correlated to the clinical response and side effects observed. The mean of TPMT activity was not significantly different in patients with unfavourable response, comparing to patients with favorable response to azathioprine [P=0.087]. No relationship was observed between total dose of corticosteroid and TPMT activity [r=0.089, P=0.583]. There was no difference between the mean of TPMT activity in patients receiving azathioprine and those not receiving this drug [P=0.36]. A direct relationship was not observed between TPMT activity and clinical efficacy and side effects in PV patients under treatment with azathioprine. Larger prospective studies in more homogenous patients are needed to evaluate the clinical relevance of TPMT polymorphism and to determine accurate azathioprine dosing guidelines based on TPMT activity

[Identification of mycobacteria species in cutaneous lesions of sarcoidosis by PCR-restriction ragment length polymorphism [PCR-RFLP] method]

Sarcoidosis is a granulomatous multisystem disease of unknown etiology. It has recently been tired to detect Mycobacteria genome in biopsy specimens of patients with sarcoidosis by Polymorphism chain reaction method. To detect and identify Mycobacteria species in cutaneous lesions of the patients with sarcoidosis by PCR-RFLP. 20 patients with clinical diagnosis of sarcoidosis were enrolled in this study. Clinical manifestations, appearance of naked granuloma under light microscope and exclusion of other diagnoses confirmed the diagnosis of sarcoidosis in the patients. By PCR-RFLP, genome of Mycobacteria species was searched in paraffin embedded specimen of skin biopsies of the patients. Four PCR positive skin biopsy specimens of patients with cutaneous tuberculosis were used as positive control. 10 skin biopsy specimens with other than tuberculosis were used as negative control. Mycobacteria genome was not detected in any specimens of the patients. Our findings do not support the role of Mycobacteria species in the pathogenesis of sarcoidosis