Monthly Archives: October 2012

Pemphigus vulgaris (PV) is a rare immunobullous dermatosis with worldwide distribution. The core manifestation of the condition is mucosal erosions and easily ruptured bullae that emerge on an apparently normal skin and mucous membranes or on an erythematous base. It is perhaps the most formidable dermatologic emergency which requires prompt treatment without which it may prove to be fatal. Though, new treatment modalities have decreased the mortality, nevertheless complications of the treatment are the main hazards presented by various clinical manifestations, and among them fever represents one of the most important presentations.

To characterize pyrexia, seventy two febrile pemphigus cases admitted to dermatology ward of a University Teaching Hospital, Tabriz University of Medical Sciences, Tabriz, Iran, were enrolled in this study, during March 2010 to February, 2011. The patients received oral therapy (oral prednisolone 1-2 mg/kg/day) and cytotoxics including azathioprine and cyclophosphamide or pulse therapy (with methyl prednisolone 500-1000 mg daily for three days and cyclophosphamide 500 mg with MESNA [2 Mercapto Ethane Sulfonate Sodium] rescue). Investigations for the management of fever included blood, cerebrospinal fluid (CSF), urine, cutaneous lesions and synovial fluid culture, gram’s and AFB (Acid Fast Bacilli) staining of sputum, complete blood count (CBC), erythrocyte sedimentation rate (ESR), chest X-ray and stool examination for ova or cyst of parasites. Statistical analysis was performed using SPSS software version 16.

Among 72 febrile pemphigus patients admitted, majority (97.2%) of them were classified as pemphigus vulgaris, with suprabasal acantholysis, while only 2.8% cases presented with pemphigus foliaceous, with more superficial (subcorneal) acantholysis. Though not significant, 56.9% of patients were females. Mean age of cases was 45.31 ± 16.75. Mean interval since diagnosis of pemphigus (and initiation of treatment) to the presence of fever was 5.72 ± 4.97 days. Oral therapy was prescribed to 91.7% of patients, while 8.3% received pulse therapy. The prime etiology of pyrexia was the presence of infection at various sites including: cutaneous lesions (19.4%), pulmonary infections (15.27%), urinary tract infections (11.1%) and gastroenteritis (5.5%). No patient was found positive for the presence of mycobacterium morphology on AFB smear of sputum. Staphylococcus aureus infection was revealed in 82.9% of cases with cutaneous erosions.

Full article can be found here: http://www.ijdvl.com/article.asp?issn=0378-6323;year=2012;volume=78;issue=6;spage=774;epage=774;aulast=Qadim

Paraneoplastic pemphigus (PNP) is a rare, life-threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the

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clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus-like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological-related neoplasms in 11 patients, with Castleman’s disease (n = 4) as the most frequent. Twelve patients were followed for 5–148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2-year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.

Full Article available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1346-8138.2012.01655.x/abstract