Etiology of pyrexia in pemphigus patients: A dermatologist’s enigma

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:;year=2012;volume=78;issue=6;spage=774;epage=774;aulast=Qadim

Posted in Around the Globe, News and Information
The P/P Registry has been approved by the Western Institutional Review Board (WIRB) and is actively enrolling participants.