Pemphigus vegetans of the Nose

Abstract

Pemphigus vegetans is an unusual form of pemphigus vulgaris. Pemphigus of the head and neck region is usually found in areas with mucosa, such as the mouth and pharynx. We present a fascinating case of pemphigus vegetans arising from the nasal dorsum that completely deformed the external nose of a 43-year-old woman. The patient was treated successfully with high-dose prednisone.

Localized pemphigus vegetans of the nose and lips: A classic case of a rare entity

“…The accurate diagnosis relies on the biopsy findings, which include the vegetative benign epithelial hyperplasia, 4 suprabasilar acantholysis that exactly recapitulates the pattern seen in PV, 5 and the appropriate inflammatory milieu that one associates with pemphigus, including intraepithelial collections of eosinophils and neutrophils (intraepithelial eosinophilic microabscesses). 6 Direct immunofluorescence studies will show intercellular epidermal deposits of complement and immunoglobulin IgG, whereas C3d and C4d in an intercellular array can be seen via an immunohistochemical technique on paraffin-embedded formalin-fixed tissue.…”

“… 4 There are 4 previous reports describing nasal PV with or without concomitant lip involvement ( Table I ). 5 , 6 , 7 , 8 …”

“…She received 1 gram rituximab-pvvr biosimilar infusion × 2, 2 weeks apart. Complete clearing 3 Sigmund GA et al, 5 2012 43 y/Woman Progressively enlarging nasal mass over 19 mo An eczematous process in the lip, along the right medial canthus, extending into the upper portion of the eyelid Initial treatment with oral antibiotics and 3 weeks of IV vancomycin Subsequent diagnosis of squamous cell carcinoma and referral for partial rhinectomy. Prednisone 60 mg/d for 2 weeks and tapering over a month and maintanence with a prednisone of 30 mg/d Near-complete clearing 4 Dhamija A et al, 7 2012 45 y/ Man Recurrent painful oral erosions for 3 years.…”