Pemphigus vegetans with isolated involvement of the nose and chest: rare variant of pemphigus vulgaris

Abstract

Pemphigus vulgaris is chronic bullous disease that manifests as bullae and erosions of skin and mucosas, with intraepidermal suprabasal cleft formation seen in the histological examination. It has a rare variant called pemphigus vegetans, where vesicles and bullae are replaced by pustular, verrucous and hyperpigmented lesions, mainly in skin folds. The treatment is similar to that for classic pemphigus vulgaris. The authors present an exuberant case of pemphigus vegetans, covering the nose and chest exclusively, without oral or flexural lesions.

A rare presentation of pemphigus vegetans as an isolated vegetative lesion—Review of histopathological clues and treatment effects in multiple biopsy specimens

“…Cerebriform tongue, an oral manifestation, has been reported 4 . Non‐flexural sites are not commonly affected, but case reports of lesions over the nose, 5 breast, 6 limbs, and digits have been documented 7–10 . Two clinical subtypes, the Neumann type and the Hallopeau type, are historically recognized 4 .…”

Case report: Mounded and refractory keratoses (MARK), a novel presentation of pemphigus vulgaris

“…Although it might be reasonable to categorize MARK as a subtype of pemphigus vegetans, it is not the goal of the authors to present MARK as a subtype of pemphigus. Interestingly, upon review of the pemphigus vegetans literature, we could identify two prior cases that appeared to have lesions consistent with MARK ( 10 , 11 ). However, strictly speaking, MARK does not overlap with pemphigus vegetans as it is most commonly defined now.…”

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. The presence of desmoglein 3 autoantibodies confirms the diagnosis of PVeg, although other antibodies potentially reflective of epitope spreading, including antibodies against desmocollin 1, desmocollin 2, and periplakin, can also be seen.…”

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

“…Associated with intranasal heroin abuse Philtrum and lips without intraoral involvement A nasal biopsy showed classic features of PVeg along with a corroborative direct immunofluorescent profile. Prednisone 40 mg/day Complete clearing 6 Feitoza RIC et al, 6 2019 89 y /Man Crusted vegetative lesion in the anterior nasal cavity and on the nasal tip evolved within 3 mo Erythematous plaque, ulcerations, thick crusts, and flaccid bulla with purulent content in chest No improvement after initial antibiotic therapy Referred for suspected skin cancer. Prednisone 1 mg/kg/day Complete remission IV , Intravenous; mo , months; PET-CT , positron emission tomography-computed tomography; wk , week; y , year.…”