Pemphigus Vulgaris Presented with Cheilitis

Abstract

Background. Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. In 50 to 70% of cases, the initial manifestations of pemphigus vulgaris are oral lesions which may be followed by skin lesions. But it is unusual for the disease to present with initial and solitary persistent lower lip lesions without progression to any other location. Main Observations. We report a 41-year-old woman with dry crusted lesions only on the lower lip, clinically resembling actinic cheilitis and erosive lichen planus, but histopathological evaluation showed unexpected results of suprabasal acantholysis and cleft compatible with pemphigus vulgaris. We treated her with intralesional triamcinolone 10 mg/mL for 2 sessions and 2 g cellcept daily. Patient showed excellent response and lesions resolved completely within 2 months. In one-year follow-up, there was no evidence of relapse or any additional lesion on the other sites. Conclusion. Cheilitis may be the initial and sole manifestation of pemphigus vulgaris. Localized and solitary lesions of pemphigus vulgaris can be treated and controlled without systemic corticosteroids.

Pemphigus vulgaris with vegetating feature confined to the scalp

“…presented with a case of PV localized only to the tongue for 18 years . In a survey of the English literature, 26 reports of long standing localized PV and six cases confined to the scalp have been described previously (Table ). To our knowledge, PV involving the scalp may lead to erythemas, erosions, hair loss, and tufted folliculitis .…”

Intralesional corticosteroid injection as an effective treatment method for oral lesions: a meta-analysis

“…The ICSI in these lesions is not recommended during relapse/flare periods due to inadequate effects. It seems that the given method is the most effective one in the maintenance phase; as more than 70% of the lesions are improved and the systemic corticosteroid is getting tapered (Fortuna & Mignogna, 2011;Mignogna et al, 2010;Nguyen & Ahmed, 2014;Abbas et al, 2014;Kalinska-Bienias et al, 2016). In this respect, a study in 2018 demonstrated that the ICSI in combination with immunosuppressive drugs could lead to reduced dose of corticosteroids and shortened time of clinical improvements (Mignogna et al, 2010).…”

Localized pemphigus vulgaris clinically masquerading as lichen planus and actinic keratosis

“…Such cases are called localized pemphigus, and a search of the published work found 24 localized PV cases. 1,2 Sites of predilection include the oral mucosa, the nose, the cheek and the scalp. There have been only two other reported cases of PV localized to the lip.…”

“…There have been only two other reported cases of PV localized to the lip. 1,3 Physical stimuli, including ultraviolet radiation, may also contribute to the localization.…”

“…Lichen planus and actinic keratosis should be carefully considered as differential diagnoses of localized pemphigus. 1,4 In fact, we initially diagnosed the lip lesion as lichen planus, misled by the fact that the lesion had responded favorably to topical corticosteroid. Actinic keratosis, especially when acantholysis is observed histopathologically, mimics localized pemphigus.…”