We present the case of a female in her 70s who presented with a solitary verrucous plaque on her left leg accompanied by painful oral erosions. Various differential diagnoses were considered, like lichen simplex chronicus, hypertrophic lichen planus, and chromoblastomycosis. We diagnosed pemphigus vegetans (PVeg) on a nonintertriginous site through comprehensive clinical examination and histopathological and immunopathological evaluations. This case highlights the importance of considering PVeg in the differential diagnosis of solitary verrucous plaques, even in atypical extra-flexural anatomical locations.

Pemphigus vegetans is a rare type of pemphigus characterized by vegetative lesions primarily localized to the intertriginous area. Despite its unique clinical presentation, the underlying pathomechanism remains unclear owing to the rarity of the disease. We report a case of pemphigus vegetans with antibodies against desmoglein 1 and desmocollins 1-3. Furthermore, immunohistochemical analyses were performed to address the pathogenesis of this disease. A 73-year-old man presented with multiple vegetative plaques with erythema on the trunk, groins, and extremities. Mucosal lesions were not observed. Laboratory examinations revealed mild leukocytosis with eosinophilia. A histopathological examination of the skin lesion showed epidermal hyperplasia and intraepidermal abscesses with marked infiltration of neutrophils and eosinophils, and infiltration of lymphocytes and eosinophils into the upper derms. Bacterial culture of the skin tissue was positive for Staphylococcus aureus. Direct immunofluorescence showed deposits of IgG and C3 on keratinocyte surfaces in the epidermis. Autoantibodies against desmoglein 1 and autoantibodies against desmocollin 1, desmocollin 2, and desmocollin 3 were detected by enzyme-linked immunosorbent assays. The diagnosis of pemphigus vegetans was made. Initiation of prednisolone (1.0 mg/kg/day) gradually improved his skin symptoms. We performed immunohistochemical analyses of the lesional skin, which revealed infiltration of CD3-positive, CD4-positive, and CD68-positive cells in the upper dermis, but CD20- or CD56-positive cells were negative. In addition, the present case showed more prominent infiltration of IL-17A- and IL-22-positive cells in the upper dermis than in pemphigus foliaceus, a type of pemphigus with autoantibodies against desmoglein 1. Furthermore, these cells co-expressed CD3 and CD68. We hypothesized that IL-22 and IL-17A produced by T cells and macrophages and their dysregulation might be involved in the pathogenesis of pemphigus vegetans. Additionally, skin colonization and/or infection with Staphylococcus aureus could potentially contribute to the pathogenesis of the disease.

BACKGROUND: Cases of autoimmune bullous dermatosis (AIBD) have been reported following COVID-19 vaccination.
OBJECTIVE: We aimed to provide an overview of clinical characteristics, treatments, and outcomes of AIBDs following COVID-19 vaccination.
METHODS: We conducted a systematic review and searched the Embase, Cochrane Library, and Medline databases from their inception to 27 March 2024. We included all studies reporting ≥ 1 patient who developed new-onset AIBD or experienced flare of AIBD following at least one dose of any COVID-19 vaccine.
RESULTS: We included 98 studies with 229 patients in the new-onset group and 216 in the flare group. Among the new-onset cases, bullous pemphigoid (BP) was the most frequently reported subtype. Notably, mRNA vaccines were commonly associated with the development of AIBD. Regarding the flare group, pemphigus was the most frequently reported subtype, with the mRNA vaccines being the predominant vaccine type. The onset of AIBD ranged from 1 to 123 days post-vaccination, with most patients displaying favorable outcomes and showing improvement or resolution from 1 week to 8 months after treatment initiation.
CONCLUSIONS: Both new-onset AIBD and exacerbation of pre-existing AIBD may occur following COVID-19 vaccination. Healthcare practitioners should be alert, and post-vaccination monitoring may be essential.

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Pemphigus vulgaris (PV) is a rare immunobullous disease. Although it classically presents as generalized flaccid blisters affecting the skin and mucosae, atypical cases of PV can be diagnostically challenging. Herein, we report an underrecognized non-blistering manifestation of pemphigus vulgaris, which we call mounded and refractory keratoses (MARK). MARK presents as exuberant scaling plaques on the scalp, often in the skin of color. When MARK features are present, pemphigus vulgaris is prone to misdiagnosis, clinically and histopathologically, leading to delays in appropriate treatment. Specifically, biopsies from these patients may resemble acantholytic dyskeratosis, resulting in initial misdiagnosis. Thus, recognizing this presentation may aid physicians in diagnosing and monitoring the recurrence of pemphigus vulgaris.

BACKGROUND: Pemphigus is a group of immunobullous dermatoses characterized by the presence of autoantibodies directed against adhesion molecules of keratinocytes, with pemphigus vegetans being the rarest form, accounting for 1%-2% of all cases of pemphigus. Pemphigus vegetans is characterized by verrucous vegetative lesions in addition to vesiculobullous lesions.
METHODS: We report a rare case of pemphigus vegetans presenting as an isolated vegetative lesion in the groin 3 months prior to the development of blisters. Owing to the atypical presentation, multiple biopsies were performed before and after corticosteroid treatment.
RESULTS: Comparing the histopathology of pre-treatment and post-treatment biopsy specimens, the resolution of intraepidermal microabscesses, and reduction in intraepidermal and dermal inflammatory infiltrates, spongiosis and interface change, attributable to treatment, were noted. However, direct immunofluorescence showed persistent intracellular intraepidermal deposition of IgG and C3 2 weeks into treatment, despite near-complete resolution of blisters on clinical examination. Clinical regression of the vegetative lesion was noted only after 6 weeks into corticosteroid treatment, while histopathological evidence of treatment was apparent at the second week.
CONCLUSIONS: The current case illustrates the importance of a liberal use of immunofluorescence studies in establishing the uncommon yet significant diagnosis of pemphigus vegetans, particularly for vegetative lesions that are persistent, in the intertriginous areas and/or in the setting of concurrent cutaneous or mucosal symptoms.

Pemphigus vegetans is a rare variant of pemphigus vulgaris, characterized by vegetating lesions primarily in the flexures. A 41-year-old male patient presented with pemphigus vegetans highly mimicking condylomata acuminata, which led to mistreatment. Careful analysis of clinical and laboratory findings enabled us to reach a correct diagnosis and successful treatment.