Juvenile and adult vulvar pemphigoid, an under recognized entity: Case series of fourteen patients

Rashid, Hanan; Oldhoff, J M; Esajas, Martha D.; Diercks, Gilles F.H.; Pas, Hendrikus; Bolling, Maria C.; Hórvath, Barbara

doi:10.1016/j.jdcr.2021.05.017

Cited by 6 publications

(3 citation statements)

References 14 publications

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“…In a published case series of 14 patients with vulvar BP, only 50% of them were BP180 positive by ELISA. 9 Moreover, if we exclude the adult women in this study, out of the six children presented, only two had positive antibodies.…”

Section: Discussionmentioning

confidence: 99%

Vulvar erosions in a young girl

Taibo

Colmenero

Campos‐Domínguez

et al. 2023

Pediatric Dermatology

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Vulvar erosions in a young girlA 6-year-old girl presented with itchy and painful erosions on the external genitalia for the last 2 months (Figure 1). She had been previously treated with topical antifungal and low-potency corticosteroids for a couple of weeks with no response. The patient did not report systemic complaints. Her past medical history was not significant.Cutaneous examination did not reveal lesions elsewhere on the skin, oral or conjunctival mucosa. A skin biopsy was performed (Figures 2 and 3).

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Section: Discussionmentioning

confidence: 99%

Vulvar erosions in a young girl

Taibo

Colmenero

Campos‐Domínguez

et al. 2023

Pediatric Dermatology

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“…While these autoimmune dermatoses are extremely rare in the pediatric population, many authors have presented case reports on this topic. This indicates the need for a thorough differential diagnosis [ 13 – 16 ]. The study of Borghi et al.…”

Section: Discussionmentioning

confidence: 99%

The Need of Differential Diagnosis Between Vulvar Lichen Sclerosus and Autoimmune Dermatoses in Adolescent Girls

Dulska,

Bodziony,

Janik

et al. 2024

Dermatol Ther (Heidelb)

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Introduction Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which can manifest in prepubertal or adolescent patients. The prevailing theories point to autoimmune and genetic factors. The primary symptoms of VLS typically include vulvar itching, discomfort, dysuria, and constipation. Physical examination often reveals a characteristic figure 8 pattern, involving the labia minora, clitoral hood, and perianal region. However, these symptoms and the age of onset are nonspecific and require differentiation from autoimmune dermatoses such as bullous diseases, pemphigus diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. We performed this study to distinguish VLS from autoimmune dermatoses, and in doing so, uncover the underlying causes of chronic vulvar changes. This knowledge will enable healthcare providers to offer appropriate medical care to affected patients. Methods The study was conducted between July 2020 and February 2021, with a sample of 55 girls aged 2–18 years who did not have any systemic diseases. The study group was composed of 20 girls previously diagnosed with vulvar lichen sclerosus, while the control group included 35 girls without VLS. Questionnaires regarding the medical history of the children were completed by their legal guardians. Blood samples were collected and analyzed biochemically to assess human immunoglobulin A (IgA), IgG, and IgM antibodies against various substrates, including the desmosome of stratum spinosum, basement membrane zone, desmoglein 1 (DSG1), desmoglein 3 (DSG3), BP180-NC16A-4X, BP230gC, pemphigoid antigen, collagen type VII NC1, transitional epithelium, gliadin (GAF-3X), endomysium (EMA), and cellular nucleus (ANA). Results The analysis of the study group revealed that the most commonly observed signs and symptoms included: itching, soreness, burning sensations, and excoriation, as well as erythema or/and pallor of the skin and perineal mucosa. Among the assessed antibodies, only anti-GAF3x antibodies and ANA antibodies were detected. However, the results did not reach statistical significance ( p > 0.5).

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“…17 Mucous Membrane Pemphigoid Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is much less common than BP but more likely to affect the vulva and mucous membranes and difficult to diagnose and treat. 1,17,18 Early MMP is characterized by pain and irritation of the oral, ocular, and genital mucosa. 17 Blisters are less frequently seen in MMP as they rapidly progress to painful erosions.…”

Section: Bullous Pemphigoidmentioning

confidence: 99%