Vulvar Cicatricial Pemphigoid of Childhood

Schoeffler, A.; Roth, B.; Causeret, A S; Kanitakis, Jean; Faure, Michel; Claudy, A

doi:10.1111/j.0736-8046.2004.21110.x

Cited by 23 publications

(7 citation statements)

References 15 publications

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“…Topical tacrolimus was reported to be effective as monotherapy in one case report of juvenile MMP 140 . In two case reports, topical corticosteroids were ineffective in controlling the progression of juvenile MMP, and dapsone was added 141,142 . Farrell et al .…”

Section: Managementmentioning

confidence: 99%

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II

Schmidt

Rashid

Marzano

et al. 2021

Acad Dermatol Venereol

112

139

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This guideline has been initiated by the task force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology, including physicians from all relevant disciplines and patient organizations. It is a S3 consensus‐based guideline that systematically reviewed the literature on mucous membrane pemphigoid (MMP) in the MEDLINE and EMBASE databases until June 2019, with no limitations on language. While the first part of this guideline addressed methodology, as well as epidemiology, terminology, aetiology, clinical presentation and outcome measures in MMP, the second part presents the diagnostics and management of MMP. MMP should be suspected in cases with predominant mucosal lesions. Direct immunofluorescence microscopy to detect tissue‐bound IgG, IgA and/or complement C3, combined with serological testing for circulating autoantibodies are recommended. In most patients, serum autoantibodies are present only in low levels and in variable proportions, depending on the clinical sites involved. Circulating autoantibodies are determined by indirect IF assays using tissue substrates, or ELISA using different recombinant forms of the target antigens or immunoblotting using different substrates. The major target antigen in MMP is type XVII collagen (BP180), although in 10–25% of patients laminin 332 is recognized. In 25–30% of MMP patients with anti‐laminin 332 reactivity, malignancies have been associated. As first‐line treatment of mild/moderate MMP, dapsone, methotrexate or tetracyclines and/or topical corticosteroids are recommended. For severe MMP, dapsone and oral or intravenous cyclophosphamide and/or oral corticosteroids are recommended as first‐line regimens. Additional recommendations are given, tailored to treatment of single‐site MMP such as oral, ocular, laryngeal, oesophageal and genital MMP, as well as the diagnosis of ocular MMP. Treatment recommendations are limited by the complete lack of high‐quality randomized controlled trials.

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Section: Managementmentioning

confidence: 99%

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II

Schmidt

Rashid

Marzano

et al. 2021

Acad Dermatol Venereol

112

139

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“…CP is a rare condition in children as only 18 cases (including ours) reported so far in the English literature (Table 1) (1–16). The majority of the cases have occurred in female patients, with 14 girls and 4 boys, aged between 20 months (our patient) and 18 years, with a mean of 10.3 years.…”

Section: Discussionmentioning

confidence: 87%

“…Oral synechia in CP may be observed but they rarely lead to functional disturbance, as in our patient who had limitation of the buccal opening because of bilateral fibrosis of lip‐commissures. Vulvar involvement has been reported in 8 cases and it was confined to the vulva in three of them (3,12,13). In such cases, other differential diagnosis can be discussed, such as childhood bullous pemphigoid, which can also be confined to the vulva, and lichen sclerosis (17).…”

Section: Discussionmentioning

confidence: 99%

“…As in vulvar bullous pemphigoid, topical tacrolimus can also be used (18). However, more severe cases had required systemic therapy with prednisolone, dapsone, or azathioprine, or associated therapy with two or three of these drugs (3,12,13). Erythromycin can be used as a steroid‐sparing therapeutic adjunct and is also much more tolerated than dapsone (19).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Early Onset Childhood Cicatricial Pemphigoid: A Case Report and Review of the Literature

Kharfi¹,

Khaled²,

Anane

et al. 2010

Pediatric Dermatology

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Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation.

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“…Vulvar alandaki plaklar zamanla skar ve sineşi gelişimine, bunun sonucunda da genitoüriner disfonksiyona yol açabilmektedir. Kaşıntı, yanma, vulvar ağrı ve dizüri hastalığın temel semptomlarıdır 4 . Olgumuzda da dizüri ve ağrı mevcuttu.…”

Section: Discussionunclassified

Vulvar Sikatrisyel Pemfigoid: Bir Çocuk Olgu

Günaydın¹,

Türk²,

Kandıloğlu³

et al. 2014

TURKDERM

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Türk derm-De ri Has ta lık la rı ve Fren gi Ar şi vi Der gi si, Ga le nos Ya yı ne vi ta ra f›n dan ba s›l m›fl t›r. Turk derm-Arc hi ves of the Tur kish Der ma to logy and Ve ne ro logy, pub lis hed by Ga le nos Pub lis hing. ÖzetVulvar sikatrisyel pemfigoid, büllöz pemfigoidin çocukluk çağında görülen, izole ve lokalize vulvar tutulumla karakterize nadir bir varyantıdır. Sum maryVulvar cicatricial pemphigoid is a rare variant of bullous pemphigoid, which is characterized by isolated and localized vulvar involvement, observed in childhood. The typical clinical finding is erosive lesions of the vulva healing with scar formation. Ocular involvement may be associated with vulvar disease or may develop during the follow-up. On histopathologic examination of the lesions, subepidermal blister and on direct immunofluorescence study, linear deposition of Ig G and C3 are observed. The differential diagnosis includes lichen sclerosus and, sexual abuse should be considered at first. Topical and systemic steroids and dapsone are the main treatment options. Ophthalmological examination and follow-up should be repeated on every six months for ocular involvement. Here, we report development of vulvar cicatricial pemphigoid in an 11-year-old girl with clinical and histopathological findings. (Turkderm 2014; 48: 51-3)

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Vulvar Cicatricial Pemphigoid of Childhood

Cited by 23 publications

References 15 publications

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II

Early Onset Childhood Cicatricial Pemphigoid: A Case Report and Review of the Literature

Vulvar Sikatrisyel Pemfigoid: Bir Çocuk Olgu

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