Cicatricial Pemphigoid: A Case of Onset at Age 5

Moy, Wesley J.; Kumar, Vijay; Friedman, RobertJ.; Schaeffer, Madeline; Beutner, Ernst E.; Helm, Frederick

doi:10.1902/jop.1986.57.1.39

Cited by 14 publications

(6 citation statements)

References 27 publications

(2 reference statements)

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“…MMP is predominantly a disease of women, with a mean age at onset of 51–62 years (Shklar and McCarthy, 1959; Hardy et al , 1971; Ahmed and Hombal, 1986; Silverman et al , 1986). Children are rarely affected, to date only 10 cases in the have been reported in the English literature presenting primarily as oral mucosal lesions (Moy et al , 1986; Wojnarowska et al , 1988; Sklavounou and Laskaris, 1990; Cheng et al , 2001; Musa et al , 2002; Kuenzli et al , 2004).…”

Section: Epidemiology Of Mmpmentioning

confidence: 99%

Number III Mucous membrane pemphigoid

2005

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Mucous membrane pemphigoid (MMP) is a sub-epithelial vesiculobullous disorder. It is now quite evident that a number of sub-epithelial vesiculobullous disorders may produce similar clinical pictures, and also that a range of variants of MMP exist, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated sub-epithelial blistering diseases (IMSEBD) has therefore been used. Immunological differences may account for the significant differences in their clinical presentation and responses to therapy, but unfortunately data on this are few. The diagnosis and management of IMSEBD on clinical grounds alone is impossible and a full history, general, and oral examination, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. No single treatment regimen reliably controls all these disorders, and it is not known if the specific subsets of MMP will respond to different drugs. Currently, apart from improving oral hygiene, immunomodulatory-especially immunosuppressive-therapy is typically used to control oral lesions. The present paper reviews pemphigoid, describing the present understanding of this fascinating clinical phenotype, summarising the increasing number of subsets with sometimes-different natural histories and immunological features, and outlining current clinical practice.

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Section: Epidemiology Of Mmpmentioning

confidence: 99%

Number III Mucous membrane pemphigoid

2005

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“…There are only 12 childhood CP cases, including ours, reported in the English language literature ( Table 1) and five of them are oral pemphigoid patients (12)(13)(14)(15)(16)(17)(18)(19)(20)(21). Childhood CP shares similar clinical, histological and immunofluorescent features with the adult form.…”

Section: Discussionmentioning

confidence: 80%

Childhood oral pemphigoid: a case report and review of the literature

Cheng¹,

Rees

Wright³

et al. 2001

J Oral Pathology Medicine

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Oral pemphigoid, defined as cicatricial pemphigoid limited to the oral cavity, is a clinical subset of cicatricial pemphigoid. Like cicatricial pemphigoid, it occurs mostly in middle-aged women and it is uncommon in children under 20 years of age. There are 11 cases of childhood cicatricial pemphigoid published previously in the English language literature, and four of them are oral pemphigoid patients. We present a case of the youngest oral pemphigoid patient reported yet. Because of the rareness of cicatricial pemphigoid in children, delayed diagnosis and treatment have been shown in many cases reported earlier. Clinical awareness and early recognition are, therefore, emphasized.

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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%

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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Cicatricial Pemphigoid: A Case of Onset at Age 5

Cited by 14 publications

References 27 publications

Number III Mucous membrane pemphigoid

Number III Mucous membrane pemphigoid

Childhood oral pemphigoid: a case report and review of the literature

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

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