Ocular Manifestations in a Case of Childhood Cicatricial Pemphigoid

Iovine, Antonello; Fimiani, Francesco; Vassallo, Patrizia; Alessio, Maria; Magli, Adriano

doi:10.1177/112067210801800424

Cited by 9 publications

(9 citation statements)

References 7 publications

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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%

“…Ophthalmologic surveillance is needed even in the absence of initial ocular lesions. An involvement of the anal mucosa has also been described, always in association with other mucous membrane involvement (1,5,10,16). Our patient also had crusted erosions, with bleeding and synechia of the nasal cavities.…”

Section: Discussionmentioning

confidence: 92%

“…Trichiasis is considered as a predicting factor of the severity of the condition. Including our case, ophthalmologic involvement in CP has been documented in 6 of the 18 reported cases (1,5,10,15,16). Onset of ocular lesions may be delayed and may appear several years after disease onset.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 92%

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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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“…2,3 Two of them presented with singular ocular involvement, whereas oral cavity and genitalia are the most frequently involved sites in children. 2,4 As maintained by Iovine et al, 1 early treatment may be relevant in pediatric cases because the clinical course tends to be more aggressive and resistant to medical treatment compared with adult cases. Thus, an aggressive therapy may not always be enough to stop progression of the disease.…”

mentioning

confidence: 97%

“…P ediatric mucous membrane pemphigoid (MMP) is extremely rare in children. 1 Only 20 cases have been reported in children between 2 months and 18 years, mainly female patients. 2,3 Two of them presented with singular ocular involvement, whereas oral cavity and genitalia are the most frequently involved sites in children.…”

mentioning

confidence: 99%

Surgical Management of Ocular Cicatricial Pemphigoid in a Pediatric Patient

Ollero

Soldevila

Paz

et al. 2021

Cornea

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Purpose:The purpose of the report was to describe the first successful tarsal fracture surgery in a 1-year-old boy diagnosed with cicatricial ocular pemphigoid whose visual and psychomotor development were notably limited.Methods:We present the case of a 1-year-old boy diagnosed with mucous membrane pemphigoid by biopsy who was treated with rituximab (375 mg/m2 intravenous infusion at 2-week interval administered twice) and stable with oral dapsone (2 mg·kg−1·d−1). His eyelid cicatricial entropion and trichiasis in both eyes prevented him from opening his eyes, impeding visual development. After 1 year of clinical stability, we performed a tarsal fracture procedure in both eyes to restore eyelid anatomy and functionality, with the aim to prevent an inflammatory reaction, administrating intravenous dexamethasone before and after surgery.Results:The intervention was successfully performed without postoperative complications. Excellent anatomic and functional results allowed him to develop normally in his daily life the first week after surgery. He is currently taking oral dapsone (2 mg·kg−1·d−1) as a maintenance treatment to stop the progression of the disease.Conclusions:Tarsal fracture surgery may be considered part of the treatment in pediatric patients with stable ocular cicatricial pemphigoid presenting with severe entropion and trichiasis.

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