Surgical Management of Ocular Cicatricial Pemphigoid in a Pediatric Patient

Abstract

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.

Childhood-Onset Ocular Mucous Membrane Pemphigoid Presenting with Peripheral Ulcerative Keratitis: A Case Report and Review of the Literature

“…The most frequently affected sites in pediatric patients included the oral cavity (n=18, 64%), followed by the conjunctiva (n=11, 39%), the genital mucosa (n=10, 35%), the skin (n=6, 21%), and the upper respiratory pathway (n=5, 62.5%) [7][8][9]. Notably, only ve patients presented isolated ocular disease [10][11][12][13]. Moreover, all patients with skin affection also presented ocular manifestations [6-9, 14-16].…”

“…Treatment in children is hindered by the extensive side effects of the drugs mentioned above and the lack of standardized regimens for MMP in this demographic. Prior cases of pediatric MMP included cyclosporine (4 mg/kg/day) in the treatment regimen; however, two out of three patients receiving this drug were unresponsive and required switching to another agent [12,13,15]. Interestingly, none of the prior cases were treated with methotrexate, an antimetabolite extensively studied in the pediatric population for other immune-mediated diseases, rendering an acceptable safety pro le [23,[25][26][27].…”

Ocular Cicatricial Pemphigoid in Young Patients

“…A few isolated case reports of early-onset OCP (onset before the age of 30 years) have been published (Table 2). [14][15][16][17][18][19][20][21][22][23][24] Of these, 4 belonged to the age group of 1 to 10 years, [14][15][16][17] 7 belonged to the age group of 11 to 20 years, and 3 belonged to the age group of 21 to 30 years. [18][19][20] Two cases of isolated OCP in young individuals (both cases aged 12 years) had been initially misdiagnosed as atopic keratoconjunctivitis and dry eye.…”