Ocular Cicatricial Pemphigoid

Alternative Names

  • OCP
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WHO-ICD-10 version:2010

Diseases of the skin and subcutaneous tissue

Bullous disorders

OMIM Number

164185

Mode of Inheritance

Autosomal dominant

Description

Ocular cicatrical pemphigoid (OCP) is an autoimmune blistering disease which affects the conjunctiva, the skin, and multiple mucous membranes (oral mucosa, pharynx, larynx, trachea, esophagus, vagina, urethra, and anus). Nearly 75 to 100% of OCP patients suffer from oral lesions while 25% of patients experience skin involvement like face, neck, and scalp.

OCP occurs in all races with female predominance and a female:male ratio of 1.5:3.1. It is an extremely rare condition in children, since only a limited number of cases have been reported in the literature. The age of onset of OCP ranges between 50 and 60 years; however, the precise age of onset might be at a younger age due to the inability of diagnosing the disease in early stages.

To date, no effective topical agent has been found that prevents OCP activity, however, subconjunctival steroid injections or subconjunctival injections of mitomycin C might help in decelerating the disease progression, whereas systemic therapy demonstrates an outcome. Patients experiencing dry eye symptoms should undergo adjuvant treatment using topical lubricants, whereas the inflammation can be controlled through topical cyclosporine and tacrolimus ointment. Several ocular surgical procedures are available including epilation, punctual occlusion, lid surgery, fornix reconstruction, corneal surgery, and cataract surgery; however these surgeries are performed following a complete control of the inflammation.

The reason for ocular cicatrical pemphigoid (OCP) occurrence is anonymous; however, genetic factors and numerous triggers are capable of increasing the likelihood of the onset of OCP. Persons with HLA-DR2, HLA-DR4 (HLA-DR*0401), and HLA-DQw7 (DQB1*0301) are more susceptible to OCP, meanwhile systemic practolol therapy and topical antiglaucoma drugs (such as phospholine iodide, idoxuridine, epinephrine, pilocarpine, humorsol, and timolol) were found to act as triggers of OCP.

Epidemiology in the Arab World

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Other Reports

Egypt

[See: Kuwait > Nanda et al., 2004].

Kuwait

Nanda et al. (2004) carried out a study Al-Sabah Hospital for 11 and a half years throughout the period between July 1991 and December 2002 to assess the clinico-epidemiological characteristics of different autoimmune bullous diseases (ABDs). The study consisted of 128 subjects with ABDs including 42 males, 86 females with a male: female ratio of 1:2.05, 110 Arabs (86%), and a mean age of 42 +/- 19.57 years. The Arab cohort contained 60 Kuwaitis, 16 Bedouins, 14 Egyptians, Seven Syrians, four Sudanese, four Palestinians, two Lebanese, one Sudanese, one Emirati, and one Somali. The most common ABD was found to be pemphigus constituting 47% (60 cases with a mean of age of 36.50 +/-11.36 years), proceeded by 22% pemphigoid (28 subjects), 19% pemphigoid gestationis (PG, 24 female patients), 7% linear IgA bullous disease (LABD, nine individuals including eight children aged below 13 years), 3% lichen planus pemphigoides (LPP, four out-patients), and 2.3% epidermolysis bullosa acquisita (EBA, three sufferers). The least estimated incidence of ABDs within a reference population of 1.14x10(6) million was anticipated at 9.8 per million per year. The pemphigus group was comprised of 80% pemphigus vulgaris (PV, 48 subjects), 18% pemphigus foliaceus (PF, 11 patients), and 2% IgA pemphigus (a 35 year old Lebanese female). Meanwhile, all patients in the pemphigoid group were of Arab ethnicity with cicatricial pemphigoid (CP) presenting one case of a 50 year old Egyptian male and 27 patients suffering from bullous pemphigoid (BP, mean age of 65.97 +/- 16.69 years). Female predominance was observed within pemphigoid cases, PG, LPP, and EBA, while male predominance was noted throughout LABD cases. Bullous lupus erythematosus was not found in any subject throughout this study whereas BP was found to be the second most common ABD. The incidence of pemphigus was estimated at 4.6 cases per million per year in Kuwait while in Saudi Arabia the incidence was estimated at 1.6 cases/million population. Nanda et al. (2004) proposed the need for surveys and studies concerning ABDs to boost the existing knowledge about these disorders.

Lebanon

[See: Kuwait > Nanda et al., 2004].

Palestine

[See: Kuwait > Nanda et al., 2004].

Saudi Arabia

[See: Kuwait > Nanda et al., 2004].

Somalia

[See: Kuwait > Nanda et al., 2004].

Sudan

[See: Kuwait > Nanda et al., 2004].

Syria

[See: Kuwait > Nanda et al., 2004].

Tunisia

Kharfi et al. (2010) described the case of a 20-month-old boy with cicatrical pemphigoid; probably the youngest patient reported yet worldwide. The onset of the disease occurred 10 months earlier with mucosal crusted erosions of the oral and nasal cavities and conjunctiva. 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. Yet, cicatrization, synechia of the nasal cavities and corneal opacities lead to a dramatic visual loss.

United Arab Emirates

[See: Kuwait > Nanda et al., 2004].

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