Cicatricial Pemphigoid

INTRODUCTION Cicatricial pemphigoid, also known as benign mucous membrane pemphigoid, essential conjunctival shrinkage, or ocular pemphigus is a progressive inflammatory disease of presumed autoimmune etiology. It variously involves mucous membranes of the mouth, conjunctiva, pharynx, nose, esophagus, vagina, urethra, and anus. Oral bullae and erosions occur in 90% of cases. Strictures of the esophagus, urethra, or anus sometimes occur late in the disease. Skin involvement is seen in less than 25% of cases and takes one of two forms: a recurrent nonscarring vesiculobullous eruption, mainly involving the extremities and inguinal region, or in the form of localized erythematous plaques with associated vesicles and bullae on the face and scalp. The latter variant heals with small atrophic scars. Another variant of localized pemphigoid known as Brunsting-Perry type has skin lesions limited to the face and neck but with no mucosal involvement. Significant scarring of affected areas often occurs, sometimes involving the eyelids. The average age of onset of cicatricial pemphigoid is in the seventh decade, but it can occur at any age, including childhood. The incidence is approximately 1:30,000 and there is a slight female predominance.

CLINICAL PRESENTATION In ocular cicatricial pemphigoid the conjunctival bullae are evanescent and therefore are seldom visualized. The initial symptoms usually include irritation and tearing due to ruptured conjunctival bullae. Subepithelial scarring with gradual obliteration of tear gland openings and loss of mucous glands ultimately results in keratoconjunctivitis sicca. In addition, surfaces on the bulbar and palpebral conunctiva fuse together, resulting in symblepharon. Eventually these symblephara obliterate the conjunctival fornices impairing lid closure. A band of symblepharon extending across the medial part of the lower cu-de-sac is typical of early ocular pemphigoid. The resulting entropion, tear deficiency, and lagophthalmos conspire to cause corneal opacification and loss of vision. Although a few unilateral cases have been seen, cicatricial pemphigoid almost always eventually becomes bilateral. However, involvement of the second eye may be delayed for up to two years.

Pemphigoid Eye
(Courtesy of Charles S. Soparkar, M.D.)

HISTOPATHOLOGY By routine light microscopy, cicatricial pemphigoid has subepidermal vesicles containing fibrin, edema fluid, and inflammatory cells. The dermis has a perivascular infiltrate of mainly lymphocytes and plasma cells, along with lesser numbers of neutrophils and eosinophils. Scarring may be prominent. Subepithelial bullae are not commonly seen in conjunctival lesions. Light microscopic features are nonspecific; diagnosis rests on demonstrating by direct immuno-fluorescence the linear deposition of immunoglobulin (usually IgG) and often of complement component C3 along the basement membrane zone.

Cicatricial Pemphigoid Conjunctiva

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes porphyria, bullous pemphigoid, sebaceous gland carcinoma, dermatitis herpetiformis, pemphigus vulgaris, Sjogren's disease, beta-hemolytic strep-tococcal infections, diphtheria, adenoviral and herpes simplex infections, trachoma, prolonged use of oral practolol, and reactions to epinephrine, pilocarpine, and/or phospholidine iodide eye drops.

TREATMENT Diagnosis can sometimes be established with a biopsy of an oral mucosal bulla, with histopatho-logic examination showing a sub-epidermal locus. Immunofluorescent antibodies fixed to conjunctiva basement membrane can be demonstrated in up to 80% of cases. Assays for circulating auto-antibodies also exist, but are positive in only about 10% of affected patients. During exacerbations, topical steroids will reduce the severity and perhaps diminish scarring. Because up to half of patients will harbor staphylococci, the lids and conjunctivae should be periodically cultured, and a course of appropriate antibiotics started if necessary. Artificial tears often help the signs and symptoms of aqueous tear deficiency. In severe cases combined therapy using systemic steroids and immunosuppressive agents is usually of benefit. Dapsone

(diaminodiphenylsulfone) in doses of 25-50 mg daily can show early benefit, but its effect appears to diminish after one to two years. Conjunctival surgery, including cryosurgery, should be avoided because it often precipitates exacerbations, ultimately leaving the patient worse off. Marked loss of conjunctiva can be repaired with mucous membrane or amniotic membrane grafts. Eyelid surgery for trichitic lashes should avoid insults to the conjunctiva if possible, and eyelid margin rotation through an anterior approach is preferred for entropion. No matter the intervention, some patients will inexorably progress despite all therapeutic measures.

REFERENCES

Bartley GB. Surgical reconstruction of the ocular surface in advanced ocular cicatricial pemphigoid and Stevens-Johnson syndrome. Am J Ophthalmol 1996; 122:752-753.

Bean S, Furey N, West C, Andrews T, Esterly NB. Ocular cicatricial pemphigoid (immunologic studies). Trans Sect Ophthalmol Am Acad Ophthal Otoloaryngol 1976; 81:806-812.

Dutton JJ, Tawfik HA, DeBacker CM, Lipham WJ. Anterior tarsal V-wedge resection for cicatricial entropion. Ophthal Plast Reconstr Surg 2000; 16:126-130.

Elder MJ, Collin R. Anterior lamellar repositioning and grey line split for upper lid entropion in ocular cicatricial pem-phigoid. Eye 1996; 10:439-442.

Elder MJ, Collin R. Lid surgery: the management of cicatricial entropion and trichasis. Dev Ophthalmol 1997; 28:207-218.

Elder MJ, Collin R. The eyelid sequelae of chronic progressive conjunctival cicatrisation. Dev Ophthalmol 1997; 28:176-181.

Griffith MR, Fukuyama K, Tuffanelli D, Silverman S Jr. Immunofluorescent studies in mucous membrane pemphigoid. Arch Dermatol 1974; 109:195-199.

Holsclaw DS. Ocular cicatricial pemphigoid. Int Ophthalmol Clin 1998; 38:89-106.

Mondino BJ, Brown SI, Lempert S, Jenkins MS. The acute manifestations of ocular cicatricial pemphigoid: Diagnosis and treatment. Ophthalmology 1979; 85:543-555.

Mondino BJ, Brown SI. Ocular cicatricial pemphigoid. Ophthalmology 1981; 88:95-100.

Mondino BJ, Brown SI. Immunosuppressive therapy in ocular cicatricial pemphigoid. Am J ophthalmol 1983; 96:453-459.

Mondino BJ, Ross AN, Rabin BS, Brown SI. Autoimmune phenomena in ocular cicatricial pemphigoid. Am J Ophthalmol 1977; 83:443-450.

Rogers RS, Perry HO, Bean SF, Jordan RE. Immunopathology of cicatricial pemphigoid: studies of complement deposition. J Invest Dermatol 1977; 58:39-43.

Shore JW, Foster CS, Westfall CT, Rubin PA. Results of buccal mucosal grafting for patients with medically controlled ocular cicatricial pemphigoid. Ophthalmology 1992; 383-385.

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