Idiopathic Ocular Cicatricial Pemphigoid (IOCP)

 

IOCP belongs to the mucuous membrane pemphigoid group of autoimmune subepithelial blistering diseases.

 

Clinical features:

(1) age usually > 60 years

(2) conjunctivitis with scarring

(3) The patient may have bullous lesions on the face, scalp and upper trunk.

(4) The patient may have bullous lesions of the oral mucous membranes.

 

Laboratory and histologic findings:

(1) antibodies to the beta-4 integrin subunit in the serum

(2) tissue immunofluorescence shows linear subepithelial deposits of IgG and C3 along the basement membrane

 

Differential diagnosis:

(1) anti-epiligrin cicatricial pemphigoid (AECP, paraneoplastic OCP)