Paraneoplastic Ocular Cicatricial Pemphigoid (POCP, Anti-Epiligrin Cicatricial Pemphigoid, AECP)

 

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

 

Clinical features:

(1) age >= 30 years

(2) presence of a carcinoma, which may be occult

(3) conjunctivitis with scarring

(4) The patient may have lesions of the oral mucosa.

(5) The patient may have mucosal lesions along the respiratory tract with cough.

(6) The patient may have skin lesions over the trunk and upper extremities.

 

Laboratory and histologic findings:

(1) antibodies to laminin 5 (anti-epiligrin) in the serum

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

 

Differential diagnosis:

(1) idiopathic OCP (OCP not associated with an underlying neoplasm)