Pseudopemphigoid as caused by topical drugs and pemphigus disease

Abstract

Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmune links. For the purpose of this review, we will describe pseudopemphigoid as caused by topical drugs, the most common etiology with ocular manifestations, and as caused by the pemphigus disease, a more rare etiology. Specifically, we will discuss the ophthalmological features of drug-induced cicatricial conjunctivitis, pemphigus vulgaris, and paraneoplastic pemphigus. Other etiologies of pseudopemphigoid exist that will not be described in this review including autoimmune or inflammatory conditions such as lichen planus, sarcoidosis, granulomatosis with polyangiitis (Wegener’s granulomatosis), erythema multiforme (minor, major, and Stevens-Johnson syndrome), bullous pemphigoid, skin-dominated linear IgA bullous dermatosis, and skin-dominated epidermolysis bullosa acquisita. Prompt diagnosis of the underlying etiology in pseudopemphigoid is paramount to the patient’s outcome as certain diseases are associated with a more severe clinical course, increased ocular involvement, and differential response to treatment. A complete history and ocular examination may find early cicatricial changes in the conjunctiva that are important to note and evaluate to avoid progression to more severe disease manifestations. When such cicatricial changes are noted, proper diagnostic techniques are needed to help elucidate a diagnosis. Lastly, collaboration between ophthalmologists and subspecialists such as dermatologists, pathologists, immunologists, and others involved in the care of the patient is needed to ensure optimal management of disease.

Keywords: Pseudopemphigoid; Mucous membrane pemphigoid; Cicatricial conjunctivitis; Pemphigus vulgaris; Paraneoplastic pemphigus; Drug-induced conjunctival cicatrization

Core tip: Pseudopemphigoid in the context of chronic cicatricial conjunctivitis mimicking mucous membrane pemphigoid is a disease with terminology that has continuously evolved since its inception. Recent understanding of the ophthalmological and systemic manifestations of pseudopemphigoid as caused by topical drugs and the pemphigus disease demonstrates that significantly decreased vision and/or increased mortality due to paraneoplastic associations may result. Proper diagnosis and treatment of the underlying disease is therefore critical in order to provide maximal care to the patient.