Ocular Mucous Membrane Pemphigoid In Young Age – Do We Need To Lower Our Diagnostic Threshold?

To study the clinical profile and outcomes of severe acute presentation of OCP in young age group in a tertiary care centre.

Tertairy care multispecialty medical education and research institution

We analyzed medical records of the patients presented with acute severe conjunctival inflammation suggestive of OCP in a single tertiary care centre in last one year (August 2021- July 2022). Demographic profile, visual acuity, clinical features, foster staging, earlier diagnosis, cicatrizing score, immuno-histochemistry results, surgical intervention done, type of immunosuppressive therapy used, time to remission and relapse were noted.Medical records of 6 patients (<30years of age), who presented with severe conjunctival inflammation and are in advance stage of the disease. 

The average age was 21.8+/- 4.8 years. Average BCVA (in decimal) at presentation was in 0.34+/- 0.31 in the right and 0.49+/-0.38 left eye respectively. The average duration of illness at the time of presentation was 19.8+/-14.88 Months. Average cicatrizing conjunctivitis score was 0.16 +/- 0.55. 8/12 (67%) eyes were noted to be in Foster stage 3 or 4. Five (83.34%) patients reported positive for immuno-histochemistry (linear deposits of IgG, IgA or C3 at BMZ) on either oral mucosal or conjunctival biopsy. Four (66%) patients needed intravenous immunosuppressive therapy (IV methyl Prednisolone, Cyclophosphamide or Rituximab) to control the initial inflammation. 

This case series highlights the importance of having greater sense of clinical suspicion towards ocular presentation of MMP, which is generally considered as rare and a disease of old age white population. These patients in our scenario are generally diagnosed as simple dry eyes. Cicatrizing conjunctivitis scoring system can be a useful adjunctive tool to make working diagnosis and guide therapy in such patients.