Ocular Onset of Paraneoplastic Pemphigus Presenting as Hyperemic Conjunctivitis and Massive Bilateral Eyelid Ulceration: A Case Report and Literature Review

Abstract

Complete slit lamp ocular study, conjunctival swabs, routine hematologic tests, serum neoplasm markers, indirect immunofluorescence study, immunoblotting, and oral mucose biopsy with direct immunofluorescence were performed under the hypothesis of a paraneoplastic sign. Total body computed tomography scan and ultrasound-guided needle prostate biopsy completed the diagnostic process and confirmed the diagnosis of prostate PNP. Complete remission of ocular clinical signs was achieved by treatment of the prostate malignancy with systemic immunosuppressive therapy and chemotherapy.

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

“…Ocular lesions may be present at the disease onset. 61 Early ophthalmological assessment is therefore recommended. 62 In one report, histopathologic and direct IF microscopy findings in ocular PNP/PAMS were similar to those found in PV.…”

“…Burning and pain, mucous discharge and decreased visual acuity are the most frequent ocular symptoms. Ocular lesions may be present at the disease onset 61 . Early ophthalmological assessment is therefore recommended 62 .…”

Ocular manifestations of dermal paraneoplastic syndromes

“…Serum auto-antibodies remained detectable for 2–4 years after surgery without recurrence of the tumour, suggesting that the immune reaction persisted [ 13 ]. Hyperaemic conjunctivitis in both eyes with large ulcerations on superior eyelids and marginal erosions on the inferior eyelids were also reported to be associated with paraneoplastic pemphigus [ 14 ]. In this case, conjunctival inflammation can be reduced by topical corticosteroids [ 14 ].…”

“…Hyperaemic conjunctivitis in both eyes with large ulcerations on superior eyelids and marginal erosions on the inferior eyelids were also reported to be associated with paraneoplastic pemphigus [ 14 ]. In this case, conjunctival inflammation can be reduced by topical corticosteroids [ 14 ]. For eyelid ulceration, topical cyclosporine with dexamethasone and ofloxacin as an antibiotic has been described [ 14 ].…”

“…In this case, conjunctival inflammation can be reduced by topical corticosteroids [ 14 ]. For eyelid ulceration, topical cyclosporine with dexamethasone and ofloxacin as an antibiotic has been described [ 14 ]. In another case, the authors presumed that paraneoplastic pemphigus caused ocular surface disease with small epithelial defects of the cornea, which eventually led to corneal melting and perforation [ 10 ].…”