Necrobiotic xanthogranuloma associated with necrotizing scleritis

Area of Science:

• Ophthalmology
• Dermatology
• Pathology

Background:

• A 57-year-old man with a decade-long history of asymptomatic annular skin plaques presented with acute red eye.
• Necrotizing scleritis, a serious inflammatory eye condition, can lead to vision loss if untreated.

Observation:

• Ophthalmological examination revealed necrotizing scleritis in the right eye, progressing to scleral melting and requiring a patch graft.
• Skin examination showed annular plaques with central atrophy and induration.
• The left eye also developed necrotizing scleritis with scleral melting.

Findings:

• Skin biopsies initially suggested necrobiosis lipoidica diabeticorum but later indicated necrobiotic xanthogranuloma due to extensive necrobiotic changes.
• Histopathology revealed palisading granulomatous inflammation, plasma cells, and multinucleated giant cells.
• Serum protein electrophoresis identified an IgG lambda monoclonal protein.

Implications:

• This case underscores the association between necrobiotic xanthogranuloma and severe ocular inflammation, specifically necrotizing scleritis.
• Early recognition and systemic workup are crucial for managing patients with unexplained scleritis and suggestive skin findings.
• The presence of a monoclonal protein warrants further investigation for underlying plasma cell dyscrasias.