Background: We report a case of nivolumab-related sarcoidosis-like syndrome that initially presented with erythema nodosum. Sarcoidosis development has been described in single and combination immunotherapy.

Methods: A 68-year-old white woman with metastatic ocular amelanotic choroid melanoma was treated with nivolumab. The patient developed histologically confirmed erythema nodosum lesions and pulmonary granuloma sarcoidosis. Nivolumab was discontinued and the patient started ipilimumab therapy.

Conclusions: Sarcoidosis-like syndrome with lymphadenopathy is a rare adverse event that is important to recognize since it can be mistaken for metastatic disease progression. Tissue biopsy of new lesions during immunotherapy is an important step in patient evaluation.