IgG4-related disease (IgG4-RD) or IgG4 syndrome is a chronic inflammatory condition of presumed autoimmune etiology, affecting mainly men in their sixth decade of life. It has a good prognosis following instituted treatment and is characterized by infiltration of IgG4 + plasma cells, predominantly in the pancreas, bile duct, lymph nodes, kidneys, retroperitoneum, lungs and salivary, parotid, submandibular and lacrimal glands. Skin manifestations of IgG4 syndrome are uncommon but should be known to the dermatologist. This report details a rare case of IgG4 syndrome involving cutaneous and lymph nodes. A 53-year-old woman described a 15-year history of erythematous and pruritic papulo-nodular lesions, mostly in photoexposed areas of the face, neck and upper limbs. She explained that the lesions evolved with ulceration, healing and residual dyschromia. Furthermore, she reported relapsing lymphadenomegaly 2 years ago, primarily in the cervical, axillary and inguinal chains.