Papulonodular Lesions in Photoexposed Areas as the First Manifestation of IgG4-Related Disease

Abstract

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