Isolated Skin Involvement as Manifestation of Immunoglobulin G4-related Disease (IgG4-RD) Relapse

Immunoglobulin G4-related disease (IgG4-RD) is a very rare and little known sclerosing inflammatory disease of recent individualization.

Cutaneous manifestations during IgG4-RD are very rare. They are described as “infrequent” and “uncommon” by the majority of authors, and are often reported as sporadic clinical cases.

We report the original observation of isolated and polymorphic skin involvement of the head and neck as manifestation of IgG4-related disease relapse in 68-year-old man.

Our observation is distinguished by the polymorphism of skin lesions, the isolated nature of skin involvement (without associated involvement of other organs), and the association of lesions with different ethiopathogenic mechanisms.