IgG4-Related Skin Disease Presenting as a Pseudolymphoma in a White Adolescent Girl

Abstract

Immunoglobulin gamma (Ig) type 4–related disease (IgG4-RD) is a chronic immunologic systemic disorder that has gained worldwide recognition in the past decade. This entity can affect almost every organ system, and its characteristic lesions have been found in a variety of organs such as lacrimal and salivary glands, pancreas, liver, bile ducts, lungs, kidneys, retroperitoneum, breast, aorta, thyroid, and prostate. This case reports a very rare case of IgG4-RD presenting with a cutaneous lesion in a young female patient mimicking a lymphoproliferative disorder. IgG4-RD affecting the skin is a rare entity and has mostly been reported in Japanese men of middle to older age. IgG4-RD with cutaneous involvement should be in the differential of non-neoplastic, lymphomas, autoimmune, and infectious disorders of the skin.

Cutaneous reactive B‐cell lymphoid proliferations

“…Multiple organs are commonly affected in IgG4‐related disease, including the lungs, lymph nodes, pancreas, eyes, and skin. Patients can present with pseudolymphomatous cutaneous nodules of the head and neck, or pruritic papules and plaques on the trunk and extremities in the context of systemic signs of disease 11,147–149 . Histopathologic characteristics of these lesions included prominent intradermal or subcutaneous germinal centers with sclerotic stroma, admixed eosinophils, lymphocytes, and abundant plasma cells (Figure 18).…”