AESOP syndrome: a potential life-saving and early clue to the diagnosis of POEMS syndrome

Adenopathy and an extensive skin patch overlying a plasmacytoma (AESOP) syndrome, first described 16 years ago, is characterized by a slowly expanding red or brown patch (classic variant) or plaque (morphea-like variant) overlying a solitary plasmacytoma of the bone. Early recognition can be life-saving, as it is an early presentation of polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome, which may be fatal. There are two forms: the classic variant, which presents as a smooth, shiny macule with abnormal visibility of skin vessels, and the morphea-like variant, which presents as a plaque where the skin is folded, giving a peau d'orange appearance. The locations are frequently above the rib cage, above the sternum, or the scalp. Seventy-five percent of the patients develop regional lymphadenopathy within a few months. Histopathologic study reveals a capillary proliferation in the dermis. The diagnosis relies on subsequent identification of the plasmacytoma. At the time of a presumptive diagnosis, only 40% of patients have fulfilled the diagnostic criteria of POEMS. Treatment depends on the final hematologic diagnosis, but radiation of the plasmacytoma is required. (C) 2020 Elsevier Inc. All rights reserved.

Automated summary

Adenopathy and an extensive skin patch overlying a plasmacytoma (AESOP) syndrome is a condition characterized by a slowly expanding red or brown patch overlying a solitary plasmacytoma of the bone. Early recognition is crucial as it may progress to the more serious POEMS syndrome. The syndrome has two forms, classic and morphea-like variants, with treatment depending on the final hematologic diagnosis and requiring radiation of the plasmacytoma.