Adenopathy and extensive skin patch overlying plasmacytoma syndrome—the clue to early diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes: A case series and literature review

Abstract

Importance: Adenopathy and extensive skin patch overlying plasmacytoma syndrome is a paraneoplastic syndrome characterized by a cutaneous vascular patch overlying a plasmacytoma and systemic manifestations. It is thought to be an early stage of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome, which is a rare, but potentially fatal multisystemic disease that is associated with plasma cell dyscrasia. Thus, a high index of suspicion is required to identify patients with adenopathy and extensive skin patch overlying plasmacytoma as they may present with early polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, which is curable if detected early. Objective: To report additional cases of adenopathy and extensive skin patch overlying plasmacytoma syndrome, describe dermatoscopic and histologic findings of the cutaneous patch and review all up to date literature on adenopathy and extensive skin patch overlying plasmacytoma syndrome. Design: Case series from a single tertiary care center. Participants: Here, we present the second case series of three patients with adenopathy and extensive skin patch overlying plasmacytoma syndrome who all meet the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. The diagnosis was suspected based on the presence of the violaceous cutaneous patch along with symptoms of systemic involvement (fatigue, weight loss, weakness). Dermoscopy revealing regular dilated parallel capillaries was suggestive of a benign/reactive vascular process. Histopathology in all three cases showed reactive vascular proliferation with a characteristic 90° branching. To date only 20 cases of adenopathy and extensive skin patch overlying plasmacytoma have been published, including ours. All patients presented with cutaneous lesions (violaceous patch and others) and most, at least 15/20, met the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. When clinical follow-up was reported, most patients had a favorable prognosis with partial or complete symptom resolution following treatment of the underlying plasmocytoma.

Concomitant AESOP and POEMS Syndrome in the Context of Osseous and Cutaneous Plasmacytomas: Answer

“…1 The occurrence of AESOP remains relatively rare, with less than 25 cases described in the literature. 2 AESOP is most commonly accompanied by an osseous plasmacytoma affecting the ribs or sternum. 3 This syndrome often presents with a violaceous or erythematous skin lesion accompanied by lymphadenopathy near the underlying plasmacytoma.…”

Adenopathy and an Extensive Skin Patch Overlying a Plasmacytoma Syndrome

“…Serologic examination is also crucial in evaluating hematologic involvement of the plasma cell neoplasm. It is essential to thoroughly investigate findings indicative of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome, including abnormal elevation of monoclonal proteins, multiple sclerotic bone lesions, and peripheral neuropathy, as more than 70% of patients with reported AESOP syndrome meet the diagnostic criteria of POEMS syndrome …”