Adenopathy and extensive skin patch overlying a plasmacytoma with unusual histologic findings in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes syndrome and Castleman disease

Abstract

A 56-year-old previously healthy man presented to the dermatology clinic with a 2-year history of an expanding, violaceous, infiltrated plaque on the right flank. Biopsy revealed a diffuse dermal vascular proliferation of bland, capillary-sized vessels admixed with conspicuous fibrohistiocytic cells including scattered multinucleated floret cells. Further workup revealed a monoclonal gammopathy, an osteolytic chest wall plasmacytoma underlying the plaque, and regional lymphadenopathy leading to a diagnosis of adenopathy and extensive skin patch overlying a plasmacytoma (AESOP).Biopsy of an enlarged lymph node revealed Castleman disease. The patient subsequently developed polyneuropathy and peripheral edema, which supported an additional diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome. Herein, we discuss the unique findings of our patient, the potential pathogenesis of AESOP, and the link between these three rare paraneoplastic entities along with review of the literature. K E Y W O R D S adenopathy and extensive skin patch overlying a plasmacytoma, AESOP, Castleman disease, POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes

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

“…3 Furthermore, only select cases observed endocrinopathy (5/20) and organomegaly (4/20). 3,5,12,14 Lymphadenopathy and skin changes other than the violaceous patch were present in 18/20 and 20/20 patients, respectively. Unfortunately, most previous studies lack significant details regarding the management and prognosis.…”

“…To date, 20 cases of AESOP syndrome have been published, including ours (Table 1). 3–6,8–17 In all cases, patients presented with an extensive ill-defined violaceous patch on the trunk, in 19 an underlying plasmacytoma was identified, and in 1 case it was a blue-cell sarcoma. 10,15 The most common location of the plasmacytoma was ribs (10/19), followed by sternum (5/19), scapula (1/19), clavicle (1/19), skull (1/19), and cervical spine/ribs (1/19).…”

“…7 To date, 20 cases of AESOP syndrome have been published, including ours (Table 1). [3][4][5][6][8][9][10][11][12][13][14][15][16][17] In all cases, patients presented with an extensive ill-defined violaceous patch on the trunk, in 19 an underlying plasmacytoma was identified, and in 1 case it was a blue-cell sarcoma. ).…”

“…endocrinopathy) and cure rates are higher. [3][4][5] Therefore, timely recognition of AESOP is important. To date, only 17 cases of AESOP have been published as either a single patient case report or small case series.…”

A case of relapsed systemic multiple myeloma mimicking adenopathy and extensive skin patch overlying a plasmacytoma

“… 5 , 6 The characteristic histopathologic findings of AESOP syndrome are diffuse capillary dilations that can be accompanied by dermal deposition of mucin and a mild inflammatory infiltrate of variable composition. 7 , 8 CD31 immunohistochemical staining can also be positive. 9 The exact cause of AESOP syndrome is unknown, but it is postulated that overproduction of vascular endothelial growth factor by the plasmacytoma could be responsible for the clinical manifestations of this paraneoplastic syndrome.…”