Adenopathy and extensive skin patch overlying a plasmacytoma (AESOP): Two morphologic variants can be outlined

Abstract

To the Editor: Adenopathy and extensive skin patch overlying a plasmacytoma (AESOP) syndrome associates a slowly enlarging erythematous skin patch overlying a solitary bone plasmacytoma (SBP) with locoregional adenopathy. It was initially reported in 1997 using the term “syndrome adéno-cutané associé au plasmocytome osseux.”1Schoenlaub P. Lipsker D. Massard G. Christmann D. Grosshans E. Syndrome adéno-cutané associé au plasmocytome osseux.Ann Dermatol Venereol. 1997; 124: 228-232PubMed Google Scholar In 2003, it was individualized and renamed AESOP.2Lipsker D. Rondeau M. Massard G. Grosshans E. The AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndrome: report of 4 cases of a new syndrome revealing POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome at a curable stage.Medicine (Baltimore). 2003; 82: 51-59Crossref PubMed Scopus (42) Google Scholar Along with the brief report of 2 new cases recently diagnosed in our institution (Figs 1 and 2), we provide a short review of the current literature about this rare but distinctive disorder to refine its clinical spectrum (21 cases in total, with data available at https://data.mendeley.com/datasets/ny945rfh3t/8).Fig 2Patient 2 diagnosed with adenopathy and extensive skin patch overlying a plasmacytoma (AESOP) syndrome. Note the brownish-red plicate and indurated plaque on the right flank, with irregular borders, overlying a plasmacytoma of the right rib, as a typical illustration of the second AESOP syndrome variant or morphea-like variant.View Large Image Figure ViewerDownload Hi-res image Download (PPT) AESOP syndrome mainly affects men (90%) with an average age of onset of 55 years. Skin involvement is distinctive in most cases, to the point of evoking in itself the diagnosis. From our own clinical experience, as well as from a careful review of pictures from the review of the literature, a continuum between 2 morphologic variants can be delineated: the classic variant presents as a smooth and shiny bright red patch, with sharp borders, and through which the underlying blood vessels can be seen; the second presents as a brownish-purple infiltrated plaque with less distinct edges, skin thickening, and a sclerotic appearance (Fig 1). The classical variant seems to occur preferentially on the thoracic region, while the morphea-like variant is generally observed in the hypochondrium regions. It can be finely scaling, warm, and itchy, and is rarely accompanied by paraesthesia. Though skin histology is nonspecific, there is an almost constant proliferation of dilated capillaries in the dermis (79%) associated with slight inflammatory infiltration of variable composition, and often with intradermal mucin deposition (37%). Firm, mobile, and homolateral enlarged lymph nodes are common (76%) but not essential to the diagnosis. In 67% of cases, an essentially sensorimotor severe polyneuropathy is associated—either demyelinating or mixed. Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is associated in 38% of cases. The SBP is most commonly of the lambda type (82% of the 11 evaluated cases) and mostly located on the ribs (57%). More than half of cases are accompanied by monoclonal gammopathy, most often immunoglobulin G lambda. Treatment has not been standardized, and many different strategies are used (chemotherapy, surgery, and radiotherapy). Disappearance of the skin lesion is the rule after effective SBP treatment. All the patients treated with radiotherapy had a complete response without any relapse or POEMS occurrence, while both reported deaths were related to the subsequent development of POEMS syndrome in patients not treated with irradiation. This study of 21 cases of AESOP syndrome further supports its autonomy and its frequent association with POEMS syndrome, for which it could be an early warning sign. In practice, despite its rarity, we believe that AESOP should be considered when encountering an enlarging bright red patch over the rib cage or the presternal area or a brownish-red plicate plaque located on the hypochondrium. The criterion standard treatment of underlying SBP is radiotherapy,3Tsang R.W. Campbell B.A. Goda J.S. et al.Radiation therapy for solitary plasmacytoma and multiple myeloma: guidelines from the International Lymphoma Radiation Oncology Group.Int J Radiat Oncol Biol Phys. 2018; 101: 794-808Abstract Full Text Full Text PDF PubMed Scopus (77) Google Scholar which we hypothesize may prevent progression toward life-threatening POEMS syndrome.