Dermatology Online Journal

Adenopathy and extensive skin patch overlying a plasmacytoma (AESOP) syndrome
Garrett Parker¹ MD, Anis Miladi² MD, Brian Thomas³ MD
Dermatology Online Journal 19 (2): 8

1. David Grant Medical Center, Travis Air Force Base, California
2. Navy Hospital Pensacola, Pensacola, Florida
3. Walter Reed National Military Medical Center, Bethesda, Maryland

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Abstract

In our manuscript we describe the cutaneous manifestations of a rare condition termed Adenopathy and Extensive Skin Patch Overlying Plasmacytoma (AESOP) syndrome. We emphasize the importance of clinically following and subsequently removing the osteolytic tumor to make the diagnosis.

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Case report

Figure 1	Figure 2
Figure 1. Violaceous, slowly growing, blanching plaque on the left flank. Figure 2. Skin biopsy with sparse perivascular infiltrate, rare eosinophils and mild telangiectasia (H&E x20) objective.

Figure 3	Figure 4
Figure 3. The lesion had doubled in size and developed a superimposed 3 x 4 cm palpable tumor within eighteen months after presentation. Figures 4 and 5. Blanching of the plaque following administration of 1% lidocaine with epinephrine.

Figure 5

A 57-year-old man presented with a six-month history of an expanding, violaceous plaque of the left flank. The patient denied pruritus, pain, neuropathy, or history of tick bite. Physical exam revealed a 10 x 20 cm violaceous, blanching plaque of the left flank (Figure 1) without associated lymphadenopathy. A punch biopsy was performed, revealing a sparse perivascular infiltrate with rare eosinophils and mild telangiectasia (Figure 2). The histological differential diagnosis for these non-specific findings included gyrate erythema, drug reaction, and viral exanthem. Complete blood count, comprehensive metabolic panel, and ultrasound study of the lesion were unremarkable. Because of the benign features seen on biopsy in association with absence of systemic symptoms, treatment was deferred and the patient was advised to follow up if the lesion changed or any associated symptoms developed.

Figure 6	Figure 7
Figure 6. Resolution of lesion after excision of the underlying plasmacytoma. Figure 7. Rib mass revealing plasma cells consistent with plasmacytoma.

Eighteen months later the patient presented again after the lesion had doubled in size and developed an underlying 3 x 4 cm palpable mass (Figure 3) without associated lymphadenopathy. At that time an incisional biopsy was obtained; following administration of lidocaine with epinephrine, the lesion blanched completely (Figures 4 and 5). This had also occurred with the initial punch biopsy. Incisional biopsy revealed a subtle capillary vascular proliferation with benign cytology throughout the dermis. Magnetic resonance imaging showed a mass within the left tenth rib as well as multiple enlarged intrathoracic regional lymph nodes. Following surgical resection of the rib mass, the violaceous skin lesion regressed completely (Figure 6). Analysis of the mass was consistent with plasmacytoma (Figure 7) with a λ light chain restriction. Further work-up to include serum and urine protein electrophoresis and bone marrow biopsy were unremarkable. Based on the above findings the patient was diagnosed with having had AESOP (Adenopathy and Extensive Skin patch Overlying a Plasmacytoma) syndrome.

Discussion

AESOP syndrome is a rare cutaneous manifestation of an osteolytic lesion with an unclear pathogenesis and only 11 cases described to date [1, 2]. Classically, the patient will present with a slowly enlarging erythematous to violaceous plaque or patch overlying a solitary plasmacytoma in association with local adenopathy [1]. These plaques can increase in size rapidly over relatively short periods of time [2]. As in the patient above, plasmacytomas seen in AESOP syndrome are located most commonly in the osseous structures of the thorax [1]. Further, the blanching of the lesion during biopsy and the vascular hyperplasia seen on histology are compatible with cytokine-induced cutaneous angiogenesis by the underlying plasmacytoma [1, 2, 3].

Of particular importance, the benign features of the lesion often cause significant delay in diagnosis, in many cases until the underlying mass is discovered. It is important for clinician to recognize this unusual presentation. Lipsker et al noted that the underlying plasmacytomas typically remain undiagnosed for over a year [2]. Although the number of AESOP syndrome cases reported in the literature is low, there is some suggestion that failure to treat the underlying plasmacytoma with either radiation or chemotherapy may be associated with poor outcome. The clinical differential diagnoses at presentation may include angiosarcoma, acquired progressive lymphangioma, aggressive systemic mastocytosis, lymphoma, metastatic disease of unknown etiology, aggressive vascular tumor, and erythema migrans. The absence of systemic symptoms in association with a primary cutaneous lesion that resembles an enlarging vascular plaque and the subtle histological findings as seen above should prompt clinicians to consider a diagnosis of AESOP syndrome and evaluate the patient for underlying plasmacytoma.

References

1. Rongioletti F, Romanelli P, Rebora A. Cutaneous mucinous angiomatosis as a presenting sign of bone plasmacytoma: a new case of (A)ESOP syndrome. J Am Acad Dermatol. 2006 Nov;55(5):909-10. [PubMed]

2. Lipsker 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 Jan;82(1):51-9. Review. [PubMed]

3. Rongioletti F, Patterson JW, Rebora A. The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies. J Cutan Pathol. 2008 Aug;35(8):705-21. Epub 2008 Mar 10. Review. [PubMed]

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