AESOP syndrome Sheinker, 1938

Last updated on: 05.11.2022

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AESOP is the acronym for "Adenopathy and Extensive Skin Patch Overlying a Plasmacytoma". AESOP syndrome is a very rare constellation of findings (there are only 11 case reports in the literature) in patients with an as yet undiagnosed solitary plasmacytoma.

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The skin lesions of AESOP syndrome are thought to arise from a process known ascontiguous cutaneous inflammation(CIS) (see also intralymphatic histiocytosis). In CIS, localized and irregularly configured erythema develops over another process, such as infection or neoplasm, localized in a deeper anatomic structure (Helmbold P et al 1999). In the case of AESOP syndrome, the plasmacytoma may produce angiogenic growth-promoting factors such as vascular endothelial growth factor(VEGF), which in turn would trigger vascular proliferation in the overlying dermis.

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18-73 years.

Clinical features
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A slow growing (growth: 5-10cm/year Rongioletti F et al. 2006) red, brown or red-brown , normal temperature, 8-10cm large, plaque with irregular, indurated possibly elevated edges as well as clearly visible blood vessels is conspicuous. The temperature of the skin site was either normal or warm.

The duration of the rash at the time of diagnosis ranged from three months to more than one year.

Furthermore, most patients showed polyneuropathies (facial and tongue paresthesias), dysesthesias and hypotonia of the limbs, areflexia, ascending paralysis of the upper and lower limbs, hypertrophy, and dyspnea.

In the majority of patients, unilateral or bilateral lymphadenopathy in the axillary, supraclavicular, cervical, and/or mediastinal areas is demonstrable after the cutaneous plaque appears (Crow RS 1956; Read D et al 1978). Palpable lymph nodes are mobile and firm with a size of 1 to 3 cm.

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Detectable on imaging studies are solitary osteolytic or osteodense masses in the bone below the skin lesion. In a few patients, the bone mass was palpable below the skin lesion. In most cases it was located on a rib or the sternum, in others on the skull, scapula, or clavicle.

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Evidence of a monoclonal spike on serum electrophoresis, Bence-Jones proteinuria, and hypercellularity and hyperproteinemia of the CSF.

Electromyographic studies confirmed the presence of multiple myeloma-myelinated motor and sensory neuropathy.

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Histo (bone): Histologic analysis of the suspected osteodense foci was consistent with plasmocytoma, as immunohistochemistry demonstrated either the monotypic kappa (κ)- or lambda (λ)-immunoglobulin light chains (Lipsker D et al.2003; Rongioletti F et al. 2006).

Lesional skin biopsies: dermal vascular hyperplasia with increased mucin in the surrounding area. In some cases, a perivascular inflammatory infiltrate of the skin was also seen, composed mainly of lymphocytes, neutrophils, and macrophages. In some cases, immunohistochemical staining was performed, which showed a deposition of λ-chains, mainly around the blood vessels of the skin. In one case, increased fibrosis was noted in the deep dermis and subcutis. The epidermis was mostly spared; only one case showed mild epidermal spongiosis and exocytosis (Lipsker D et al.2003).1

The histopathologic findings of dermal vascular hyperplasia without endothelatypia and increased dermal mucin are not specific; the term "mucinous angiomatosis". However, it is worth noting that other diseases have elevated dermal mucin associated with increased immunoglobulin deposition (i.e., scleromyxedema and persistent papular mucinosis) (Rongioletti F et al 2006).

Radiation therapy
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It is now well established that radiotherapy can be curative in patients with plasmacytomas. Most patients who received radiation or a combination of surgery and radiation had a favorable outcome.

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There is an association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes in about 1/3 of patients (Leite AC et al. 2007).

Malignant plasma cell proliferations, such as multiple myeloma, are commonly known to cause neuropathic symptoms. These symptoms usually occur a median of 1.5 years before tumor diagnosis (Sakemi H et al 1992). Neuropathy associated with plasma cell dyscrasias is usually a result of compression of a nerve or its vascular supply by the tumor mass.

Histopathologic examination of the harvested lymph nodes revealed either nonspecific findings (such as hyperplasia of native lymphocytes and sinusoidal lymphoplasmacytosis) or , histologic findings consistent with Castleman lymphoma, a disease known to be associated with POEMS syndrome(Castleman B et al 1956; Barrie JR et al 1981).

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  1. Barrie JR et al (1981) Castleman's disease of the lung: radiographic, high-resolution CT and pathologic findings. AJR Am J Roentgenol 166:1055-1056.
  2. Bataille R et al (1981) Solitary myeloma: clinical and prognostic features of a review of 114 cases. Cancer 48:845-851.
  3. Castleman B et al (1956) Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 9:822-830.
  4. Crow RS (1956) Peripheral neuritis in myelomatosis. Br Med J 2:802-804.
  5. Helmbold P et al (1999) Contiguous inflammation of the skin. Eur J Dermatol 9:48-50.
  6. Kuwabara S (2010) Crow-Fukase (POEMS) syndrome. Brain Nerve. 62:395-400.
  7. Leite AC et al (2007) POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin lesions) syndrome: a South America's report. Arq Neuropsiquiatr 65:516-520.
  8. Lipsker D et al.(2003) 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) 82:51-59.
  9. Read D et al (1978) Peripheral neuropathy and solitary plasmacytoma. J Neurol Neurosurg Psychiatry 41:177-184.
  10. Rongioletti F et al (2006) Cutaneous mucinous angiomatosis as a presenting sign of bone plasmacytoma: a new case of (A)ESOP syndrome. J Am Acad Dermatol 55:909-910.
  11. Sakemi H et al (1992) An autopsy case of Crow-Fukase syndrome which developed 18 years after the first manifestation of plasmacytoma. Intern Med 31:50-54.
  12. Sheinker I (1938) . Myeloma and the nervous system. Dtsch Z Nervenheik. 147:247-273.
  13. Tremblay V et al (2022) A case of relapsed systemic multiple myeloma mimicking adenopathy and extensive skin patch overlying a plasmacytoma. JAAD Case Reports 25: 97-99

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Last updated on: 05.11.2022