POEMS AESOP Syndrome | Dermatopathology

Final Diagnosis: Consistent with an evolving Kappa light chain restricted plasma cell dyscrasia in a background of neovascularization, significant reactive neutrophilia and granulomatous lymphangitis as might be seen with POEMS syndrome/multicentric Castleman's disease with features reminiscent of AESOP syndrome.

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Slide 1: There is a very unusual vascular proliferation that is noted throughout the dermis. (H&E, 100X)

Slide 2: The vascular congeries define the epicenter of the supervening striking inflammatory process that is also prevalent throughout the sample. The vessels range from congeries of capillaries and venules to markedly dilated, thin-walled structures of possible lymphatic derivation. They focally assume a somewhat sinusoidal pattern of growth within the dermis. (H&E, 200X)

Slide 3: The vascular proliferations are associated with a mixed infiltrate of histiocytes, neutrophils, many plasma cells, and lymphocytes. (H&E, 400X)

Slide 4: Certain foci have a component of intravascular neovascularization imparting an almost glomeruloid hemangiomatous quality to them. (H&E, 400X)

Slide 5: Certain foci have a component of intravascular neovascularization imparting an almost glomeruloid hemangiomatous quality to them. (H&E, 400X)

Slide 6: Certain foci have a component of intravascular neovascularization imparting an almost glomeruloid hemangiomatous quality to them. (H&E, 400X)

Slide 7: In certain foci, there is occlusion of vascular structures by many histiocytes whereby the degree of intravascular histiocytic infiltration defines a component of an occlusive granulomatous lymphangitis. (H&E, 400X)

$Slide 8: Some vessels demonstrate intravascular deposits of proteinaceous material consistent with immunoglobulin including an abnormal protein, i.e. paraprotein. The proteinaceous deposits have a pink refractile quality. (H&E, 400X)$

Slide 9: PASD stains corresponding to the same vessels captured in images 8 and 10. The PASD stains highlight the abnormal proteinaceous precipitates within the vascular lumens. (PASD, 400X)

$Slide 10: Some vessels demonstrate intravascular deposits of proteinaceous material consistent with immunoglobulin including an abnormal protein, i.e. paraprotein. The proteinaceous deposits have a pink refractile quality. (H&E, 400X)$

Slide 11: PASD stains corresponding to the same vessels captured in images 8 and 10. The PASD stains highlight the abnormal proteinaceous precipitates within the vascular lumens. (PASD, 400X)

Slide 12: The kappa preparation highlights the plasma cells very extensively. (Kappa ISH stain, 200X)

Slide 13: In contradistinction, the degree of immunoreactivity for lambda is relatively minimal. (Lambda ISH stain, 200X).