The main objective of this article is to present and discuss a case of localized ulcerative nodular amyloidosis with deep involvement clinically manifesting as ulcerative panniculitis and discuss its impact on the choice of treatment. We present a year-old woman with a history of painful ulcerated nodules on the inferior limbs. Microscopy confirmed amyloid deposits deep in the dermis and subcutaneous fat. No systemic involvement was found. Considering that skin-directed treatments often are not able to reach subcutaneous fat or were contraindicated because of the ulcers, she was successfully treated with cyclophosphamide and prednisone. Localized ulcerative nodular amyloidosis with deep involvement is a rare clinical presentation that can present as ulcerative panniculitis.
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Washington, DC. This work was done during Dr. In lichen amyloidosus, characteristic manifestations were pruritic papules on the lower extremities and deposits of amyloid limited to the papillary corium. Crystal violet was the most reliable preparation for amyloid.
In autopsy material, amyloid was not found in extracutaneous sites. No patient with systemic amyloidosis had the clinicopathologic findings of lichen amyloidosus. Thus, when lichen amyloidosus is recognized, investigation for systemic disease is not indicated. In macular amyloidosis, the lower extremities were the common site of involvement, pruritus was frequent, and deposits of amyloid were limited to the dermal papillae.
In the tumefactive form of primary localized cutaneous amyloidosis lesions were found on the extremities, trunk, genitals, or face. The histologic picture resembled that found in cutaneous lesions of primary systemic amyloidosis. The electron microscopic appearance of the amyloid deposits was similar to that found in other forms of cutaneous amyloidosis. Clinically insignificant microdeposits of secondary localized cutaneous amyloidosis were observed in a variety of disorders, including seborrheic keratoses and Bowen's disease.
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