2.
3.
Diagnosis: Nodular localized primary cutaneous amyloidosis (NLPCA)
Histology: Histology reveals extracellular amorphous eosinophilic masses in the papillary dermis and thinning and obliteration of the rete ridges. There are diffuse deposits in the reticular dermis and subcutis with infiltration into blood vessel walls and pilosebaceous units. There is a marked infiltration of plasma cells (Figs.2,3).
Discussion: Amyloid is a pathologic proteinaceous substance, deposited between cells in a variety of tissues and organs as a result of a many different disease processes. Systemic amyloidosis is a progressive condition, and can be primary, related to plasma cell dyscrasias or secondary, related to chronic disease such as infections, malignancies, and rheumatologic disorders.
Cutaneous amyloidosis can occur secondary to systemic amyloidosis or as a primary localized form. There are three types of primary cutaneous amyloidosis, macular, papular (lichen amyloidosis), and nodular (tumefactive).
Nodular localized primary cutaneous amyloidosis is the rarest of the cutaneous amyloidoses and can often be clinically indistinguishable from systemic amyloidosis. It presents as single or multiple waxy or anetodermic nodules on the limbs, face, trunk, or genitalia. There is usually little or no itching that accompanies the lesions.
NLPCA is similar to an extramedullary plasmacytoma since the amyloid deposits are made up of immunoglobulin light chains derived from a local clonal expansion of plasma cells. There is no accompanying clonal expansion in the bone marrow. Patients do need close follow-up because over time patients may develop systemic amyloidosis. The most recent series followed 15 patients with NLPCA for an average of 12 years (range 2-23 years) and reports only 1 patient (7%) developing systemic amyloidosis. Other series have found the incidence of progression of NLPCA to be between 15-50%.
Treatment: Many different modalities have been used to treat nodular cutaneous amyloidosis including surgical excision, electrodessication and curettage, cryotherapy, intralesional corticosteroid injection, CO2 laser, and pulsed dye laser. Lesions vary in their response to different therapies and cosmetic outcomes. NLPCA also has a high recurrence rate after treatment.
References:
Breathnach, SM. Amyloidosis of the Skin. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, Fitzpatrick TB, eds. Fitzpatrick's Dermatology in General Medicine. New York: McGraw-Hill, 1999; 1756-1765.
Steciuk A, Dompmartin A, Troussard X, Verneuil, L, Macro M, Comoz F, Leroy, D. Cutaneous amyloidosis and possible association with systemic amyloidosis. Int J Dermatol 2002; 41: 127-132.
Woollons A, Black MM. Nodular localized primary cutaneous amyloidosis: a long-term follow-up study. Br J Dermatol 2001; 145: 105-109.
Hagari Y, Mihara M, Konohana I, Ueki H, Yamamoto O, Koizumi H. Nodular localized cutaneous amyloidosis: further demonstration of monoclonality of infiltrating plasma cells in four additional Japanese patients. Br J Dermatol 1998; 138: 652-654.
Alster TS, Manaloto RP. Nodular Amyloidosis Treated with a Pulsed Dye Laser. Dermatol Surg 1999; 25: 133-135.
This case was prepared with the assistance of Keri Clifford, Dr. Pamela Weinfeld, Dr. D. Santa Cruz.
