Nodular Primary Localized Cutaneous Amyloidosis
A 43-year-old man presented for a routine skin check with a several year history of unilateral, yellowish, pearly papules on the left lower eyelid. The lesions were completely asymptomatic and did not affect his vision.
The patient reported no allergies, wasn't taking any medications, and had an otherwise unremarkable medical history—only describing a shoulder injury as a teenager. Furthermore, he reported no family history of similar skin findings or autoimmune disease. A punch biopsy was obtained for histological examination.
Ultimately, the patient was diagnosed with nodular primarylocalized cutaneous amyloidosis (NPLCA).
Amyloidosis describes a broad range of diseases in which amyloid proteins are deposited extracellularly and lead to changes in tissue architecture and function.1-3 Clinical classification of amyloidosis depends on the type of fibrils present as well as the fibrils’ deposition pattern.1-2 Amyloidosis may present as localized or systemic forms and may range from a completely benign course to one of high morbidity and mortality. Localized amyloidosis is, most commonly, secondary to various skin growths. Primary cutaneous amyloidosis refers to disease only involving the skin with no evidence of systemic involvement.4 There are three main types of primary localized cutaneous amyloidosis: macular, lichen (papular) and nodular (tumefactive). Of the three, the nodular type is the rarest form.5
Patients with NPLCA present with one or more waxy yellow papules and nodules ranging in size from millimeters to centimeters. It is diagnosed by characteristic histological findings on biopsy of the lesion. Under light microscopy, amyloid material will appear acellular, amorphous, and eosinophilic.1,3,4 Hyaline deposition is located from the papillary dermis to the subcutis, affecting blood vessels and adnexae.6
Patients suspected to have NPCLA must be worked up and ruled out for systemic amyloidosis. First, patients should undergo a thorough physical exam. Additionally, an extensive lab work should be performed.
There is no one specific treatment for NLPCA, as the underlying cause is unknown. Lesions may be considered for cosmetic therapies when they are small, in a highly visible area (eg, on the face) and are amenable to treatment. Corticosteroids (topical and/or intra-lesional) have not been found to be effective in NLPCA. Treatment is not curative and lesions recur frequently.
Biopsy demonstrated findings consistent with nodular amyloidosis on both hematoxylin and eosin and congo red stains in this patient. No immunohistochemical staining was available. Extensive testing for systemic disease was negative. The patient is periodically being monitored for any potential transformation to systemic amyloidosis and is not receiving any treatment. ■
This case was previously published in August 2012 issue of The Dermatologist.
References:
1.Breathnach SM. Amyloid and amyloidosis. J Am Acad Dermatol. 1988;18(1):1-16.
2.Touart DM, Sau P. Cutaneous deposition diseases: part I. J Am Acad Dermatol. 1998;39(2):149-171.
3.Kalajian AH, Waldman M, Knable AL. Nodular primary localized cutaneous amyloidosis after trauma: A case report and discussion of the rate of progression to systemic amyloidosis. J Am Acad Dermatol. 2007;57(2Suppl):S26-29.
4.Moon AO, Calamia KT, Walsh JS. Nodular amyloidosis: review and long-term follow up of 16 cases. Arch Dermatol. 2003;139:1157-1159.
5.Woollons A, Black MM. Nodular localized primary cutaneous amyloidosis: a long-term follow-up study. Br J Dermatol. 2001;145:105-109.
6.Huilgol SC, Ramnarain N, Carrington P, Leigh IM, and Black MM. Cytokeratins in primary cutaneous amyloidosis. Australas J Dermatol. 1998;39:81-85.