ABSTRACT
Primary cutaneous amyloidosis includes several forms of localized amyloidosis characterized by superficial amyloid deposits occurring at or near the dermal-epidermal junction in the absence of systemic involvement. Primary cutaneous amyloidosis of the auricular concha and external ear represents a rarely described variant. There have been 27 cases reported in the English language literature, and herein we report 17 additional cases. This article demonstrates that the amyloid observed in this context is generally positive for Congo red, crystal violet and thioflavin T. It also expresses cytokeratin 34ßE12 via immunohistochemistry. Our immunohistochemical results and review of the literature suggest that the amyloid in amyloidosis of the external ear is the result of basal keratinocyte degeneration and does not signify deposition from a systemic or generalized process.
Subject(s)
Amyloid/metabolism , Amyloidosis , Dermis , Ear/pathology , Epidermis , Keratins/metabolism , Skin Diseases , Adult , Aged , Amyloidosis/metabolism , Amyloidosis/pathology , Dermis/metabolism , Dermis/pathology , Epidermis/metabolism , Epidermis/pathology , Female , Humans , Immunohistochemistry , Keratinocytes/metabolism , Keratinocytes/pathology , Male , Middle Aged , Skin Diseases/metabolism , Skin Diseases/pathologyABSTRACT
We report on a 43-year-old Caucasian female who presented with bright red macules in a unilateral distribution in the left C5-8 and L3-5 dermatomes. Histopathologic examination showed superficial papillary dermal telangiectasia with minimal chronic inflammation. Immunohistochemical stains for estrogen and progesterone receptors (ER/PR) were negative. A diagnosis of unilateral nevoid telangiectasia syndrome (UNTS) was given. UNTS is an uncommon disorder first described by Alfred Blaschko in 1899. It is comprised of telangiectasias occurring in a predominantly unilateral dermatomal distribution and often affecting the trigeminal, cervical, and upper thoracic dermatomes. It can be either congenital or acquired and has a 2:1 female:male ratio. UNTS has been reported in relation to hyperestrogenic states, with half of the reported cases related to pregnancy, puberty, or liver disease. However, the vast majority of cases show no increase in estrogen and progesterone receptors in lesional skin. UNTS may be more common than previously believed, and shows some response to vascular laser therapy. Differential diagnoses include hemangioma, angioma serpiginosum, and rarely, nevus flammeus.
