Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis

• Published in: Journal of cutaneous pathology Vol. 47; no. 11; pp. 1050 - 1053
• Main Authors: Vork, Diana L., Shah, Kabeer K., Youssef, Molly J., Wieland, Carilyn N.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.11.2020; Wiley Subscription Services, Inc
• Subjects: acral; Amyloid; Amyloidosis; Biopsy; Bone marrow transplantation; cutis laxa; Electron microscopy; Fibers; Histopathology; Inflammation; Mass spectroscopy; Monoclonal gammopathy; plasma cell dyscrasia; Skin; Stroma
• ISSN: 0303-6987; 1600-0560
• DOI: 10.1111/cup.13791

Acral localized acquired cutis laxa (ALACL) is a rare variant of acquired cutis laxa, and the clinical appearance is characterized by loose, redundant and wrinkled skin of the distal extremities. By definition, histopathology of affected tissue reveals sparse or fragmented elastic fibers. However, this can be difficult to assess on routine staining, and sometimes requires electron microscopy. The condition has been associated with plasma cell dyscrasias or recurrent inflammatory states. We present a case of a 65‐year‐old man who presented with enlarged and doughy finger pads. Skin biopsy showed diffuse dermal amyloid deposition displacing dermal stroma and reduction of elastic fibers, although these changes were subtle on routine hematoxylin and eosin staining. Mass spectrometry of laser capture microdissected tissue showed AL kappa‐type amyloid and further workup revealed a diagnosis of primary systemic AL‐kappa amyloidosis requiring bone marrow transplantation. This case represents an unusual presentation of acquired cutis laxa and highlights the need for a high index of suspicion when reviewing histopathology of this entity. In addition, the case highlights the importance of investigation into possible systemic associations, such as plasma cell dyscrasias.