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Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis.
J Cutan Pathol. 2020 Jun 28 [Online ahead of print]JC

Abstract

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.

Authors+Show Affiliations

Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

32594539

Citation

Vork, Diana L., et al. "Acral Localized Acquired Cutis Laxa as Presenting Sign of Underlying Systemic Amyloidosis." Journal of Cutaneous Pathology, 2020.
Vork DL, Shah KK, Youssef MJ, et al. Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis. J Cutan Pathol. 2020.
Vork, D. L., Shah, K. K., Youssef, M. J., & Wieland, C. N. (2020). Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis. Journal of Cutaneous Pathology. https://doi.org/10.1111/cup.13791
Vork DL, et al. Acral Localized Acquired Cutis Laxa as Presenting Sign of Underlying Systemic Amyloidosis. J Cutan Pathol. 2020 Jun 28; PubMed PMID: 32594539.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis. AU - Vork,Diana L, AU - Shah,Kabeer K, AU - Youssef,Molly J, AU - Wieland,Carilyn N, Y1 - 2020/06/28/ PY - 2020/02/05/received PY - 2020/05/24/revised PY - 2020/06/17/accepted PY - 2020/7/1/pubmed PY - 2020/7/1/medline PY - 2020/6/29/entrez KW - acral KW - amyloidosis KW - cutis laxa KW - plasma cell dyscrasia JF - Journal of cutaneous pathology JO - J. Cutan. Pathol. N2 - 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. SN - 1600-0560 UR - https://www.unboundmedicine.com/medline/citation/32594539/Acral_localized_acquired_cutis_laxa_as_presenting_sign_of_underlying_systemic_amyloidosis_ L2 - https://doi.org/10.1111/cup.13791 DB - PRIME DP - Unbound Medicine ER -
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