Frequency of cutis laxa-like clinical features and elastolysis in scleromyxedema: A retrospective clinicopathologic study of 19 patients with scleromyxedema

Tania M. Gonzalez Santiago, Julia Lehman, J. Noelle Buonaccorsi, Amer N. Kalaaji, Carilyn N. Wieland

Research output: Contribution to journalArticle

Abstract

Background: Cutis laxa-like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining. Methods: We retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. We also evaluated available skin biopsies with an elastin stain and assessed whether dermal elastin fibers were diminished in density or were fragmented (or both). Results: Nineteen patients with scleromyxedema and 34 skin biopsies were identified. Alcian blue (mucin) stain was used to grade mucin deposition as weakly positive (24%), positive (44%) and markedly positive (32%). Eight patients (42%) had clinical findings of cutis laxa, which were often observed in conjunction with areas of papular eruption or induration. Elastic tissue fibers were normal in 9 of 34 skin specimens (26%), 18 of 34 specimens (53%) had diminished elastic fiber density and 7 of 34 (21%) had markedly decreased density. The elastic tissue was fragmented in 25 specimens (74%). Conclusions: A cutis laxa-like clinical presentation and decreased elastic tissue density on skin biopsy were consistent findings. Dermatologists and dermatopathologists should be aware of these previously unreported clinical and histopathologic findings.

Original languageEnglish (US)
JournalJournal of Cutaneous Pathology
DOIs
StateAccepted/In press - 2016

Fingerprint

Scleromyxedema
Cutis Laxa
Elastic Tissue
Retrospective Studies
Skin
Elastin
Mucins
Biopsy
Coloring Agents
Alcian Blue
Staining and Labeling

Keywords

  • Cutis laxa
  • Elastic tissue stain
  • Lichen myxedematosus
  • Monoclonal gammopathy
  • Scleromyxedema

ASJC Scopus subject areas

  • Dermatology
  • Pathology and Forensic Medicine
  • Histology

Cite this

Frequency of cutis laxa-like clinical features and elastolysis in scleromyxedema : A retrospective clinicopathologic study of 19 patients with scleromyxedema. / Gonzalez Santiago, Tania M.; Lehman, Julia; Buonaccorsi, J. Noelle; Kalaaji, Amer N.; Wieland, Carilyn N.

In: Journal of Cutaneous Pathology, 2016.

Research output: Contribution to journalArticle

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abstract = "Background: Cutis laxa-like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining. Methods: We retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. We also evaluated available skin biopsies with an elastin stain and assessed whether dermal elastin fibers were diminished in density or were fragmented (or both). Results: Nineteen patients with scleromyxedema and 34 skin biopsies were identified. Alcian blue (mucin) stain was used to grade mucin deposition as weakly positive (24{\%}), positive (44{\%}) and markedly positive (32{\%}). Eight patients (42{\%}) had clinical findings of cutis laxa, which were often observed in conjunction with areas of papular eruption or induration. Elastic tissue fibers were normal in 9 of 34 skin specimens (26{\%}), 18 of 34 specimens (53{\%}) had diminished elastic fiber density and 7 of 34 (21{\%}) had markedly decreased density. The elastic tissue was fragmented in 25 specimens (74{\%}). Conclusions: A cutis laxa-like clinical presentation and decreased elastic tissue density on skin biopsy were consistent findings. Dermatologists and dermatopathologists should be aware of these previously unreported clinical and histopathologic findings.",
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