Nephrogenic fibrosing dermopathy with systemic involvement

William W TING; Mary Seabury Stone; Kathi C MADISON; Kevin KURTZ

doi:10.1001/archderm.139.7.903

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Nephrogenic fibrosing dermopathy with systemic involvement

Journal article

Open access

Peer reviewed

Nephrogenic fibrosing dermopathy with systemic involvement

William W TING, Mary Seabury Stone, Kathi C MADISON and Kevin KURTZ

Archives of dermatology (1960), Vol.139(7), pp.903-906

2003

DOI: 10.1001/archderm.139.7.903

PMID: 12873886

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https://doi.org/10.1001/archderm.139.7.903View

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Abstract

Background: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported. Observations: We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. Autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD. Conclusion: A subset of patients with NFD may have significant systemic involvement.

Dermatology

Biological and medical sciences

Medical sciences

Skin involvement in other diseases. Miscellaneous. General aspects

Details

Title: Subtitle: Nephrogenic fibrosing dermopathy with systemic involvement
Creators: William W TING - Department of Dermatology, University of Iowa College of Medicine, Iowa City, United States
Mary Seabury Stone - Department of Dermatology, University of Iowa College of Medicine, Iowa City, United States
Kathi C MADISON - Department of Dermatology, University of Iowa College of Medicine, Iowa City, United States
Kevin KURTZ - Department of Pathology, University of Iowa College of Medicine, Iowa City, United States
Resource Type: Journal article
Publication Details: Archives of dermatology (1960), Vol.139(7), pp.903-906
DOI: 10.1001/archderm.139.7.903
PMID: 12873886
NLM abbreviation: Arch Dermatol
ISSN: 0003-987X
eISSN: 1538-3652
Publisher: American Medical Association; Chicago, IL
Language: English
Date published: 2003
Academic Unit: Dermatology; Pathology
Record Identifier: 9984025575902771

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