Sjögren’s Syndrome Associated With Erythema Dyschromicum Perstans: A Rare Dermatological Manifestation

Sjögren's syndrome is an autoimmune disorder with a complex, multifactorial etiopathogenesis that predominantly affects women, typically in their middle-aged years. The condition is associated with a variety of skin manifestations beyond the characteristic skin tightening and thickening. These include erythema multiforme, lichen planus, erythema nodosum (dermo-panniculitis), chilblain-like erythema, vasculitis, livedo reticularis, and granuloma annulare. One rare dermatological manifestation of Sjögren's syndrome is erythema dyschromicum perstans (EDP), also known as ashy dermatosis or dermatosis cenicienta, which is an acquired condition characterized by symmetrical hyperpigmentation on the trunk and extremities. Although few cases of ashy dermatosis have been reported in association with Sjögren's syndrome, we present the case of a 50-year-old woman diagnosed with Sjögren's syndrome and ashy dermatosis based on biopsy, marking what appears to be the first reported case from Pakistan.

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Journal
Cureus
Published
2025-05-16
DOI
https://doi.org/10.7759/cureus.84234
Primary Topic
Salivary Gland Disorders and Functions
Type
article
Field-Weighted Citation Impact
0.00
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article

Sjögren’s Syndrome Associated With Erythema Dyschromicum Perstans: A Rare Dermatological Manifestation

Zara Saeed, Humaira Talat, Naseem Shah, Sulhera Khan et al.
Cureus
Salivary Gland Disorders and Functions
article

Sjögren’s Syndrome Associated With Erythema Dyschromicum Perstans: A Rare Dermatological Manifestation

Zara Saeed, Humaira Talat, Naseem Shah, Sulhera Khan, Sonia Golani
article en

Abstract

Sjögren's syndrome is an autoimmune disorder with a complex, multifactorial etiopathogenesis that predominantly affects women, typically in their middle-aged years. The condition is associated with a variety of skin manifestations beyond the characteristic skin tightening and thickening. These include erythema multiforme, lichen planus, erythema nodosum (dermo-panniculitis), chilblain-like erythema, vasculitis, livedo reticularis, and granuloma annulare. One rare dermatological manifestation of Sjögren's syndrome is erythema dyschromicum perstans (EDP), also known as ashy dermatosis or dermatosis cenicienta, which is an acquired condition characterized by symmetrical hyperpigmentation on the trunk and extremities. Although few cases of ashy dermatosis have been reported in association with Sjögren's syndrome, we present the case of a 50-year-old woman diagnosed with Sjögren's syndrome and ashy dermatosis based on biopsy, marking what appears to be the first reported case from Pakistan.

CureusVol. 17(5)
Jinnah Postgraduate Medical Center (PK)
Zero hunger
Openalex Percentile: Top 86%
Salivary Gland Disorders and Functions
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