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

TL;DR

This paper reports a rare case of Sjögren's syndrome associated with ashy dermatosis in a 50-year-old woman from Pakistan.

Contribution

The study presents the first reported case of ashy dermatosis linked to Sjögren's syndrome from Pakistan.

Findings

A 50-year-old woman was diagnosed with Sjögren's syndrome and ashy dermatosis through biopsy.

The case highlights the rare dermatological manifestation of erythema dyschromicum perstans in Sjögren's syndrome.

This is the first documented case of the condition in Pakistan.

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.

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