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

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.

Keywords: ashy dermatosis; erythema cenicienta; erythema dyschromicum perstans; sjogren syndrome antibodies or ssa ssb; sjogren syndrome.

Figure 1. Hyperpigmented, violaceous linear macules and patches on the anterolateral and posterior aspects of the neck, consistent with EDP

EDP: erythema dyschromicum perstans

Figure 2. Violaceous, purplish macules and patches on the dorsal and ventral surfaces of the forearms and hands, consistent with EDP

EDP: erythema dyschromicum perstans

Figure 3. Violaceous, purplish macules on the abdomen

Figure 4. The overall architecture of the epidermis and dermis, including the undulating rete ridges (a), pigmentary incontinence (b), and a few colloid bodies (c)

Histopathological examination of skin tissue (Hematoxylin & Eosin stain). (Above) Low-power view (10x objective). (Below) High-power view (40x objective). Scale bars: A=10 µm, B=20 µm.

Figure 5. Sections from the minor salivary gland show mucinous acini with abundant foamy macrophages (top right), lymphoid aggregates in a background of interstitial fibrosis (top left), and mucinous acini with a few atrophied glands (bottom right and left images), suggestive of Sjögren's syndrome

Histopathological examination of minor labial salivary gland tissue (Hematoxylin & Eosin stain). (Top right) Low-power view (4x objective). (Top left) High-power view (40x objective). (Below right and left) Intermediate magnification (10x-20x objective). Scale bars: top right=200 µm, top left=20 µm, below right and left=50 µm.