Pigmented skin patches without scleroderma as a predominant clinical symptom revealing systemic sclerosis

Abstract

Skin induration remains the major clinical symptom of systemic sclerosis (SSc), an autoimmune disease with potentially life-threatening visceral involvement. However, skin induration can be absent in some patients, making the diagnosis difficult to confirm and leading to delay in management. Skin pigmentation abnormalities have been reported in patients with SSc, and can be important to recognize for diagnosis. We report two patients who developed hyperpigmented skin patches without any sign of scleroderma, as a major clinical skin symptom of incipient SSc.

Association of skin hyperpigmentation disorders with digital ulcers in systemic sclerosis: Analysis of a cohort of 239 patients

“…6 Several patterns of skin pigmentation disorders have been described so far. [7][8][9][10][11][12][13][14][15][16] The 3 most frequently described types are vitiligo-like depigmentation with perifollicular hyperpigmentation (also called salt-and-pepper skin), 7,12,15 diffuse hyperpigmentation with accentuation in sun-exposed areas, 4,5,16 and combined hyperpigmentation and hypopigmentation localized in the areas of sclerosis.…”

DDR1 and DDR2 in skin

“…The interrelationships between these processes are unknown, and the elucidation of the interplay between these three pathologic hallmarks would enhance knowledge of the pathogenesis of this disease and, consequently, improve treatment options [42]. Around half of SSc patients have pigmentary disturbances (hypo or hyperpigmentation), which can occur prematurely even before fibrosis [43,44]. One of these pigmentary disorders is vitiligo-like depigmentation [45][46][47] but DDR1 expression in melanocytes from these patients has not yet been investigated.…”