Increased pigmentation in scleroderma

Increased pigmentation is found in patients with scleroderma (systemic sclerosis, SSc) even in areas that have never clinically been involved with skin thickening. We wanted to determine if the pigmentation is due to subclinical sclerodermatous changes, or a systemic factor such as an increase in adrenocorticotropic hormone (ACTH).

To determine if clinically uninvolved skin that is pigmented differs from nonpigmented skin, skin biopsies were taken from patients with scleroderma from 2 different sites: (1) from pigmented but otherwise clinically normal skin, and (2) from nonpigmented clinically normal skin adjacent to the first site. Biopsies were examined by a dermatopathologist for evidence of dermal and epidermal changes. Cosyntropin stimulation tests were performed after baseline cortisol and ACTH samples were obtained.

Six patients with diffuse SSc (dSSc) and 4 with limited SSc (ISSc) had skin biopsies. Patients with dSSc were more likely than those with ISSc to have increased pigmentation in uninvolved skin. Pigmented skin specimens had either a higher content of epidermal melanin and/or a more severe degree of pigmentary incontinence with a higher number of dermal melanophages in the superficial dermis. The cosyntropin stimulation tests were normal and there were no differences between subjects with diffuse and limited SSc or between those with and without increases in pigmentation.

Clinically uninvolved skin in many of these patients with SSc was abnormal, and subtle changes of SSc were present, especially in the pigmented biopsies. There was no evidence of adrenal deficiency in these patients. It is difficult pathologically to differentiate the changes in pigmented compared to unpigmented skin in patients with SSc, except for changes of increased melanin and pigmentary incontinence.