Case Report: Can Salt-&-Pepper Skin Mean Systemic Sclerosis?

A salt-and-pepper appearance of the skin is … one of the earliest cutaneous findings in systemic sclerosis.

Discussion

Figure 5. A skin biopsy revealed superficial and deep dermal perivascular lymphocytic infiltrate and focally increased dermal collageny.

Systemic sclerosis is a heterogeneous disorder characterized by dysfunction of the endothelium, dysregulation of fibroblasts (resulting in excessive production of collagen) and immune system abnormalities.⁷ Although the cause of SSc remains unknown, genetic and environmental factors are believed to contribute to host susceptibility.⁸ SSc is divided into diffuse cutaneous and limited cutaneous syndromes, depending on the extent of skin thickening. These classically manifest as Raynaud’s phenomenon, sclerodactyly, esophageal dysmotility and a range of organ-specific complications.¹ Systemic involvement may occur in the form of pulmonary vasculopathy, interstitial fibrosis, cardiomyopathy, arrhythmia, conduction abnormality, acute renal crisis, etc.²

A salt-and-pepper appearance features patchy hypopigmentation and hyper­pigmentation of the skin manifesting as vitiligo-like depigmentation with perifollicular pigmentary retention.⁵ Vitiligo and the depigmented lesions in SSc share many features. Clinically, both are characterized by chalk-white macules with well-defined borders. Both cellular and humoral immune factors, in combination with external factors such as trauma or inflammation, may trigger the destruction of melanocytes.⁶

In 2009, Adelowo et al. found that salt-and-pepper skin emerged as the most common presentation among 14 Nigerian SSc patients.¹⁰ In 2006, Sharma et al., in a study of 100 SSc patients performed at the All India Institute of Medical Sciences in New Delhi, found that 51% of SSc patients had a salt-and-pepper skin appearance.¹¹

A salt-and-pepper skin appearance is just one of many changes observed in the skin of patients with systemic sclerosis, but we think paying attention to this sign may help early diagnosis of systemic sclerosis. And again, delayed diagnosis may be associated with complications, such as pulmonary fibrosis or hypertension, myocarditis, pericardial effusion, arthritis or renal involvement.

Wesam Gouda, MBBCh, MSc, is an assistant lecturer of rheumatology at Aswan University, Egypt.

Abdelhafeez Moshrif, MBBCh, MSc, MD, is a lecturer of rheumatology at Al-Azhar University, Assiut, Egypt.

Fatma H. El Nouby, MBBCh, MSc, MD, is a lecturer of rheumatology in the rheumatology department at Aswan University, Egypt.

Amal Fehr, MBBCh, MSc, MD, is a lecturer of rheumatology in the rheumatology department at Helwan University, Egypt.

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