Case of salt-and-pepper skin changes in systemic sclerosis
This article describes the case of a 68-year-old man who presented to the rheumatology clinic with skin changes on his chest and back with a history of 5 years. His medical history revealed that he was a smoker and had been hospitalised for shortness of breath, 3 months ago. Doctors advised a computed tomography of the chest which showed fibrotic and emphysematous changes in the lower lobes of both lungs. A transthoracic echocardiography was also done which showed elevated pulmonary artery systolic pressure. Physical examination was consistent with clubbing of the fingers, crackles in both lung bases and “salt and pepper” changes on the chest and back. The areas were characterised with hypopigmentation and hyperpigmentation in areas of sclerotic skin. In this case the diffuse skin thickening was seen extending down each arm to a point proximal of the elbow.
Diagnosis and treatment
The patient further tested positive for antitopoisomerase I (anti–Scl-70) antibodies. Based on these findings, the patient was diagnosed with cutaneous systemic sclerosis. Treatment included immunosuppressive therapy. 3 months later there was partial regression of skin thickening with salt-and-pepper changes. The patient also reported that his shortness of breath improved.
Systemic sclerosis is an autoimmune disease that causes small blood vessels to occlude, activates the immune system and leads to abnormal fibrosis of the skin and other organs. The salt-and-pepper appearance, vitiligo like skin manifestation, is a characteristic feature of the autoimmune disease. The skin changes are also evident in the sclerotic area. Previous studies have shown that majority of the patients diagnosed with systemic sclerosis had a salt-and-pepper appearance.
In such cases, clinicians are advised to focus on characteristic skin findings that can lead to the diagnosis of the autoimmune disease. External factors such as trauma and inflammation are also thought to trigger melanocytes, based on immunological factors. Salt-and-pepper appearance is one of the earliest cutaneous findings and sometimes also the only finding.
References
Salt-and-Pepper Skin Changes in Systemic Sclerosis https://www.nejm.org/doi/full/10.1056/NEJMicm2103390