Serpentine Supravenous Hyperpigmentation – A Rare Cutaneous Manifestation of Progressive Systemic Sclerosis

A 35-year-old male complaints of binding down of skin and increased pigmentation over body for the past 3 years. Cutaneous examination revealed binding down of skin over face, neck, trunk and limbs. Salt and pepper pigmentation along with retained pigmentation over supratrochlear and supraorbital veins and also over their immediate branches was present over forehead and temple area [Figure 1]. This preservation of normal pigment overlying the superficial veins is known as serpentine supravenous hyperpigmentation. The pathophysiology behind this is depicted in Figure 2.

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Figure 1:

Salt and pepper pigmentation along with serpentine supravenous hyperpigmentation over forehead and temple area.

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Figure 2:

Pathophysiology of serpentine supravenous hyperpigmentation in systemic sclerosis. IL: interleukin, TNF: Tumor necrosis factor.

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Jawitz et al.^[1] first described pigment retention over temporal and scalp vessels in three patients of progressive systemic sclerosis (SSc). Sindhuja et al.^[2] also reported supraventine sparing over forehead and frontal scalp in a 13-year-old diffuse SSc patient.

All patients with supraventine sparing reported in literature had progressive SSc including our patient. Therefore, it can serve as an important cutaneous sign of progressive SSc.