Reticulate hyperpigmentation in systemic sclerosis: a case report and review of the literature

Abstract

Introduction: Systemic sclerosis is a systemic connective tissue disease with variable cutaneous presentations. Although pigmentary disturbances have been described in systemic sclerosis, a reticulate hyperpigmentation has only been reported in one case of systemic sclerosis to date.

Case presentation: We describe a previously healthy 51-year-old Thai woman who presented with a reticulate hyperpigmentation affecting her trunk and extremities, together with sclerodactyly and proximal sclerosis, resulting in a new diagnosis of systemic sclerosis.

Conclusions: To date, the pathogenesis of reticulate hyperpigmentation in systemic sclerosis remains unclear. Increased melanin synthesis and altered thermoregulatory mechanism are proposed to be involved in the pathogenesis of this presentation. This case represents an unusual cutaneous feature of reticulate hyperpigmentation in the setting of systemic sclerosis.

Fig. 1

Clinical presentation. a, b Widespread reticulate hyperpigmentation over the trunk and extremities with discrete erythematous indurated plaques over the chest wall and anterior abdomen, c Sclerodactyly with cool periphery of all fingers

Fig. 2

Histopathological findings. A biopsy from the hyperpigmented induration on the right thigh showed increased basal pigmentation with pan-dermal sclerosis. Mild superficial and deep perivascular and perieccrine lymphocytic infiltrate was observed with some dilated dermal blood vessels and decreased adventitious fat surrounding sweat glands. (Hematoxylin and eosin, ×10 objective)