Keloid Morphea and Nodular Scleroderma: Two Distinct Clinical Variants of Scleroderma?

Abstract

Background: For nearly a century, the terms “keloid morphea” and “nodular scleroderma” have been used interchangeably without defined clinical or histologic criteria. Objective: To define the conditions “keloid morphea” and “nodular scleroderma” by correlating the clinical and histologic features. Methods: We retrospectively identified six patients with keloidal lesions and nodules from 70 consecutive patients with scleroderma seen in the dermatology clinic. The clinical presentation and histopathological findings were reviewed. Results: Six of 70 patients with scleroderma (45 systemic and 25 morphea) exhibited keloidal or nodular lesions. All these patients had systemic sclerosis. Clinically one patient (case 1) had nodules; five (cases 2–6) had keloids. The nodular lesions had histologic findings consistent with keloid, while the keloidal plaques were variably keloids or morphea histopathologically. There was no correlation between the clinical morphology and the histologic findings, except for cases 5 and 6. These patients were African–American, with a family history of keloids and typical keloids clinically and histologically that developed from sites with normal skin. Conclusion: Keloid morphea and nodular scleroderma are clinical terms that describe keloidal and nodular lesions in patients most commonly with scleroderma. The clinicopathologic association is variable. Based on a review of the English literature and our series of six patients, we identified two clinical variants; (1) keloidal or nodular lesions arising from sclerodermatous skin with histologic findings of keloid or scleroderma, (2) typical keloids clinically and histologically, arising in normal skin in patients with a family history of keloids. Awareness of these entities is important for proper diagnosis of the cutaneous lesions and for recognizing that the cutaneous findings may be a sign of systemic sclerosis.