Nodular Scleroderma: A Report of 2 Cases

Abstract

Nodular scleroderma, also known as keloidal scleroderma, is a rare form of scleroderma that may occur with either systemic sclerosis or localized scleroderma. Clinically, this disorder is characterized by keloidal nodules that form in sclerodermatous areas. These nodules may histologically show the presence of keloidal collagen. Because of the rarity of this condition, clinicians may not be familiar with the clinical and histologic features relevant to this scleroderma variant. In this report, we describe 2 cases of nodular scleroderma.

Nodular scleroderma presenting as multiple spontaneous keloidal scars

“…[1][2][3] The presence of Raynaud phenomenon (even mild cases), high-titer antinuclear antibodies, and salt-and-pepper pigment changes similar to those found in this patient are suggestive of scleroderma and warrant further evaluation. 1 (Fig 1). A bone scan performed because of low back pain indicated sacral recurrence and bony metastases in the L2 and T7 vertebrae, right scapula, and left 10th rib.…”

Scleroderma with Nodular Scleroderma

“…Nodular or keloidal scleroderma is a rare variant of scleroderma [1, 2, 3, 4, 5]. These terms can be used interchangeably.…”

“…Nevertheless, in the literature, they are sometimes used as distinctive entities. The term “nodular scleroderma” is used when the pathological change resembles scleroderma, and the term “keloidal scleroderma” is used when the pathological change shows hyalinization of thick sclerotic collagens similar to keloid [1, 6, 7]. …”

“…Nodular or keloidal scleroderma have been reported in either systemic sclerosis or localized scleroderma, usually 6 months after the presentation of systemic sclerosis [1, 2, 3, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17]. However, also the presence of nodular scleroderma before the onset of systemic sclerosis has been reported [12].…”

“…However, also the presence of nodular scleroderma before the onset of systemic sclerosis has been reported [12]. This condition can be found in systemic sclerosis patients either with or without active systemic involvements [1, 2, 8, 11, 12, 13, 14, 15, 16, 17, 18, 19]. …”