Scleroderma with Nodular Scleroderma

Abstract

Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition. Although no additional treatment was given, the lesions remained stable without progression. Conclusions: Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques.

Nodular morphea keloidal type: A rare case with paradigmatic histopathology significantly accompanied by a flawless surgical scar

“…It is clinically characterized by one or more raised lesions that usually occur in association with classic morphea, developing on the affected areas or on apparently normal skin. Similar abnormal growths can arise in the course of generalized scleroderma, thus distinguishing a form of the disease specified as nodular scleroderma 4‐6 …”

“…Similar abnormal growths can arise in the course of generalized scleroderma, thus distinguishing a form of the disease specified as nodular scleroderma. [4][5][6] These growths usually appear as nondescript protuberances, histopathologically composed of sclerodermatous tissue, [1][2][3][4][5][6] but sometimes they show specific clinical and histopathological characteristics of keloids. [7][8][9][10][11][12][13] In this case, they have a firm consistency and an uneven surface with arciform or irregularly curvy contours, which can protrude outward with pseudopod-like projections.…”

“…12,13 Our case meets the criteria for the clinical and histopathological diagnosis of a keloidal type of nodular morphea. [1][2][3][4][5][6][7][8][9][10][11][12][13] In fact, it is characterized by firm keloidal-like plaques not related to a side effect of the radiotherapy, which was administered several years after their first appearance. Our findings of negative autoimmune serology are consistent with the literature data.…”

“…[1][2][3] Accordingly, the association of such outgrowths with systemic sclerosis is designated as nodular scleroderma. [4][5][6] Sometimes these lesions appear as firm, erythematous and irregularly curvy plaques resembling keloids or hypertrophic scars, thus characterizing keloidal morphea or keloidal scleroderma. [7][8][9][10] These mystifying features can make the diagnosis challenging, especially in the absence of a well-documented medical history.…”

“…Nodular morphea is a rare variant of localized scleroderma, clinically and histopathologically characterized by cutaneous nodules or plaques associated or superimposed to the flat lesions of classic morphea 1‐3 . Accordingly, the association of such outgrowths with systemic sclerosis is designated as nodular scleroderma 4‐6 . Sometimes these lesions appear as firm, erythematous and irregularly curvy plaques resembling keloids or hypertrophic scars, thus characterizing keloidal morphea or keloidal scleroderma 7‐10 .…”