Nodular scleroderma

Abstract

We describe a 40-year-old woman with systemic scleroderma who had hundreds of firm nodules that developed on the trunk and upper extremities during several months. We briefly review previously reported cases of this rare variant of scleroderma.

Nodular Scleroderma: Increased Expression of Connective Tissue Growth Factor

“…Nodular scleroderma is a fibrosing condition, usually associated with SSc [11, 12, 13, 14, 15, 16, 17, 18, 19, 20]or morphea [21, 22, 23, 24, 25, 26, 27]. Additionally, a case of nodular scleroderma showing no association with scleroderma has recently been reported [28].…”

What Is Nodular-Keloidal Scleroderma?

“…Histological findings are likewise highly variable and may be characteristic of hypertrophic or keloid scarring [10,12,13,16], of scleroderma [9,11,23,25,27], of both morphea and keloid type in the same biopsy [8,15,24] or of morphea-type initially and keloid-type subsequently [29]. In the patient reported in this issue [35], there were no histological signs of scleroderma in biopsies performed 5-20 months after disease onset.…”

“…Some authors [27,31] also question whether cases with subcutaneous lesions are a type of deep morphea, which are different from those with raised lesions. We would suggest that the various clinical manifestations observed in fact reflect the existence of two basic types of lesion, the first hemispherical and 2-30 mm in diameter and the second plaque-like, which are modified by 4 factors: -Lesions may differ in their vertical level: both types of lesion may be subcutaneous only or, raised above the surrounding skin, entirely subcutaneous hemispheric lesions or nodules [8,23], raised hemispherical lesions or papules [8,11,13,16,17,24,25,27], subcutaneous plaques with the appearance of deep morphea [32] and raised plaques with the appearance of keloids [9,31]. -Lesions may occur on normal skin or on skin affected by the previous scleroderma; thus, some patients show sclerotic papules [8,25,28,29] or subcutaneous nodules [8] on previously sclerotic skin, while others show lesions on normal skin [10,16], which may remain isolated or converge to form subcutaneous or raised plaques.…”

“…Almost all patients had suffered from classic scleroderma for months [11,19] or years [10,12,13,15,24,25,29] before the onset of the keloidal lesions, these being either localized or generalized morphea lesions [6,7,9,[23][24][25][26][27][28][29][30][31][32][33] or systemic sclerosis [4,5,8,[10][11][12][13][14][15][16][17]. However, in 4 cases the keloidal lesions were the only manifestation of a putative underlying scleroderma [6,7,27,32]; in 2 cases the type of associated scleroderma is not known because we have been unable to consult the original or related reports [18,21], while another 2 cases have subsequently been considered as possible scleromyxoedemas, despite the original diagnosis of keloidal scleroderma [22,34].…”

Nodular/Keloidal Scleroderma: Acquired Collagenous Nodules in Systemic Sclerosis

“…Nodular or keloidal scleroderma has been reported as a variant of localized scleroderma that can occur alone, variably referred to as nodular or keloidal morphea 6 , or in patients with SSc 2 . Clinically, the skin lesions are characteristically firm, nontender papules that can occur in isolated 7 , generalized 8 , and linear 3 distributions with a predilection for the trunk 9 . Most lesions have been reported to develop within the first few months of the onset of SSc symptoms, during a time of clinically active disease 8,10 .…”