Nodular morphea in a linear pattern

Abstract

A 9‐year‐old black girl presented with papules and nodules in a linear distribution on her right inner arm, right axilla, and right chest ( ). The lesions were hyperpigmented, indurated, and had been present for 3 years. She was otherwise healthy. A biopsy of one of the lesions revealed a sparse superficial and deep perivascular infiltrate of lymphocytes, compactly arranged collagen bundles in the upper part of the dermis, and abundant mucin throughout the reticular dermis, which was consistent with morphea with dermal mucinosis ( ). Biopsy of another lesion revealed classic histologic features of morphea: thickened bundles of collagen crowded in the reticular dermis aligned parallel to the skin surface. 1 Discrete nodules in a linear distribution on the right arm 2 Biopsy of a lesion

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?

“…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]. 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.…”

“…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]. 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].…”

Nodular/Keloidal Scleroderma: Acquired Collagenous Nodules in Systemic Sclerosis

“…As in the plaques of localized scleroderma, these collagenous nodules can occur as an isolated skin disease or they can appear in the setting of SSc 1,2 . Histologically, these nodules can resemble scleroderma: thickened sclerotic collagen bundles without increased spindled fibroblasts intercalating between the collagen, and are therefore termed nodular scleroderma 3 . In contrast, there have also been reports of patients with scleroderma who develop nodules that histologically have the features of keloid: hypocellular zones of thick glassy hyalinized collagen bundles, which have been referred to as keloidal scleroderma 4 .…”