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

Castelli, E.; Orlando, Elisabetta; Pardo, Nicola; Turdo, Alice; Pistone, Giuseppe; Bongiorno, Maria Rita

doi:10.1111/cup.13810

Cited by 1 publication

(6 citation statements)

References 21 publications

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“…All patients exhibited characteristic skin lesions and a potential systemic involvement was evaluated. We identified four case reports [1,[8][9][10], and together with the case from our institution, a total number of five patients with nodular/keloidal scleroderma. Significant clinical and serological information is summarized in Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis is supported by the presence of keloidal collagen aggregations (bright eosinophilic bundles) and a variable number of fibroblasts and/or myofibroblasts on a background of classical scleroderma (thickened hypocellular dermis with swollen collagen bundles) [9]. In rare cases, there are also histological descriptions of focal groups of spindle cells and lymphoplasmacytic inflammatory infiltrates, the latter being described in more recent lesions as small perivascular and periadnexal cellular groups [2,8,9].…”

Section: Histologymentioning

confidence: 99%

“…Although there is a noticeable variety in the clinical picture, most cases show multiple papular lesions in areas prone to keloid formation, such as the neck, trunk, upper arms, and legs [4]. In some cases, they mimic the morphology of keloids, with a firm consistency, irregular surface, and arciform margins, which can extend with pseudopodlike projections [9]. However, unlike true keloids, the lesions in nodular/keloidal scleroderma are generally asymptomatic, with pruritus reported only in a small number of cases [4].…”

Section: Clinical Picturementioning

confidence: 99%

“…Histological findings exhibit an endo-exophytic growth determined by a mixture of dermal sclerosis with eosinophilic or glassy collagen. The hyalinized or homogenized collagen fibers may be oriented in whorl-like patterns in hypocellular areas (keloid-specific) or run parallel to the epidermis (sclerodermatous features); the center of the lesion may also include fragmented elastic fibers [2,8,9]. The diagnosis is supported by the presence of keloidal collagen aggregations (bright eosinophilic bundles) and a variable number of fibroblasts and/or myofibroblasts on a background of classical scleroderma (thickened hypocellular dermis with swollen collagen bundles) [9].…”

Section: Histologymentioning

confidence: 99%

“…The hyalinized or homogenized collagen fibers may be oriented in whorl-like patterns in hypocellular areas (keloid-specific) or run parallel to the epidermis (sclerodermatous features); the center of the lesion may also include fragmented elastic fibers [2,8,9]. The diagnosis is supported by the presence of keloidal collagen aggregations (bright eosinophilic bundles) and a variable number of fibroblasts and/or myofibroblasts on a background of classical scleroderma (thickened hypocellular dermis with swollen collagen bundles) [9]. In rare cases, there are also histological descriptions of focal groups of spindle cells and lymphoplasmacytic inflammatory infiltrates, the latter being described in more recent lesions as small perivascular and periadnexal cellular groups [2,8,9].…”

Section: Histologymentioning

confidence: 99%

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Nodular/Keloidal Scleroderma with No Systemic Involvement—A Case Report and a Review of the Literature

Trufin,

Ungureanu,

Halmágyi

et al. 2024

JCM

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Nodular or keloidal scleroderma is a rare condition with unclear cause and sporadic mentions in the medical literature. It was first recognized in the 19th century, yet its classification is still debated due to the limited number of reported cases. This rare variant of scleroderma is associated with either progressive systemic sclerosis or localized morphea. Clinically, it presents with asymptomatic nodules or plaques, resembling spontaneous keloid formation, often found on the trunk and proximal extremities. Recent literature reviews show a predominance of women with a mean age of 44 years. Diagnosis relies on clinical and histopathological findings, which usually show overlapping features of both scleroderma and true keloids, secondarily to an excessive fibrosing reaction attributed to collagen formation. We present an unusual case of a 70-year-old female patient who displayed the coexistence of two distinct subtypes of morphea (nodular/keloidal and linear), and exclusive skin involvement, which contrasts with the typical presentation of nodular/keloidal scleroderma, often associated with organ-specific disease. However, recent publications have diverged from previous ones regarding systemic sclerosis, with no systemic involvement reported between 2018 and 2024, which we evaluated in our descriptive literature review. With less than 50 cases reported in total, our case underlines the importance of recognizing this rare disease, ensuring appropriate evaluation, treatment, and follow-up.

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Section: Discussionmentioning

confidence: 99%