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Firm Red-Brown Plaques in a Patient With Systemic Scleroderma

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 60s with anti-RNA polymerase III antibody–positive systemic sclerosis complicated by scleroderma kidney crisis, heart failure, interstitial lung disease, sclerodactyly, and Raynaud phenomenon while receiving mycophenolate mofetil, 1 g twice daily, presented with a pruritic rash that had been present for 3 months. The patient denied any known preceding triggers or trauma to the area of the rash. He had tried treating with topical aloe, without improvement. Review of systems was negative for any new or worsening constitutional, respiratory, or gastrointestinal symptoms. The patient’s medical history was otherwise unremarkable. Examination findings were notable for firm, red-brown plaques without overlying scale on the proximal upper extremities and anterior trunk (Figure, A and B). There was no appreciable skin tightening in the affected areas. Complete blood cell count with differential was unremarkable. Lesional punch biopsy specimens were obtained (Figure, C and D).

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C. Keloidal scleroderma

In light of the patient’s history of scleroderma, the clinical and histopathologic findings were compatible with keloidal scleroderma, also known as nodular scleroderma, pseudokeloidal scleroderma, keloidal morphea, and nodular morphea.13 Given the patient’s known systemic sclerosis and clinical and histologic findings akin to keloids, we prefer the term keloidal scleroderma for this case. Keloidal scleroderma is a rare variant of scleroderma in which lesions can be morphologically and histopathologically indistinguishable from hypertrophic scar or keloid.1,3,4 Other times, such as in the present case, lesions can appear scarlike but not entirely classic for keloids. Lesions can occur either in sclerotic or nonsclerotic skin and either before or after a diagnosis of systemic sclerosis is made.1,3,4 It is postulated to represent a keloidal response to skin inflammation inherent to systemic sclerosis in individuals already predisposed to keloid formation.2,3,5,6

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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Natasha C. Zacher, MD, Department of Dermatology, Stanford University School of Medicine, 450 Broadway, Pavilion C, 2nd Floor, Redwood City, CA 94063 (nzacher@stanford.edu).

Published Online: August 17, 2022. doi:10.1001/jamadermatol.2022.3241

Conflict of Interest Disclosures: Dr Rieger reported receiving personal fees from Pfizer for consulting outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Perez-Wilson  J , Pujol  RM , Alejo  M , Geli  C , de Moragas  JM .  Nodular (keloidal) scleroderma.   Int J Dermatol. 1992;31(6):422-423. doi:10.1111/j.1365-4362.1992.tb02673.xPubMedGoogle ScholarCrossref
2.
James  WD , Berger  TG , Butler  DF , Tuffanelli  DL .  Nodular (keloidal) scleroderma.   J Am Acad Dermatol. 1984;11(6):1111-1114. doi:10.1016/S0190-9622(84)70268-4PubMedGoogle ScholarCrossref
3.
Yu  D , Ibarra  BS , Akkurt  ZM , Ahn  C , Sangüeza  OP .  Morphea with keloidal features: a case report and review of the literature.   Am J Dermatopathol. 2020;42(10):766-768. doi:10.1097/DAD.0000000000001629PubMedGoogle ScholarCrossref
4.
Wriston  CC , Rubin  AI , Elenitsas  R , Crawford  GH .  Nodular scleroderma: a report of 2 cases.   Am J Dermatopathol. 2008;30(4):385-388. doi:10.1097/DAD.0b013e3181766177PubMedGoogle ScholarCrossref
5.
Lortscher  DN , Cohen  PR , Bangert  CA , Paravar  T .  Nodular scleroderma revisited: systemic sclerosis presenting as annular keloidal sclerotic plaques.   J Clin Aesthet Dermatol. 2016;9(6):56-57.PubMedGoogle Scholar
6.
Krell  JM , Solomon  AR , Glavey  CM , Lawley  TJ .  Nodular scleroderma.   J Am Acad Dermatol. 1995;32(2 pt 2):343-345. doi:10.1016/0190-9622(95)90400-XPubMedGoogle ScholarCrossref
7.
Barzilai  A , Lyakhovitsky  A , Horowitz  A , Trau  H .  Keloid-like scleroderma.   Am J Dermatopathol. 2003;25(4):327-330. doi:10.1097/00000372-200308000-00007PubMedGoogle ScholarCrossref
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