Brief ReportMorphea With Keloidal Features: A Case Report and Review of the LiteratureYu, Dongfang MD*; Ibarra, Barbara S. MD*; Akkurt, Zeynep M. MD†; Ahn, Christine MD*,†; Sangüeza, Omar P. MD*,†Author Information Departments of *Pathology, and †Dermatology, Wake Forest University School of Medicine, Winston Salem, NC. Correspondence: Christine Ahn, MD, Assistant Professor of Dermatology, Departments of Pathology, and Dermatology, Wake Forest University School of Medicine, 4618 Country Club Road, Winston Salem, NC 27104 (e-mail: [email protected]). The authors declare no conflicts of interest. The American Journal of Dermatopathology: October 2020 - Volume 42 - Issue 10 - p 766-768 doi: 10.1097/DAD.0000000000001629 Buy Metrics Abstract Keloidal morphea is a rare variant of scleroderma, which often can be clinically confused with keloid or scar formation. We report a 34-year-old woman with a medical history of asthma and Raynaud's phenomenon, presented for the evaluation and management of multiple erythematous hyperpigmented annular plaques reportedly developed after taking trimethoprim/sulfamethoxazole. An initial skin biopsy showed findings supportive of a drug eruption. She was treated with oral prednisone and achieved some improvement. She presented 1 year later with enlargement of the plaques and emergence of new lesions. Skin biopsies revealed an unremarkable epidermis with marked fibrosis of the mid-to-deep dermis with sparing of the papillary dermis, and superficial and deep perivascular and perieccrine lymphoplasmacytic inflammation. Verhoeff–Van Gieson staining demonstrated the loss of elastin fibers within the fibrotic areas of the biopsy specimens, which supported the diagnosis of keloidal morphea. Her laboratory tests were positive for antinuclear antibody (greater than 1:1280). She continued treatment with oral prednisone and topical steroids, and she showed improvement. This case highlights the importance of differentiating keloidal scleroderma from a hypertrophic scar or keloid to reveal an underlying systemic process. A correlation of clinical and histopathological findings is paramount to reach a correct diagnosis, ensure appropriate treatment, and monitor for comorbid disease. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.