Trufin Ioana Irina, Ungureanu Loredana, Halmágyi Salomea-Ruth, Apostu Adina Patricia, Șenilă Simona Corina
Clinical Hospital of Infectious Diseases, 400000 Cluj-Napoca, Romania.
Department of Dermatology, "Iuliu Hațieganu" University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania.
J Clin Med. 2024 May 1;13(9):2662. doi: 10.3390/jcm13092662.
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.
结节性或瘢痕疙瘩样硬皮病是一种病因不明的罕见疾病，在医学文献中仅有零星提及。它于19世纪首次被认识，然而由于报告的病例数量有限，其分类仍存在争议。这种罕见的硬皮病变体与进行性系统性硬化症或局限性硬斑病相关。临床上，它表现为无症状的结节或斑块，类似于自发性瘢痕疙瘩形成，常见于躯干和近端肢体。最近的文献综述显示，患者以女性为主，平均年龄为44岁。诊断依赖于临床和组织病理学发现，通常显示硬皮病和真性瘢痕疙瘩的重叠特征，这继发于胶原蛋白形成导致的过度纤维化反应。我们报告了一例不寻常的病例，一名70岁女性患者同时存在两种不同亚型的硬斑病（结节性/瘢痕疙瘩样和线性），且仅累及皮肤，这与通常与器官特异性疾病相关的结节性/瘢痕疙瘩样硬皮病的典型表现不同。然而，最近关于系统性硬化症的出版物与之前的有所不同，在我们的描述性文献综述中，2018年至2024年期间未报告有系统性受累情况。由于总共报告的病例不到50例，我们的病例强调了认识这种罕见疾病、确保进行适当评估、治疗和随访的重要性。
