Bhullar Harmeet, Wada Miki, Nikpour Mandana, Saracino Amanda M
Department of Dermatology, Peter MacCallum Cancer Center, Melbourne, VIC, Australia.
Department of Medicine, Alfred Health, VIC, Australia.
J Scleroderma Relat Disord. 2024 Oct;9(3):NP1-NP4. doi: 10.1177/23971983241254442. Epub 2024 May 28.
Morphoea is a disorder characterised by fibrosis and inflammation of the skin and on rare occasions can be precipitated by malignancy. Here, we describe a case of morphoea unmasking two malignancies.
A 73-year-old woman presented with circumferential lower limb skin thickening, associated with violaceous, doughy oedema and significantly impaired mobility. Histology confirmed dermal sclerosis with no increased mucin and broader investigations excluded systemic sclerosis, scleromyxoedema and scleroedema. An atypical morphoea was diagnosed. In the context of atypical and subsequently treatment-resistant disease, further imaging uncovered a lung adenocarcinoma which was promptly treated. Despite this, the patient's atypical oedematous skin sclerosis continued to progress proximally, and she developed flatulence, bloating and atypical flushing. This prompted further investigation, which revealed a metastatic neuroendocrine tumour. The patient was commenced on octreotide, with rapid improvement in all her cutaneous and systemic symptoms.
Atypical morphoea can be a herald for an underlying malignancy, representing a paraneoplastic presentation. Progressive treatment-resistant morphoea may be an indicator of metastatic disease, or in our case a second malignancy.
硬斑病是一种以皮肤纤维化和炎症为特征的疾病，极少数情况下可由恶性肿瘤诱发。在此，我们描述一例硬斑病揭示出两种恶性肿瘤的病例。
一名73岁女性，出现下肢皮肤环形增厚，伴有紫红色、面团样水肿，活动能力明显受损。组织学检查证实为真皮硬化，无黏蛋白增加，进一步检查排除了系统性硬化症、硬化性黏液水肿和硬肿病。诊断为非典型硬斑病。鉴于病情不典型且随后对治疗耐药，进一步影像学检查发现了肺腺癌并立即进行了治疗。尽管如此，患者非典型的水肿性皮肤硬化仍继续向近端发展，并且出现了肠胃胀气、腹胀和非典型潮红。这促使进一步检查，结果发现了转移性神经内分泌肿瘤。患者开始使用奥曲肽治疗，其所有皮肤和全身症状迅速改善。
非典型硬斑病可能是潜在恶性肿瘤的先兆，代表一种副肿瘤表现。对治疗耐药的进行性硬斑病可能是转移性疾病的指标，在我们的病例中则是第二种恶性肿瘤的指标。
