Novel case of a scleroderma-mimicking syndrome associated with gastrointestinal stromal tumour

Abstract

We report a case of a 54-year-old man who developed an atypical systemic syndrome involving Raynaud’s phenomenon, pulmonary fibrosis and skin thickening. These features were initially suggestive of newly diagnosed scleroderma. However, he displayed atypical clinical features of same, antinuclear antibody was negative and symptoms were refractory to various immunosuppressive therapies. CT imaging revealed a gastric mass, which later proved to be a gastrointestinal stromal tumour (GIST). Resection of the GIST leads to minimal symptomatic improvement. Surveillance imaging 1 year later revealed metastatic deposits. He was subsequently initiated on imatinib therapy, which led to a rapid improvement in fibrotic changes within weeks. While there have been previous descriptions of paraneoplastic fibrotic disorders, this is the first description of a scleroderma mimic in the setting of a GIST. It highlights an important potential overlap in the pathogenesis of these disease processes and the potential efficacy of tyrosine kinase inhibitors for scleroderma-like fibrotic disorders.

Sclerodermatous eruption in a patient with metastatic colon cancer treated with an FLT3/CDK inhibitor

“…In 2001, Vilalta et al 1 described a technique called plicature and forced approximation, also known as forced advancement plicature (FAP), with the aim of obtaining the advantages of second intention healing and accelerating patient recovery. It consists in identifying the direction in which the edges of the wound are the closest and applying interrupted stitches on the mentioned axis (Figures 1 and 2).…”

“…This technique decreases the wound diameter and inverts the edges of the skin towards the wound bed, facilitating keratinocyte production and migration to the deepest areas of the wound. 1,2 Nevertheless, further data related to some aspects of this technique are currently very scarce. 1,3 Therefore, we sought to measure and evaluate the functional and aesthetic outcomes of FAP in a cohort of our hospital.…”

“…1,2 Nevertheless, further data related to some aspects of this technique are currently very scarce. 1,3 Therefore, we sought to measure and evaluate the functional and aesthetic outcomes of FAP in a cohort of our hospital. Patients were included between January 2020 and June 2021.…”

“…5 Scleroderma can also develop as a paraneoplastic phenomenon, reported in association with breast, lung, liver, hematologic cancers, and gastrointestinal stromal tumours (GIST). 1,4 It is possible that our patient's skin manifestations are a paraneoplastic phenomenon associated with his metastatic colon adenocarcinoma. Close temporal relationships have been observed between onset of cancer and scleroderma across multiple tumour types, and there have been cases of scleroderma significantly improving following cancer treatment.…”

“…To the Editor, Scleroderma refers to a heterogeneous group of rare autoimmune skin disorders characterised by hardening and tightening of the skin. 1,2 Multiple drugs have been implicated in the formation of scleroderma-like lesions, including chemotherapeutic agents. 3 Scleroderma can also develop in a paraneoplastic fashion.…”