Painful ANA-Positive Scleroderma-Like Disease With Acral Ulcerations: A Case of Chronic Gangrenous Ergotism

Abstract

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.

Transient Anti PM-Scl75 Antibody Positive Acral Ischemia Syndrome in a 15 Years-Old Girl

“…A mild acral ischemia could be interpreted as incomplete Raynaud phenomenon [10,11,12,13,14,20]. Acral ischemia was found in COVID-19 [15,16,9], chronic gangrenous ergotism [17], t-cell lymphoma [18], essential thrombocythemia [3], thrombotic microangiopathy [12], Hodgkin lymphoma [7], application of immune checkpoint inhibitors [19], nivolumab [2] and epinephrine [20] and closed injury [6]. It remains a rare acral vascular event in children and teenagers like in our case.…”

Acrocyanosis – A Symptom with Many Facettes

“…Ergot alkaloids used for the treatment of headaches and migraine can cause acrocyanosis and hand or leg ulcers [ 30 ]. Particular attention has to be paid in case of antiretroviral therapy since alkaloid action can be potentiated due to an inhibition of cytochrome P450 [ 31 ].…”

Update on cutaneous calciphylaxis

“…[173031] Winiwarter–Buerger disease and ergotism can cause painful digital ulcerations. [3132] Calciphylaxis may clinically resemble pyoderma gangrenosum, but histopathology is different. [33]…”