Refractory cutaneous ulcers in anti-MDA5 dermatomyositis were not associated with rapidly progressive interstitial lung disease, and responded to tadalafil.
Objective: The purpose of this study was to analyze the characteristics of an ulcerative rash in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody and to assess the efficacy of tadalafil in the management of refractory cutaneous ulcers.
Methods: Medical records of DM patients with anti-MDA5 antibody were reviewed. Skin rash data, radiologic characteristic and serum markers were collected. Patients with refractory cutaneous ulcers treated with tadalafil were reviewed retrospectively.
Results: Eighty-two consecutive cases with anti-MDA5-antibody positive DM (anti-MDA5 DM) were identified, including 62 (75.6 %) female patients. Approximately 95.0 % of the patients had interstitial lung disease (ILD) and 45.1 % had rapidly progressive ILD (RP-ILD). All patients (100 %) had typical skin rashes, such as Gottron's sign (87.8 %), heliotrope sign (69.5 %), and the holster sign (24.4 %). Twenty-six patients (31.7 %) had cutaneous ulcers. Univariate analysis showed that ulcer patients were prone to Gottron's sign (p = 0.026), perlungual erythematosus (p = 0.007), and arthritis (p = 0.031), while shortness of breath after exercise (p = 0.027) and RP-ILD (p = 0.024) are more likely to appear in patients with non-skin ulcers. Among the anti-MDA5 DM patients with skin ulcers, 10 (38.5 %) were refractory cutaneous ulcers, including five male patients. After tadalafil was added, eight (80 %) patients showed improvement in the physician global assessment (PGA), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score and the patient's pain score using the visual analog score (p-VAS), within one to two months. In addition, the refractory cutaneous ulcers of five patients completely resolved after treatment.
Conclusions: This study found that ulcerative rash in anti-MDA5 DM may not be associated with RP-ILD. The results also suggest that tadalafil may be a useful therapy for refractory cutaneous ulcers.