Refractory cutaneous ulcers in anti-MDA5 dermatomyositis were not associated with rapidly progressive interstitial lung disease, and responded to tadalafil

Abstract

Objectives

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.