Wong Type Dermatomyositis Complicated by Interstitial Lung Disease

Abstract

INTRODUCTION: Wong type dermatomyositis is a rare subset of dermatomyositis which presents with atypical cutaneous findings such as hyperkeratotic papules and palmoplantar keratoderma. To the best of our knowledge, we present the first case of Wong type dermatomyositis complicated by interstitial lung disease (ILD). CASE PRESENTATION: A 39-year-old white male presented to rheumatology clinic with a rash, decline in physical endurance, and a dry cough. Physical examination revealed a scaly rash of the bilateral palms, Gottron's papules, Holster sign of the bilateral anterior thighs, and keratoderma of the soles of the feet (Figure 1). Laboratory analysis showed elevated creatine kinase and aldolase with negative myositis antibodies. CT chest exhibited subpleural bibasilar predominance of ground glass opacities and nodules (Figure 2). Contrast enhanced MRI of the lower extremities demonstrated enhancement of the fascia consistent with inflammatory myopathy, which was confirmed by the presence of perivascular inflammatory infiltrate on muscle biopsy. Bronchoalveolar lavage showed a cell differential of 43% macrophages, 33% lymphocytes, 12% neutrophils, and 12% eosinophils with negative cultures. Mild restriction was discovered on pulmonary function testing (PFT). The patient started on pulse dose corticosteroids and hydroxychloroquine. He responded well to therapy with near resolution of cutaneous findings and significant improvement on subsequent PFT. Extensive evaluation did not show evidence of internal malignancy. DISCUSSION: ILD is a common complication of dermatomyositis however, it has never been described in patients with the Wong variant. Patients classically present with features of dermatomyositis and atypical cutaneous findings as described in our case. Management is based solely on case reports given the rarity of the disease. High dose corticosteroids and hydroxychloroquine is the initial treatment of choice. CONCLUSIONS: Clinicians should consider Wong type dermatomyositis in patients presenting with palmoplantar keratoderma and interstitial lung disease.

A case of Wong‐type dermatomyositis treated with rituximab and IVIG

“…There is also a case report of malignancy in a patient with Wong‐type DM developing 5 years after their diagnosis, though it is not clear whether the two were related 1 . Only one case of Wong‐type DM associated with interstitial lung disease has been reported to date 3 …”

Wong‐type dermatomyositis with pulmonary involvement in a teenager: case report

“…There is one published case of a 39-year-old patient with Wong-type DM and lung involvement, together with groundglass opacities on pulmonary imaging, suggestive of ILD [7]. There are no reports of this association in adolescents or children.…”

Dermatomyositis vom Wong‐Typ mit Lungenbeteiligung im Teenageralter: ein Fallbericht

“…Es existiert ein veröffentlichter Fall einer 39-jährigen Patientin mit Wong-Typ-DM und Lungenbeteiligung mit Ground-Glass-Verschattungen, die an eine interstitielle Lungenerkrankung denken lassen [7]. Bei Jugendlichen oder Kindern ist eine solche Assoziation bisher nicht beschrieben.…”