Case report
Limb edema and anasarca associated with severe dermatomyositis: Report of four cases

https://doi.org/10.1016/j.nmd.2011.03.003Get rights and content

Abstract

Dermatomyositis is an autoimmune disorder that causes proximal muscle weakness and skin changes which include generalized erythema, heliotrope rash and/or Gottron’s papules. Generalized or limb edema is an uncommon manifestation of dermatomyositis. Here, we report four cases who presented with generalized or limb edema, proximal muscle weakness, erythematous skin rash and/or dysphagia. Muscle biopsy revealed perifascicular fiber atrophy, a characteristic finding of dermatomyositis. The absence of other causes indicated that the generalized or limb edema was caused by dermatomyositis. None of our patients showed significant improvement with steroids alone, and more aggressive immunotherapy eventually resolved the edema. We concluded that generalized or limb edema may be a hallmark of a severe form of dermatomyositis and requires prompt and aggressive therapies.

Introduction

Dermatomyositis manifests with proximal muscle weakness and several characteristic skin rashes including Gottron’s sign, heliotrope periorbital rash and other skin changes such as erythroderma, periungual abnormalities, flagellate erythema on the trunk, psoriasiform changes in scalp, calcinosis cutis, a roughening and cracking of the skin of the tips and lateral aspects of the fingers (mechanic’s hands), a diffuse, flat erythematous lesion occurring over the chest and shoulders (Shawl sign) and a V-shaped distribution over the anterior neck and chest (V sign) [1]. Edema is not uncommon in eyelids and juvenile dermatomyositis had been found to be associated with periorbital and facial edema. However, anasarca or limb edema caused by juvenile and adult dermatomyositis was rare and only reported in isolated cases [2], [3], [4], [5], [6], [7], [8]. Here, we report four adult dermatomyositis cases that had generalized or limb edema which improved with aggressive immunotherapy.

Section snippets

Patients

Patient #1 was a 62-year-old male who presented with a dysphagia, proximal muscle weakness and generalized swelling for the past two months. Examination showed Gottron’s papules on the extensor surface of joints in both hands (Fig. 1A) and generalized non-pitting edema (Fig. 1B). Muscle strength was 3/5 proximally and 4+/5 distally on the Medical Research Council (MRC) scale. Sensation was intact to all modalities. Deep tendon reflexes were normal. Serum creatine kinase (CK) was 400 IU/L (normal

Discussion

Proximal muscle weakness, skin rashes, increased serum muscle enzyme, typical findings on EMG and muscle biopsy in our four cases were compatible with dermatomyositis. In addition, all patients had a generalized or limb swelling. Patients #1 and #2 had limb edema on the side of their port-a-cath placement. However, there was no clear evidence of DVT or significant venous narrowing. Although a malfunctional port-a-cath can cause focal edema, it cannot be assumed the sole cause, as replacement

Acknowledgements

The authors thank Rachel Young for critical reading of the manuscript, Sue Maccarino for her technique assistance for nerve conduction studies and Mariallan Shadle for her technique assistance for muscle biopsies.

References (16)

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