Case reportLimb edema and anasarca associated with severe dermatomyositis: Report of four cases
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)
- et al.
Polymyositis and dermatomyositis
Lancet
(2003) - et al.
A rare complication of generalized edema in juvenile dermatomyositis: a report of one case
Brain Dev
(2004) - et al.
Juvenile dermatomyositis presenting with anasarca: a possible indicator of severe disease activity
J Pediatr
(2001) - et al.
Acute oedematous dermatomyositis
Ann Rheum Dis
(2000) - et al.
Acute inflammatory myopathy with severe subcutaneous edema, a new variant? Report of two cases and review of the literature
Rheumatol Int
(2001) - et al.
A case of edematous dermatomyositis
J Rheumatol
(2003) - et al.
Acute dermatomyositis with subcutaneous generalized edema
Clin Rheumatol
(2006) - et al.
Severe subcutaneous generalized edema in a patient with dermatomyositis
Mod Rheumatol
(2007)
Cited by (16)
Dermatomyositis associated with hyponatremia and anasarca
2021, JAAD Case ReportsCitation Excerpt :Spongiotic dermatitis can be seen in DM, and increased MxA distinguishes DM from the spongiosis seen in eczema.3 Both adult- and juvenile-onset DM with anasarca as well as peripheral limb edema have been reported in the literature,1 although rarely so, and only included in a handful of case reports. DM is also reported to be associated with hyponatremia in rare cases.2
Severe edematous dermatomyositis
2016, Annales de Dermatologie et de VenereologieJuvenile Dermatomyositis
2015, Textbook of Pediatric RheumatologyDermatomyositis presenting with severe subcutaneous edema: Five additional cases and review of the literature
2014, Seminars in Arthritis and RheumatismCitation Excerpt :Generalized and/or limb edema in IIM in either DM or PM is very rare, although Wagner included this sign in the original description of the PM in 1887 [6]. Regarding DM, only 14 cases have been reported in adults to date [7–16]. Diagnosis relies on the clinical, typical electromyography pattern, elevated muscle enzymes, and muscle biopsy according to the criteria by Bohan and Peter [17] and Dalakas and Hohlfeld [4].
Severe dermatomyositis with pronounced generalized subcutaneous edema and dysphagia: A rare manifestation of a highly active disease
2014, Dermatologica SinicaCitation Excerpt :All patients had improved swelling only after aggressive immunotherapy, a finding which strongly suggested that the edema had a close relation to the pathophysiology of DM.9 The clinical features of the 14 reported cases of DM associated with generalized edema1–10 and the present case are summarized in Table 1. Altogether, there are 10 females and 5 males, with ages ranging from 23 years to 78 years (mean = 49.7 years), a finding consistent with the female predominance in adult patients with DM.
Edema and dermatomyositis. Migratory edema and edematous and vesiculobullous dermatomyositis overlap
2018, Reumatologia Clinica