Clinical and Histopathological Features of Adult Patients With Dermatomyositis and MDA-5 Autoantibody Seropositivity Status Clinical and Experimental Dermatology

TAKE-HOME MESSAGE

• The authors evaluated the results of the commercially available melanoma differentiation-associated (MDA)-5 autoantibodies in 73 patients with a diagnosis of dermatomyositis (DM). MDA-5 autoantibodies were seen in 45% of patients and these patients demonstrated an increased prevalence of Raynaud phenomenon (RP), cutaneous ulcerations, mechanic hands, palmar papules, oral ulcers, and alopecia. Systemic findings such as joint involvement, pulmonary involvement, and dysphagia were more commonly seen in DM patients who were positive for MDA-5. On histopathology, vasculopathy was a significant finding seen only in DM patients positive for MDA-5, while spongiosis was only seen in cases of DM that were negative for MDA-5.

• This study confirms some of the previously associated findings with MDA-5 positivity in DM patients, while also adding RP and dysphagia as new associations noted.

–  Alex Noble, MD

Abstract 

BACKGROUND

Although melanoma differentiation associated (MDA)-5 autoantibodies have been widely explored in dermatomyositis (DM), most studies have relied on MDA-5 autoantibody testing performed in research settings, rather than the now-available commercial laboratory tests.

AIM

To characterize the clinical and histopathological data in patients with DM and circulating MDA-5 autoantibodies, as defined by commercially available testing.

METHODS

This was a retrospective review of patients with DM who underwent MDA-5 antibody testing. All available skin biopsy slides were reviewed.

RESULTS

Cutaneous features more prevalent in MDA-5-positive DM included Raynaud phenomenon (RP) (P < 0.001), cutaneous ulcerations (P = 0.01), mechanic hands (P < 0.02), palmar papules (P < 0.01), oral ulcers (P = 0.024) and alopecia (P = 0.03). Joint and pulmonary involvement were more common in patients with MDA-5-positive DM (both P < 0.001) as was dysphagia (P < 0.01). Myopathy (P = 0.4) and malignancy (P = 0.34) were not statistically different between the cohorts. Vasculopathy was more common in MDA-5-positive DM (P < 0.01), while spongiosis was less common (P < 0.02).

CONCLUSION

This study not only confirms some known associations between disease manifestations and MDA-5 autoantibody status, as determined by commercially available tests, but also identifies new associations, including RP and dysphagia.

Clinical and Experimental Dermatology

Clinical and histopathological features of adult patients with dermatomyositis and melanoma differentiation associated-5 autoantibody seropositivity status, as determined by commercially available testing: a retrospective, single-institution comparative cohort study

Clin Exp Dermatol 2021 Aug 03;[EPub Ahead of Print], H Shakshouk, MA Deschaine, DA Wetter, LA Drage, FC Ernste, JS Lehman 

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Benjamin Hidalgo-Matlock
Skin Care Physicians of Costa Rica

Clinica Victoria en San Pedro: 4000-1054
Momentum Escazu: 2101-9574

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