Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Mar;9(5):436.
doi: 10.21037/atm-20-5252.

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Rochelle L Castillo  1 , Alisa N Femia  2
Affiliations
Review

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Rochelle L Castillo et al. Ann Transl Med. 2021 Mar.

Abstract

Dermatomyositis (DM) is a strikingly heterogenous disease characterized by a broad and ever-evolving spectrum of cutaneous manifestations that transcend the classic "hallmarks" defined by Peter and Bohan in 1975. Despite the increasing preponderance and ubiquity of autoantibody, radiologic, and electrophysiologic testing, the diagnosis of DM still hinges largely on prompt detection of cutaneous manifestations of this condition. While pathognomonic cutaneous features of DM are more readily recognizable, many patients present with subtle and/or atypical skin manifestations, and diagnosis of DM may require clinician identification of these cutaneous clues. In this review, we highlight several of the lesser-known skin manifestations of DM, specifically, panniculitis, diffuse subcutaneous edema, erythroderma, calcinosis, ulceration, flagellate erythema, Wong-type DM, gingival telangiectasias, and the ovoid palatal patch. We describe the clinical and histopathologic presentation of these cutaneous findings. While manifesting less frequently than the heliotrope rash, Gottron's papules, and Gottron's sign, these cutaneous clues are equally important for clinicians to recognize in order to facilitate timely diagnosis and early intervention.

Keywords: Skin; cutaneous; dermatomyositis (DM); myositis.

PubMed Disclaimer

Conflict of interest statement

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/atm-20-5252). The series “Rheumatologic Skin Disease” was commissioned by the editorial office without any funding or sponsorship. ANF reports personal fees from Octagon Therapeutics, outside the submitted work. The authors have no other conflicts of interest to declare.

Figures

Figure 1
Figure 1
Flagellate erythema. Streak-like erythematous eruption on the arms and mid- to lower back of a patient with DM. DM, dermatomyositis.
Figure 2
Figure 2
Wong-type dermatomyositis. Pinkish, hyperkeratotic, follicular papules with “islands of sparing” in the lumbosacral area.
Figure 3
Figure 3
Ovoid palatal patch. Well-defined, ovoid, non-ulcerative erythematous patch on the posterior hard palate.
See this image and copyright information in PMC

References

    1. Bohan A, Peter JB. Polymyositis and Dermatomyositis. N Engl J Med 1975;292:344-7. 10.1056/NEJM197502132920706 - DOI - PubMed
    1. Aggarwal R, Rider LG, Ruperto N, et al. 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organi-sation Collaborative Initiative. Ann Rheum Dis 2017;76:792-801. 10.1136/annrheumdis-2017-211400 - DOI - PMC - PubMed
    1. Sontheimer RD. Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis siné myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002;46:626-36. 10.1067/mjd.2002.120621 - DOI - PubMed
    1. Gerami P, Schope JM, McDonald L, et al. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol 2006;54:597-613. 10.1016/j.jaad.2005.10.041 - DOI - PubMed
    1. Femia AN, Vleugels RA, Callen JP. Cutaneous Dermatomyositis: An Updated Review of Treatment Options and Internal Associations. Am J Clin Dermatol 2013;14:291-313. 10.1007/s40257-013-0028-6 - DOI - PubMed

LinkOut - more resources