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Dermatomyositis - Nail and Distal Digit
See also in: Overview,Cellulitis DDx,External and Internal Eye
Other Resources UpToDate PubMed

Dermatomyositis - Nail and Distal Digit

See also in: Overview,Cellulitis DDx,External and Internal Eye
Contributors: Shari Lipner MD, PhD, Kyle Cheng MD, Bertrand Richert MD, Robert Baran MD, Susan Burgin MD
Other Resources UpToDate PubMed


Dermatomyositis is a multisystem autoimmune connective tissue disease that is most often characterized by a symmetric proximal extensor inflammatory myopathy, a characteristic violaceous cutaneous eruption, and pathogenic circulating autoantibodies. Dermatomyositis demonstrates a bimodal incidence, with the adult form most commonly seen in individuals aged 45-60 and the juvenile form found in children aged 10-15 years. A 2:1 female-to-male incidence ratio exists in adults.

The distinctive nail findings of dermatomyositis help distinguish it from other connective tissue disorders. Thickened, hyperkeratotic, ragged cuticles and telangiectasias of the proximal nail fold (PNF) are the characteristic nail findings. Some other rare nail findings have been reported, including complete loss of several toenails, red lunulae, and ventral pterygium. The presence of ischemic lesions might be predictive of malignancy in dermatomyositis of adulthood.

In addition to the nail findings, other cutaneous findings such as periorbital heliotrope rash, atrophic dermal papules of dermatomyositis (ADPDM, formerly called Gottron papules) (slightly atrophic, flat-topped papules over the proximal interphalangeal joints), and poikiloderma should also be present. ADPDM may involve the vicinity of the PNF. Other characteristic features may include flat erythema of the upper back and posterior neck and shoulders (shawl sign) as well as a similarly presenting macular erythema of the anterior neck and upper chest (V sign) that can worsen with ultraviolet exposure. Patients may also have poikiloderma over the lateral hip (holster sign). Muscle involvement affects proximal muscle groups in a symmetric fashion.

Dermatomyositis may be induced by medications, including hydroxyurea, penicillamine, interferon beta, and ipilimumab. Acute onset / flares of dermatomyositis have been reported in association with ingestion of IsaLean, an herbal supplement.


M33.10 – Other dermatomyositis, organ involvement unspecified

396230008 – Dermatomyositis

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Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed:07/25/2019
Last Updated:12/21/2021
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Patient Information for Dermatomyositis - Nail and Distal Digit
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Contributors: Medical staff writer


Dermatomyositis is an inflammatory disease that causes muscle weakness and skin rashes.

Who’s At Risk

Dermatomyositis is a rare disease that is more common in women than in men. Adults most often develop dermatomyositis between the ages of 45 and 60. There is also a childhood form of the disease called juvenile dermatomyositis.

Signs & Symptoms

A violet rash develops, most commonly on your face and eyelids. The rash can also appear on your knuckles, chest, knees, nails, and back. The purple, patchy rash is often the first sign of dermatomyositis.

Dermatomyositis also causes muscle weakness that tends to start in the trunk area, such as your hips, shoulders, neck, upper arms, and thighs. The weakness tends to get gradually worse.

Self-Care Guidelines

The rash is sensitive to the sun. Avoid too much exposure to the sun and wear protective clothing and SPF 45 or higher sunscreen.

For support groups, see the Myositis Association website at

When to Seek Medical Care

There is no cure for dermatomyositis. However, the sooner treatment is started, the better the results. It is important to keep in touch with your doctors during treatment.


Corticosteroids (prednisone) are effective in controlling dermatomyositis symptoms. However, they have side effects after prolonged use. Corticosteroid-sparing agents can be used to reduce the dosage and side effects of corticosteroids.

If the rash is severe, antimalarial medication can be administered.

Physical and speech therapy can help you improve your muscle strength.

Depending on your symptoms, there may be other options you will want to discuss with your health care provider. Your doctors will tailor individual treatment to you.
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Dermatomyositis - Nail and Distal Digit
See also in: Overview,Cellulitis DDx,External and Internal Eye
A medical illustration showing key findings of Dermatomyositis (Cutaneous manifestations) : Burning skin sensation, Erythema, Eyelid edema, Nail fold telangiectasia, Photosensitivity, Pruritus
Clinical image of Dermatomyositis - imageId=583874. Click to open in gallery.  caption: 'Eyelid erythema of heliotrope rash.'
Eyelid erythema of heliotrope rash.
Copyright © 2023 VisualDx®. All rights reserved.