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Clinical presentation and evaluation of dermatomyositis.

Marvi U, Chung L, Fiorentino DF - Indian J Dermatol (2012)

Bottom Line: Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis.Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy.This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

View Article: PubMed Central - PubMed

Affiliation: Division of Immunology and Rheumatology, Stanford University, USA.

ABSTRACT
Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50-70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron's papules, Gottron's sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, "mechanic's hands", palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35-40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

No MeSH data available.


Related in: MedlinePlus

(a) Gottron's papules: Violaceous, scaling papules on the skin overlying the joints and proximal nailfolds. (b) Gottron's sign: Violaceous patches overlying the knees. (c) “V neck” sign: Erythematous and hyperpigmented macules on the chest. (d) Shawl sign: Violaceous macules and patches on the upper back and shoulders. (e) Scalp disease in dermatomyositis: Deeply erythematous scaling plaques are seen diffusely on the posterior scalp
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Figure 1: (a) Gottron's papules: Violaceous, scaling papules on the skin overlying the joints and proximal nailfolds. (b) Gottron's sign: Violaceous patches overlying the knees. (c) “V neck” sign: Erythematous and hyperpigmented macules on the chest. (d) Shawl sign: Violaceous macules and patches on the upper back and shoulders. (e) Scalp disease in dermatomyositis: Deeply erythematous scaling plaques are seen diffusely on the posterior scalp

Mentions: The characteristic rash of DM can occur before, shortly after, or at the same time as muscle weakness. As mentioned above, a proportion of patients will have the characteristic cutaneous findings of DM, and never develop clinical or laboratory signs of myositis. A hallmark sign of DM are Gottron's papules [Figure 1a]. These lesions primarily consist of erythematous to violaceous papules and plaques over the extensor surfaces of the metacarpophalangeal and interphalangeal joints. These lesions may have accompanying scale, and can sometimes develop ulcerations; active lesions tend to resolve with dyspigmentation, atrophy, and scarring. Gottron's sign refers to erythematous macules and patches overlying the elbows and/or knees, and are less specific findings for DM [Figure 1b].[20] Another hallmark sign of DM is the heliotrope rash which consists of violaceous erythema of the upper eyelids often with associated edema and telangiectasia. It should be noted that in darker skin types, this erythema may be subtle and overlooked. Erythematous patches and/or plaques in other characteristic sun-exposed or nonsun-exposed areas may also be seen in DM. A confluent erythema in the malar distribution involving the cheeks and extending over the nasal bridge may be seen, and can often involve the nasolabial folds. More extensive involvement can be seen in other areas including the forehead, lateral face, and ears. Confluent macular erythema over the lower anterior neck and upper anterior chest can also be seen (the so-called “V” sign) [Figure 1c]. A shawl sign refers to erythema over the upper back, posterior neck, and shoulders, sometimes with extension to the lateral arms [Figure 1d]. Nonsun-exposed areas can also be involved, especially the scalp [Figure 1e], lower back, and lateral thighs (Holster sign).[20] Erythroderma with extensive areas of confluent erythema involving over 50% of the body surface area is rare. Also rarely, patients can present with a more ichthyotic variant which appears as severely dry and cracked skin.


Clinical presentation and evaluation of dermatomyositis.

Marvi U, Chung L, Fiorentino DF - Indian J Dermatol (2012)

(a) Gottron's papules: Violaceous, scaling papules on the skin overlying the joints and proximal nailfolds. (b) Gottron's sign: Violaceous patches overlying the knees. (c) “V neck” sign: Erythematous and hyperpigmented macules on the chest. (d) Shawl sign: Violaceous macules and patches on the upper back and shoulders. (e) Scalp disease in dermatomyositis: Deeply erythematous scaling plaques are seen diffusely on the posterior scalp
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3482801&req=5

Figure 1: (a) Gottron's papules: Violaceous, scaling papules on the skin overlying the joints and proximal nailfolds. (b) Gottron's sign: Violaceous patches overlying the knees. (c) “V neck” sign: Erythematous and hyperpigmented macules on the chest. (d) Shawl sign: Violaceous macules and patches on the upper back and shoulders. (e) Scalp disease in dermatomyositis: Deeply erythematous scaling plaques are seen diffusely on the posterior scalp
Mentions: The characteristic rash of DM can occur before, shortly after, or at the same time as muscle weakness. As mentioned above, a proportion of patients will have the characteristic cutaneous findings of DM, and never develop clinical or laboratory signs of myositis. A hallmark sign of DM are Gottron's papules [Figure 1a]. These lesions primarily consist of erythematous to violaceous papules and plaques over the extensor surfaces of the metacarpophalangeal and interphalangeal joints. These lesions may have accompanying scale, and can sometimes develop ulcerations; active lesions tend to resolve with dyspigmentation, atrophy, and scarring. Gottron's sign refers to erythematous macules and patches overlying the elbows and/or knees, and are less specific findings for DM [Figure 1b].[20] Another hallmark sign of DM is the heliotrope rash which consists of violaceous erythema of the upper eyelids often with associated edema and telangiectasia. It should be noted that in darker skin types, this erythema may be subtle and overlooked. Erythematous patches and/or plaques in other characteristic sun-exposed or nonsun-exposed areas may also be seen in DM. A confluent erythema in the malar distribution involving the cheeks and extending over the nasal bridge may be seen, and can often involve the nasolabial folds. More extensive involvement can be seen in other areas including the forehead, lateral face, and ears. Confluent macular erythema over the lower anterior neck and upper anterior chest can also be seen (the so-called “V” sign) [Figure 1c]. A shawl sign refers to erythema over the upper back, posterior neck, and shoulders, sometimes with extension to the lateral arms [Figure 1d]. Nonsun-exposed areas can also be involved, especially the scalp [Figure 1e], lower back, and lateral thighs (Holster sign).[20] Erythroderma with extensive areas of confluent erythema involving over 50% of the body surface area is rare. Also rarely, patients can present with a more ichthyotic variant which appears as severely dry and cracked skin.

Bottom Line: Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis.Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy.This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

View Article: PubMed Central - PubMed

Affiliation: Division of Immunology and Rheumatology, Stanford University, USA.

ABSTRACT
Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50-70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron's papules, Gottron's sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, "mechanic's hands", palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35-40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.

No MeSH data available.


Related in: MedlinePlus