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Case 349

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50 year-old male patient, refers proximal-member weakness and skin rash on arms and proximal interphalangeal and metacarpophalangeal articulations’ extensor face (Images 1 and 2). Denies photosensitivity. Antinuclear auto-antibody (FAN) positive on homogeneous (1:160) and speckled (1:640) patterns, and creatine-phosphokinase of 1935 U/L. Lesion biopsy revealed chronic superficial inflammatory infiltrate.

Considering the clinical data and the images, the most likely diagnosis is:

a) Dermatomyositis

25%

b) Systemic lupus erythematosus

25%

c) Scleroderma

25%

d) Henoch-Schonlein Purpura

25%
   

Image analysis

Image 1: Photography of right hand’s dorsum. Presence of irregular erythematous patches, barely defined on the proximal interphalangeal metacarpophalangeal (2nd and 4th), as well as the distal interphalangeal (3rd and 4th) articulations’ extensor face, compatible with Gottron’s papules. 

 

Image 2: Photography of left hand’s dorsum. Presence of irregular erythematous patches, barely defined on the proximal interphalangeal metacarpophalangeal (2nd and 3rd), as well as the distal interphalangeal (2nd to 4th) articulations’ extensor face, compatible with Gottron’s papules. 

Highlights

  • - Dermatomyositis is a rare rheumatologic disease with a bimodal peak of incidence (5-15 and 40-60 years) and more frequently among females (2:1);

  • - Gottron’s papules are erythematous papules or patches on the extensor surface of metacarpophalangeal and interphalangeal articulations, occurring symmetrically and being pathognomonic for dermatomyositis; 

  • - Diagnosis relies on the combination of the dermatological findings with muscular injury evaluated by the clinical condition, serum enzymes, muscle biopsy and/or electromyography;

  • - Antinuclear auto-antibody is usually positive in those patients, although with low specificity; 

  • - Treatment is based on inflammatory control with glucocorticoid, associated or not to immunosuppressors such as Azathioprine and Methotrexate. 

References

- Mainetti C, Terziroli Beretta-Piccoli B, Selmi C. Cutaneous Manifestations of Dermatomyositis: a Comprehensive Review. Clin Rev Allergy Immunol. 2017;53(3):337–56. 

- Muro Y, Sugiura K, Akiyama M. Cutaneous Manifestations in Dermatomyositis: Key Clinical and Serological Features — a Comprehensive Review. Clin Rev Allergy Immunol [Internet]. 2016;51(3):293–302. Available from: http://dx.doi.org/10.1007/s12016-015-8496-5.

- Miller ML, Vleugels RA. Clinical manifestations of dermatomyositis and polymyositis in adults[Internet]. UpToDate. 2019 [cited 2019 May 4]. Available from: https://www.uptodate.com/contents/clinical-manifestations-of-dermatomyositis-and-polymyositis-in-adults?search=dermatomiosite&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1. 

Author

Bruno Campos Santos, 6th year medical student at Universidade Federal de Minas Gerais.

Email: bruno_campos[at]outlook.com

Supervisor

Rejane Pinheiro Damasceno, MD, Rheumatologist and Preceptor at the Instituto de Previdência dos Servidores do Estado de Minas Gerais.

E-mail: rejanepinheirod[at]gmail.com 

Reviewers

Gustavo Monteiro, Letícia de Melo Elias, André Luiz Marzano, Felipe Eduardo Lopes and Viviane Santuari Parisotto Marino, MD, Ph.D.

Translated by

Bruno Campos Santos, 6th year medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]outlook.com

Test question

(Medical Residence 2009 – UNB) 

A 48 year-old patient referred progressive and symmetric loss of proximal muscle strength over the past six months. Refers loss of 8 kg on the last year. At examination, shows signs of arthritis on the small and big articulations, besides heliotrope and malar rash (including the nasolabial folds) and Gottron’s papules. 

From the clinical data, the most likely diagnosis for this patient is:

a) Centronuclear myopathy

25%

b) Nemaline myopathy

25%

c) Myasthenia gravis

25%

d) Dermatomyositis

25%

e) Eaton-Lambert syndrome

25%
   

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