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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor= {{YD}} (Reviewed by  {{YD}})
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Pathology
|MainCategory=Pathology
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|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Musculoskeletal/Rheumatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Pathology
|MainCategory=Pathology
|MainCategory=Pathology
|MainCategory=Pathology
|MainCategory=Pathology
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|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Musculoskeletal/Rheumatology
|SubCategory=Musculoskeletal/Rheumatology
|Prompt=A 62 year old woman presents to the physician's office complaining of muscle weakness. She notes that she has recently been unable to comb her hair but is able to button a shirt. She also explains that she has been unable to stand from a seated position. Review of systems shows she also complains of arthralgia involving the hand joints, wrists, and knees. Physical examination is remarkable for symmetric proximal muscle weakness. Inspection of her face shows periorbital violaceous erythema with edematous eyelids. Further inspection of her dorsal interphalangeal and metacarophalangeal joints of her hands also show violaceous flat-topped papules and plaques. Her lab work-up shows elevated levels of creatine kinase. EMG is performed; it shows patterns consistent with muscular impairment. What is the most likely pathological feature of the patient's condition?
|Prompt=A 62-year-old woman presents to the physician's office with complaints of of muscle weakness for the past 3 weeks. She states that she has recently been unable to comb her hair or stand from a seated position. When asked, she says that she has no difficulty buttoning shirts. Review of systems is remarkable for arthralgia that involves the hand joints, wrists, and knees. Inspection of the patient's face shows periorbital violaceous erythema with edematous eyelids. Physical examination is remarkable for symmetric proximal muscle weakness. Further evaluation of her dorsal interphalangeal and metacarpophalangeal joints reveals violaceous flat-topped papules and plaques. Lab work-up is remarkable for elevated concentration of creatine kinase. What is the histopathological feature of this patient's condition?
|Explanation=The patient is presenting with symptoms and signs consistent with dermatomyositis.  Dermatomyositis is an inflammatory myopathy chaacterized by symmetric proximal weakness with truncal weakness. Pain may or may not be present in dermatomyositis. Physical exmimnation typically shows Gottron papules, defined as violaceous flat-topped papules and plaques in the dorsal aspect of the hand, and heliotrope rash, defined as periorbital violaceous erythema with periorbital edema. The latter is specific for dermatomyositis. Work-up shows elevation of muscle biomarkers, such as creatine kinase. Pathologically, dermatomyositis is considered a perimysial inflammation with lymphocytic infiltration generally confined to the septa.
|Explanation=Dermatomyositis is an inflammatory myopathy characterized by symmetric proximal and truncal weakness with or without associated pain. Physical examination is typically remarkable for Gottron papules (violaceous flat-topped papules and plaques in the dorsal aspect of the hand) and characteristic heliotrope rash (periorbital violaceous erythema with periorbital edema). Work-up often demonstrates elevation of muscle biomarkers, such as creatine kinase. Pathologically, dermatomyositis is considered a perimysial inflammation with lymphocytic infiltration that is generally confined to the septa. Dermatomyositis has a bi-modal peak incidence. In adult patients > 50 years of age, the diagnosis of dermatomyositis prompts the investigation of a malignancy due to frequent association. This is not true, however, for those diagnosed at young age.
 
Dermatomyositis has a bimodal peak incidence. In adult patients > 50 years of age, the diagnosis of dermatomyositis prompts the investigation of a malignancy due to frequent association. This is not true, however, for those diagnosed at young age.
 
Educational Objective:
Dermatomyositis is characterized by perimysial inflammation.
 
References:
Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin N Am. 2011; 37:143-158.


Dalakas M. Polymyositis, dermatomyositis, and inclusion-body myositis. N Engl J Med. 1991; 325:1487-1498.
|AnswerA=Endomysial inflammation
|AnswerA=Endomysial inflammation
|AnswerAExp=Endomysial inflammation is characteristic of polymyositis
|AnswerAExp=Endomysial inflammation is characteristic of polymyositis.
|AnswerB=Perimysial inflammation
|AnswerB=Perimysial inflammation
|AnswerBExp=Perimysial inflammation is characteristic of dermatomyositis.
|AnswerBExp=Perimysial inflammation is characteristic of dermatomyositis.
|AnswerC=Non-caseating granulomas
|AnswerC=Non-caseating granulomas
|AnswerCExp=Non-caseating granulomas are commonly seen in sarcoidosis.
|AnswerCExp=Non-caseating granulomas are commonly observed in sarcoidosis.
|AnswerD=Precipitation of monosodium urate crystals
|AnswerD=Precipitation of monosodium urate crystals
|AnswerDExp=Precipitation of monosodium urate crystals is characteristic of gout.
|AnswerDExp=Precipitation of monosodium urate crystals is characteristic of gout.
|AnswerE=Autoimmune inflammatory destruction of synovial joints
|AnswerE=Autoimmune inflammatory destruction of synovial joints
|AnswerEExp=Rheumatoid arthritis (RA) is characterized by the autoimmune inflammatory destruction of synovial joints.
|AnswerEExp=Rheumatoid arthritis (RA) is characterized by the autoimmune inflammatory destruction of synovial joints.
|EducationalObjectives=Dermatomyositis is characterized by perimysial inflammation.
|References=Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin N Am. 2011; 37:143-158.<br
Dalakas M. Polymyositis, dermatomyositis, and inclusion-body myositis. N Engl J Med. 1991; 325:1487-1498.<br>
First Aid 2014 page 429.
|RightAnswer=B
|RightAnswer=B
|WBRKeyword=polymyositis, dermatomyositis, myopathy, inflammatory, proximal, muscle, weakness, perimysial, inflammation, endomysial, myopathies, gottron, papules, gottron's, heliotrope, rash
|WBRKeyword=Dermatomyositis, Myopathy, Inflammatory, Proximal muscle weakness, Perimysial inflammation, Endomysial inflammation, Gottron papules, Heliotrope rash
|Approved=No
|Approved=Yes
}}
}}

Latest revision as of 01:34, 28 October 2020
Author [[PageAuthor::Yazan Daaboul, M.D. (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Musculoskeletal/Rheumatology
Prompt [[Prompt::A 62-year-old woman presents to the physician's office with complaints of of muscle weakness for the past 3 weeks. She states that she has recently been unable to comb her hair or stand from a seated position. When asked, she says that she has no difficulty buttoning shirts. Review of systems is remarkable for arthralgia that involves the hand joints, wrists, and knees. Inspection of the patient's face shows periorbital violaceous erythema with edematous eyelids. Physical examination is remarkable for symmetric proximal muscle weakness. Further evaluation of her dorsal interphalangeal and metacarpophalangeal joints reveals violaceous flat-topped papules and plaques. Lab work-up is remarkable for elevated concentration of creatine kinase. What is the histopathological feature of this patient's condition?]]
Answer A AnswerA::Endomysial inflammation
Answer A Explanation AnswerAExp::Endomysial inflammation is characteristic of polymyositis.
Answer B AnswerB::Perimysial inflammation
Answer B Explanation AnswerBExp::Perimysial inflammation is characteristic of dermatomyositis.
Answer C AnswerC::Non-caseating granulomas
Answer C Explanation AnswerCExp::Non-caseating granulomas are commonly observed in sarcoidosis.
Answer D AnswerD::Precipitation of monosodium urate crystals
Answer D Explanation AnswerDExp::Precipitation of monosodium urate crystals is characteristic of gout.
Answer E AnswerE::Autoimmune inflammatory destruction of synovial joints
Answer E Explanation AnswerEExp::Rheumatoid arthritis (RA) is characterized by the autoimmune inflammatory destruction of synovial joints.
Right Answer RightAnswer::B
Explanation [[Explanation::Dermatomyositis is an inflammatory myopathy characterized by symmetric proximal and truncal weakness with or without associated pain. Physical examination is typically remarkable for Gottron papules (violaceous flat-topped papules and plaques in the dorsal aspect of the hand) and characteristic heliotrope rash (periorbital violaceous erythema with periorbital edema). Work-up often demonstrates elevation of muscle biomarkers, such as creatine kinase. Pathologically, dermatomyositis is considered a perimysial inflammation with lymphocytic infiltration that is generally confined to the septa. Dermatomyositis has a bi-modal peak incidence. In adult patients > 50 years of age, the diagnosis of dermatomyositis prompts the investigation of a malignancy due to frequent association. This is not true, however, for those diagnosed at young age.

Educational Objective: Dermatomyositis is characterized by perimysial inflammation.
References: Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin N Am. 2011; 37:143-158.<br Dalakas M. Polymyositis, dermatomyositis, and inclusion-body myositis. N Engl J Med. 1991; 325:1487-1498.
First Aid 2014 page 429.]]

Approved Approved::Yes
Keyword WBRKeyword::Dermatomyositis, WBRKeyword::Myopathy, WBRKeyword::Inflammatory, WBRKeyword::Proximal muscle weakness, WBRKeyword::Perimysial inflammation, WBRKeyword::Endomysial inflammation, WBRKeyword::Gottron papules, WBRKeyword::Heliotrope rash
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