EVIDENCE-BASED MEDICINE - CASE SCENARIOS

Year : 2018  |  Volume : 16  |  Issue : 1  |  Page : 20-21

Not just skin deep

Manna Sera Jacob, Ajay Kumar Mishra
Department of General Medicine, Christian Medical College, Vellore, Tamil Nadu, India

Correspondence Address:
Dr. Manna Sera Jacob
Department of General Medicine, Christian Medical College, Vellore, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/cmi.cmi_5_18

Case Scenario

1. What condition are the above skin findings classical of? What are the other common associated cutaneous findings in the same?
2. What is dermatomyositis and how is it diagnosed?
3. What are the different clinical presentations of the above condition?
4. What further investigation should be done once this condition is diagnosed?
5. What is the treatment?

1. The patient has heliotrope rash (erythematous-to-violaceous lesions in the upper eyelids associated with lid edema) [Figure 1], shawl sign (erythematous lesions over the upper back) [Figure 2], Gottron's sign (an area of dull violaceous erythema with scaling over the elbow). These are classical cutaneous manifestations of dermatomyositis [DM]. Other skin findings which can be seen in dermatomyositis but were not present in our patient are Gottron's sign (red–purple keratotic, atrophic erythema, or macules on the extensor surface of finger joints), V sign (erythema over the anterior chest and neck), and calcinosis cutis.^[1]
2. It is an idiopathic inflammatory muscle disease (myopathy) which is characterized by predominant proximal muscle weakness (arms, thighs) along with very high levels of muscle enzyme creatinine phosphokinase (CPK) and classical cutaneous manifestations as stated above. While the presence of a typical clinical feature in the presence of extremely high muscle CPK is highly suggestive of the diagnosis, Bohan and Peter criteria as established in 1975 are still relevant and extensively used. The five criteria's under the same are (1) symmetric proximal muscle weakness, (2) typical cutaneous features of DM, (3) elevated serum muscle enzymes, (4) myopathic changes in electromyography (EMG), (5) typical muscle biopsy abnormality showing perimysial perivascular inflammation of CD4 + T-cells. The presence of cutaneous lesions along with 3 other criteria is required to establish the definite diagnosis. Patients without any alternate diagnosis could be diagnosed as possible and probable DM. This patients' EMG and biopsy revealed the features of DM.^[2]
1. Classical dermatomyositis: classical skin lesions with proximal myopathy (proven by EMG, raised creatine kinase, and muscle biopsy)
2. Hypomyopathic dermatomyositis: classical skin lesions with no clinical myopathy; but EMG and biopsy shows evidence of myopathy (as seen in this individual)
3. Amyopathic dermatomyositis: classical skin lesions with no clinical or laboratory evidence of myopathy.

Figure 1: Heliotrope rash. Click here to view

Figure 2: Shawl sign. Click here to view



Hypomyopathic DM and amyopathic DM are together called as clinically amyopathic dermatomyositis


4. Significant minority of patients have paraneoplastic dermatomyositis which mandates a detailed history and physical examination of lymph nodes, breast, thyroid, rectal, and vaginal examination. Routine tests including complete blood count, liver function test, urine analysis, plain X-ray chest, and stool for occult blood should be done for all. Age-specific screening for adenocarcinomas of the cervix, ovaries, pancreas, bladder, stomach, and lung should be done. In our patient, evidence of T-cell lymphoma was identified ^[3]
5. This individual was treated with intravenous methylprednisolone 1000 mg/day for three days and was continued with steroids (1 mg/kg Prednisolone). Patients who are severely ill are managed with intravenous methylprednisolone. Our patient developed features of disseminated intravascular coagulation and hence was treated accordingly. Azathioprine, methotrexate, and mycophenolate mofetil are used routinely as glucocorticoid-sparing agent.

References

1.	Jose N, Mishra A, Kumar V, David T. Erythrodermic Dermatomyositis. JCR 2015;5:246-7.
2.	Narasimhaiah DA, Premkumar JA, Moses V, Chacko G. Carcinoma of gall bladder presenting as dermatomyositis. Ann Indian Acad Neurol 2011;14:44-6.   [PUBMED]  [Full text]
3.	Miller L. Malignancy in dermatomyositis and polymyositis. UpToDate. Available at https://www.uptodate.com/contents/malignancy-in-dermatomyositis-and-polymyositis. [Last accessed on 2018 Mar 01].