|Year : 2015 | Volume
| Issue : 2 | Page : 32-36
Dermatomyositis in a patient with nasopharyngeal carcinoma
Ogochukwu I Ezejiofor1, Angela G Ozor2, Chibuzo I Okpala2, Nkechi A Enechukwu2, Henry M Nwankwo2
1 Department of Medicine, Nnamdi Azikiwe University, Awka, Nigeria
2 Department of Medicine, Dermatology Unit, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria
|Date of Web Publication||19-May-2016|
Ogochukwu I Ezejiofor
Department of Medicine, Nnamdi Azikiwe University, Nnewi Campus, Nnewi, Anambra State
Source of Support: None, Conflict of Interest: None
Dermatomyositis (DM) is a rare inflammatory autoimmune disease of the muscle and skin characterized primarily by proximal myopathy and various cutaneous signs, with multi-systemic manifestations. The hallmark cutaneous changes are confluent macular violaceous erythema and edema in characteristic anatomic locations including eyelids (heliotrope rash), posterior neck, shoulder girdle and upper arm (shawl sign), extensor elbow, knee, and knuckles (Gottron's sign).Proximal muscle weakness is also a common manifestation of the disorder.Dermatomyositis can occur as manifestation of internal malignancy.This case report is that of a 53 year old man with Dermatomyositis and Nasopharyngeal carcinoma.
Keywords: Dermatomyositis, internal malignancies, nasopharyngeal carcinoma
|How to cite this article:|
Ezejiofor OI, Ozor AG, Okpala CI, Enechukwu NA, Nwankwo HM. Dermatomyositis in a patient with nasopharyngeal carcinoma. Niger J Surg Sci 2015;25:32-6
|How to cite this URL:|
Ezejiofor OI, Ozor AG, Okpala CI, Enechukwu NA, Nwankwo HM. Dermatomyositis in a patient with nasopharyngeal carcinoma. Niger J Surg Sci [serial online] 2015 [cited 2019 Dec 13];25:32-6. Available from: http://www.njssjournal.org/text.asp?2015/25/2/32/182677
| Introduction|| |
Dermatomyositis (DM) is a rare inflammatory autoimmune disease of the muscle and skin characterized primarily by proximal myopathy and various cutaneous signs, with multi-systemic manifestations.  The hallmark cutaneous changes are confluent macular violaceous erythema and edema in characteristic anatomic locations including eyelids (heliotrope rash), posterior neck, shoulder girdle and upper arm (shawl sign), extensor elbow, knee, and knuckles (Gottron's sign).
Other lesions include flat-topped, polygonal violaceous papules on the knuckles (Gottron's papule), periungual telengiectasia, hyperkeratotic, fissured and hyperpigmented palms, and sides of the fingers (mechanic's hand). Poikiloderma may occasionally be seen.
Proximal muscles weakness occurs and manifest as difficulty with raising arms above the head and climbing the staircase. DM is pathologically characterized by microangiopathy affecting the skin and muscle; activation and deposition of compliment cause lysis of endomysial capillaries and muscle ischemia.  However, the exact mechanism of cutaneous damage is not well-understood.
Glucocorticoids remain the mainstay of treatment although other immunosuppressive agents have been used. Adult onset DM is often associated with internal malignancies and reports are few from this part of the world.
| Case report|| |
A 53-year-old man who presented to our Dermatology Clinic with a 6 weeks history of painful and occasionally itchy erythematous to violaceous, edematous rashes which started from the scalp and then spread to involve the neck, shoulder girdle, upper back, upper chest, abdomen, and extensor surface of the forearm. The pain was burning and worse on exposure to sunlight. He also complained of difficulty with lifting the upper limbs above the head and climbing staircase.
There was an associated left-sided neck swelling that started insidiously and progressively increased in size, accompanied with pain and difficulty with swallowing, cough with scanty sputum production, hemoptysis, and weight loss. He also complained of disabling joint pains involving the wrist, elbow, and knee joints.
Physical examination revealed erythematous to violaceous and oedematous eyelids (heliotrope rash) [Figure 1], confluent macular violaceous erythema of the upper chest [Figure 2], abdomen and extensor forearm [Figure 3], shoulder girdles, neck and arms (shawl sign) [Figure 4], erythematous to violaceous polygonal flat topped papules on the knuckles of the fingers (Gottron's papule) [Figure 5] and periungual nail fold infarcts [Figure 6].
Examination of the neck showed an enlarged left posterior cervical lymph node, measuring 8 cm × 6 cm, firm, nontender, not attached to the overlying or underlying tissue. Power in the flexors, extensors, and abductors of the shoulder and hip joints were reduced. Systemic examinations revealed no other abnormality.
Investigations showed creatine kinase (CK) of 864 u/l (15-195 u/l), erythrocyte sedimentation rate (ESR) = 54 mm/h.
The anti-nuclear antibody and anti Jo-1 antibody (histidyl tRNA synthetase) were negative.
Fine needle aspiration cytology of lymph node showed malignant cells, and nasopharyngeal biopsy and histology shows poorly differentiated nasopharyngeal carcinoma [Figure 7] and [Figure 8] which was also suggested by postnasal space computerized tomography scan.
|Figure 7: Histology of nasopharyngeal biopsy at lower magnification showing poorly differentiated nasopharyngeal carcinoma|
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|Figure 8: Histology of nasopharyngeal carcinoma at a higher magnification showing poorly differentiated carcinoma|
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Sputum AFBX3 was negative, and sputum cytology showed no sign of inflammation or malignancy.
The final diagnosis of classic DM associated with nasopharyngeal carcinoma was made.
| Discussion|| |
The idiopathic inflammatory myopathies are a heterogenous group of genetically determined autoimmune disorders that predominantly target the skeletal muscles and/or skin and typically result in symptomatic skeletal muscle weakness and/or cutaneous inflammatory disease. These disorders are comprehensively classified as DM which include adult onset disease further classified as classic DM (classic DM alone, classic DM with malignancy, classic DM as an overlap connective tissue disorder) and clinically amyopathic DM (amyopathic DM and hypomyopathic DM). The Juvenile onset is classified as either classic DM or clinically amyopathic DM. Others include polymyositis (PM) either alone as part of overlap connective tissue disease or PM associated with malignancy.
DM was diagnosed in this patient based on the diagnostic criteria proposed by Tanimoto et al.  These criteria include the presence of diagnostic parameters such as heliotrope rash, Gottron's papule and Gottron's sign, proximal muscle weakness, muscle grasping and spontaneous pain, nondestructive arthritis or arthralgia, elevated CK or aldolase level, presence of systemic inflammatory signs, myogenic changes on electromyogram, positive anti Jo-1 antibody, and pathologic findings compatible with inflammatory myositis. The presence of one of 3 skin lesion item and 4 other items are diagnostic.  Based on the criteria, the index patient had Gottron's papule, heliotrope rash, proximal muscle weakness, elevated CK, elevated ESR (systemic inflammatory sign), and arthralgia, thus meeting the criteria. The absence of Jo-1 antibody is not suprising in this case because it is less frequently displayed in patients with classic cutaneous manifestations of DM like seen in this patient.
While the etiology of DM remains unknown, malignancies (ovarian, breast, lung, nasopharyngeal, and stomach), drugs (hydroxyurea, penicillamine, statins etc.), and viruses (Coxsackie, echovirus, human T-cell lymphotropic virus type 1, HIV) have been implicated as possible triggers.  However, the susceptibility phase of DM is thought to result largely from a genetic predisposition. DM is one of the human immune diseases; that is linked to the 8.1 ancestral haplotype (HLA-1A, C7, B8, C4AQO, C4B1, DR3, and DQ2).
Malignancy is thought to be the trigger of the disease in this case as nasopharyngeal carcinoma was present at the point of diagnosis. Both juvenile and adult-onset DM has been associated with increased risk of malignancy. Though this risk is considered negligible in juvenile onset DM, the definite risk of malignancy is up to 20-30% in adult onset classic DM, especially among patients who are 50 and above.  The malignancy is discovered before, simultaneously, or after the DM in almost equal proportion, with the highest probability of finding an associated tumor occurring within 2 years of diagnosis.  Recognized risk factors for malignancy include age (the fifth and sixth decade), constitutional symptoms, rapid onset of DM, lack of Raynaud phenomenon, and a grossly elevated ESR or CK. 
It has been suggested that other systemic manifestations of DM such as interstitial lung disease are negatively correlated with the risk of internal malignancy.  The type of carcinoma frequently found vary in different regions. Hill et al., reported that ovarian, lung, gastric, colorectal, and pancreatic cancers were most strongly associated with DM in several Northern European Countries,  whereas Wong reported that nasopharyngeal carcinoma was as high as 75% in Hong Kong,  and Ang et al., reported a rate of 50% in Singapore.  On the other hand, in Japan, gastric, lung, breast, and ovarian cancers were most associated with DM (in order of frequency). 
Unfortunately, there is a paucity of the report on DM associated with malignancy in this part of the world, and the frequency of occurrence of a different type of cancer associated with DM is unknown. Due to the significant risk of malignancy in adult patients with DM, routine screening for occult malignancy is always indicated, especially in patients 50 years and above who have the greatest risk.
DM associated with cancer is generally more resistant to corticosteroid and cytotoxic therapies than idiopathic DM. DM activity reflects the state of cancer, and some reports have described improvement of DM without immunosuppressive drugs after resection of cancer.
| Conclusion|| |
Classic DM is a rare genetically predisposed autoimmune inflammatory disorder with environmental triggers characterized by classical cutaneous features and proximal muscle weakness. Association with malignancy is a common feature and adequate screening to exclude that must be routinely undertaken, especially in the middle age and elderly individuals.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]