Dermatomyositis and esophageal cancer.

Objective: A case of dermatomyositis and esophageal cancer is described.

Case Report: A 58-year-old male recently diagnosed with esophageal cancer was admitted to the hospital with complaints of progressive dysphagia, generalized muscle weakness and skin rash. The weakness started symmetrically in the proximal limb muscles. He also developed a characteristic skin rash on the eyelids, the upper chest and around the nails that was thought to be most indicative of dermatomyositis. Creatine kinase, aspartate aminotransferase and aldolase were elevated. A muscle biopsy showed various degrees of degeneration with perivascular interstitial infiltration of lymphoplasma cells, a finding consistent with "dermatomyositis." The patient was started on corticosteroids and within two weeks, his muscle strength was found to be markedly improved and the rash almost disappeared.

Conclusion: Dysphagia can be the presenting symptom of both dermatomyositis and esophageal cancer. In the setting of an underlying malignancy, these symptoms can be misleading and one can miss the diagnosis of dermatomyositis. However, recognition of the characteristic skin rash may provide a clue to the diagnosis. Another aspect of our case that is worth acknowledgment is the quick response to treatment with corticosteroids.

Key Words: dermatomyositis, polymyositis, esophageal carcinoma

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The case report of a patient with dermatomyositis and esophageal cancer is reported and their association is discussed. To our knowledge, there have been only two such case reports published in English. We describe the case of a patient who had been recently diagnosed with esophageal cancer and was incidentally found to have dermatomyositis that subsequently responded to treatment.

A 58-year-old white male patient was admitted to the hospital with a chief complaint of generalized muscle weakness and skin rash. According to the patient, the weakness started symmetrically, first in the proximal arm muscles and then in the proximal leg muscles. The symptoms progressed for 16 weeks. The patient also complained of a skin rash on the lower abdomen, nape of neck, around the eyes, on the upper chest and around the nails. His appetite had been decreasing and he had lost about 75 pounds over the previous 8 months. He complained of difficulty swallowing food that was worse with solids and this had been getting worse over the previous 3 weeks. He also complained of a hoarse voice, dry skin, and shortness of breath on exertion. His medical history included diabetes x 8 years, hypertension, hyperlipidemia, and morbid obesity. He had recently been diagnosed with stage III adenocarcinoma of the esophagus. His father had esophageal cancer. He denied drinking but had a smoking history of 40 pack years.

On physical examination, the patient was obese and fatigued, but was in no acute distress. He weighed 337 pounds and was 5 feet and 6 inches. His vital signs were stable. A visible erythematous rash was seen on the eyelids, nape of the neck and the upper chest (Fig. 1). A rash around the nails and on the knuckles (Fig. 2) was also evident. The patient had a systolic murmur heard in the aortic area with trace ankle edema. He had symmetric weakness in all the proximal limb muscles but had intact deep tendon reflexes and sensations. The rest of the examination was unremarkable. Laboratory investigations revealed a creatine kinase of 241 U/L, aspartate aminotransferase of 88 U/L and aldolase of 7.6. C-reac-tive protein was reported to be 8.0 mg/dL and TSH was 1.83 mclU/mL. His intact parathyroid hormone was 74 pg/mL with a calcium of 8.3 mg/dL and a phosphorus of 3.4 mg/dL. Muscle biopsy showed various degrees of degeneration with perivascular interstitial infiltration of lymphoplasma cells that was consistent with dermatomyositis. From the clinical findings and investigations, a diagnosis of dermatomyositis was made and the patient was started on prednisone 60 mg daily. He was seen regularly in the clinic after his discharge from the hospital. On his second follow-up visit, his muscle power was markedly improved and the had no complaints of weakness in his muscles during his daily activities. The rash on his skin, including the periungual rash and the Gottron papules on his knuckles, were fading.

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Our patient experienced a rare case of dermatomyositis associated with esophageal cancer that subsequently responded to treatment. Similar to our case, Karp et al (1) described the case of a 63-year-old male with dermatomyositis and squamous cell carcinoma of the esophagus who presented with dysphagia and muscle weakness. These two cases are similar in that both patients responded to high-dose prednisone and muscle power in all groups returned to 4/5. Mastaglia et al, (2) Dalakas et al, (3) Christopher et al (4) and Wortmann et al (5) have suggested that about 90% of patients will respond only partially to corticosteroid therapy and some 50 to 75% might achieve complete remission. And if a relapse occurs after remission, patients should be started on high doses of prednisone and immunosuppressive agents like azathioprine, methotrexate, and cyclophosphamide, and cyclosporin should be used when the steroids are being tapered down. In 2002, Callen et al (6) reported that mycophenolate mofetil can also be tried in dermatomyositis.

In our case, the patient's initial complaints were "dysphagia" and "generalized body weakness." In the setting of an established diagnosis of an underlying malignancy, these complaints can be misleading and one can miss the diagnosis of dermatomyositis. De Merieux et al (7) described the commonality of "dysphagia" being the presenting symptom in both esophageal cancer and dermatomyositis, especially in cases which had an incomplete response to treatment with immunosuppressive drugs or corticosteroids, illustrating that the underlying mechanism of dysphagia in dermatomyositis is the involvement of the skeletal muscles of the pharynx and esophagus and some structural abnormalities of the distal esophagus. Other case reports similar to ours are worth mentioning. Tanabe et al (8) has described a 78-year-old Japanese woman with dermatomyositis who also had Barrett esophagus-associated adenocarcinoma, which showed neuroendocrine differentiation and this patient presented as a case of "humoral hypercalcemia of malignancy." This was due to the ectopic PTHrp production from the tumor. Treluyer et al, (9) Bessonov et al, (10) and Lombardo et al (11) have reported similar patients.

In 1975, Bohan et al (12) established criteria for the diagnosis of dermatomyositis (Table). Tanimoto et al (13) further elucidated the importance of the first three criteria and concluded that the definitive diagnosis of dermatomyositis can be made with the first three of the four criteria and the presence of the rash. According to this criteria, an elevated muscle enzyme level seems to be the only laboratory criterion included in the American College of Rheumatology criteria for the diagnosis of polymyositis and dermatomyositis.

The pathogenesis of the cutaneous disease is poorly understood, but it appears that dermatomyositis (DM) and polymyositis (PM) have different pathogeneses. It has been suggested that DM is caused by complement-mediated vascular inflammation whereas PM is probably due to a direct cytotoxic effect of CD[8.sup.+] lymphocytes on muscle.

Dermatomyositis and esophageal cancer can both present with dysphagia as the presenting symptom. In the presence of an underlying malignancy, these symptoms can be misleading. Early recognition of the skin rash can give an initial clue. The mainstay of treatment remains the corticosteroids and although a fast treatment response has not been reported, one may expect a quick recovery, as in our case.

References

1. Karp SJ. Acute dermatomyositis associated with squamous carcinoma of the oesophagus. J R Soc Med 1985 Sep;78:770-771.

2. Mastaglia FL, Garlepp MJ, Phillips BA, et al. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve 2003;27:407-425.

3. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003;362:971-982.

4. Christopher-Stine L, Plotz PH. Adult inflammatory myopathies. Best Pract Res Clin Rheumatol 2004;18:331-344.

5. Wortmann R. Inflammatory and metabolic diseases of muscles. In: Klippel JH (ed): Primer on the Rheumatic Diseases. Atlanta, Arthritis Foundation, 2001, 369-76.

6. Callen JP. Dermatomyositis. Available at: http://www.e-medicine.com. Accessed October 19, 2005.

7. de Merieux P. Verity MA, Clements PJ, et al. Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis. Arthritis and Rheumatism 1983;26:961-968.

8. Tanabe S, Mitomi H, Sada M, et al. Parathyroid hormone-related protein production by adenocarcinoma in Barrett's esophagus patient with dermatomyositis. Dig Dis Sci 2001;46:1584-1588.

9. Treluyer JM, Largenton C, Guesnon P, et al. [Dermatomyositis and cancer of the esophagus. Case report, review of the literature and discussion of the relation between dermatomyositis and cancer] [Article in French]. Chirurgie 1988;114:130-133.

10. Bessonov IA, et al. [Dermatomyositis associated with carcinoma of the prostate and esophagus] [Article in Russian]. Vrach Delo 1971;12:99-101.

11. Lombardo F, Ferrari G, et al. [On a case of dermatomyositis associated with esophageal neoplasm] [Article in Italian]. Minerva Med 1968:59: 3727-3730.

12. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292:344-347.

13. Tanimoto K, Nakano K, Kano S, et al. Classification criteria for polymyositis and dermatomyositis [published erratum appears in J Rheumatol 1995:22:1807]. J Rheumatol 1995;22:668-674.

The measure of a man's character is what he would do if he knew he
Never would be found out.
--Thomas Babington Macaulay

Imran Iftikhar, MD, Dima Abdelmannan, MD, MRCP, and Hamed A. Daw, MD

From the Departments of Internal Medicine and Hematology/Oncology, Cleveland Clinic Cancer Center, Moll Pavilion, Fairview Hospital, Cleveland, OH.

Reprint requests to Hamed A. Daw, MD, Cleveland Clinic Cancer Center, Moll Pavilion, Fairview Hospital, 18200 Lorain Avenue, Cleveland, OH 44111. Email: [e-mail]

Accepted February 3, 2006.

RELATED ARTICLE: Key Points

* The incidence of dermatomyositis and esophageal cancer is rare.

* It is important to differentiate dysphagia due to a malignancy from dermatomyositis.

* The mainstay of treatment remains corticosteroids and with early recognition and initiation of treatment, a faster response can be expected.

Table. Diagnosis of dermatomyositis and polymyositis

1. Symmetric weakness of limb girdle muscle and anterior neck flexors,
   progressing over weeks to months, with or without dysphagia or
   respiratory muscle involvement.
2. Positive muscle biopsy.
3. Elevation of muscle enzymes.
4. Electromyographic evidence with a triad of
   i) Short, small polyphasic motor units
   ii) Fibrillations, positive sharp waves, insertional irritability
   iii) Bizarre, high frequency repetitive discharges
5. Dermatologic features, including heliotrope rash and Gottron sign

Adapted from Bohan A. Peter JB. Polymyositis and dermatomyositis. N Engl
J Med 1975:292:344-347: and Bohan A. Peter JB. Polymyositis and
dermatomyositis. N Engl J Med 1975:292:403-407.