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CORRESPONDENCE
Year : 2020  |  Volume : 65  |  Issue : 1  |  Page : 80-81
Paraneoplastic dermatomyositis in association with poorly differentiated esophageal carcinoma – A rare association and a brief review of literature


Department of Dermatology and Venereology, Government Medical College, Trivandrum, Kerala, India

Date of Web Publication13-Jan-2020

Correspondence Address:
S Pradeep Nair
Department of Dermatology and Venereology, Government Medical College, Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_617_18

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How to cite this article:
Subhash S, Nair S P, Samad K A, Simon SA, Gomathy M. Paraneoplastic dermatomyositis in association with poorly differentiated esophageal carcinoma – A rare association and a brief review of literature. Indian J Dermatol 2020;65:80-1

How to cite this URL:
Subhash S, Nair S P, Samad K A, Simon SA, Gomathy M. Paraneoplastic dermatomyositis in association with poorly differentiated esophageal carcinoma – A rare association and a brief review of literature. Indian J Dermatol [serial online] 2020 [cited 2020 Jan 27];65:80-1. Available from: http://www.e-ijd.org/text.asp?2020/65/1/80/275772




Sir,

Dermatomyositis (DM) is an autoimmune connective tissue disorder involving the skin, muscles, and other organs. Many consider it to be a paraneoplastic manifestation of organ specific malignancy. Paraneoplastic dermatomyositis (PDM) is most commonly associated with lung carcinoma in men and ovarian and breast carcinoma in women.[1],[2] The reported frequency of malignancy in literature varies from 7% to 60%.[1],[2] The association between PDM and esophageal carcinoma is very rare.

A 45-year-old male presented with reddish skin lesions, muscle weakness, dysphagia, and joint pain of 2 months duration. The skin lesions first appeared on the abdomen and later involved the chest, back, upper limbs, and thighs. This was soon followed by dysphagia to both solid and liquid foods and joint pain involving the shoulder, hip, and ankle joints. The patient also had difficulty in raising his arms, in climbing stairs, and getting up from the squatting position. On examination the patient had bilateral ill-defined erythematous edematous plaques periorbitally more prominent on the lower eyelids (heliotrope rash) [Figure 1], multiple discrete and confluent erythematous patches with erosions, scaling, and areas of hyperpigmentation distributed on the anterior abdomen, posterior trunk, upper limbs, and thighs (Holster sign). Periungal telangiectasias were present along with ragged cuticles. There was painful limitation of movement of the shoulder, hip, and ankle joints.
Figure 1: Edematous erythematous plaques around eyes suggestive of heliotrope rash Figure 2: Skin biopsy of the rash on the back showing basal cell vacuolation (white arrow), pigmentary incontinence (white arrow) and perivascular infiltrate (interphase dermatitis) suggestive of dermatomyositis (H and E, ×400) Figure 3: Biopsy of the esophagus showing cells with hyperchromatic nuclei, scanty cytoplasm, pleomorphic cells, and bizarre cells (black arrows) suggestive of poorly differentiated carcinoma, (H and E, ×400)

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Antinuclear antibody (ANA) profile, anti-Jo-1, anti-Mi-2, and antimelanoma differentiation antibodies (MDA-5) were negative. Serum creatine phosphokinase (CPK) level (1115, normal: 25–195) and lactate dehydrogenase (LDH) level (414, normal: 135–225) were elevated. However, electromyogram (EMG) and muscle biopsy were normal. A fiber-optic esophago-gastro-duodenoscopy (OGD-scopy) showed an ulcero-proliferative growth at the distal end of the esophagus and extending to the esophageal-gastric junction suggestive of malignancy. A high contrast CT-scan of the abdomen and chest did not show any secondaries or malignancies elsewhere. A skin biopsy of the lesions on the trunk showed interphase dermatitis with perivascular infiltrates suggestive of DM [Figure 2]. A biopsy of the ulcero-proliferative growth of the esophagus showed a neoplasm arranged in sheets and nests, composed of pleomorphic cells with hyperchromatic nuclei, scanty cytoplasm, and bizarre cells, suggestive of a poorly differentiated carcinoma [Figure 3]. Immunocytochemistry showed cytokeratin [Figure 4], while p63, CD3, and CD20 were negative ruling out secondaries in the esophagus. We made a final diagnosis of paraneoplastic DM with poorly differentiated carcinoma of the esophagus.
Figure 2: Skin biopsy of the rash on the back showing basal cell vacuolation (white arrow), pigmentary incontinence (white arrow) and perivascular infiltrate (interphase dermatitis) suggestive of dermatomyositis (H and E, ×400)

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Figure 3: Biopsy of the esophagus showing cells with hyperchromatic nuclei, scanty a primary carcinoma of the esophagus (cytokeratin stain, ×100) differentiated carcinoma, (H and E, ×400)

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Figure 4: Biopsy of the esophagus showing positive stain for cytokeratin indicating a primary carcinoma of the esophagus (cytokeratin stain, ×100)

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Our patient presented with heliotrope rash, Holster sign, ragged cuticles, and an erythematous rash histologically consistent with DM. The patient had clinically elicited proximal muscle weakness and elevated CPK and LDH levels. Thus, with the characteristic rash of DM and with two criteria of myositis of the Bohan and Peter criteria, a diagnosis of probable DM was made. The hallmark of this case was the very rare occurrence of esophageal carcinoma in association with paraneoplastic DM. There are less than 10 case reports in literature.[3] Another hallmark of this case was the presence of the carcinoma at the esophageal-gastric junction which to the best of our knowledge was not reported elsewhere. Another very important highlight of our case was the severe dysphagia seen in our case. Moderate to severe dysphagia is the hallmark of DM due to the involvement of the cricopharyngeus and other skeletal muscles of the pharynx and esophagus. Hence, this symptom is considered to be a part and parcel of DM and usually OGD-scopy is not done. However, in our case, the severe symptoms and dysphagia to both solid and liquid foods prompted us to do OGD-scopy and we could pick up the esophageal carcinoma. Hence, we advocate that an OGD-scopy be made mandatory for any patient with DM to rule out esophageal carcinoma.

Acknowledgments

We deeply appreciate the help by Dr. G Nanda Kumar and Dr. Preethamol S from the Department of Pathology, Government Medical College, Trivandrum in providing the histopathology slides for the manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Wakata N, Kurihara T, Saito E, Kinoshita M. Polymyositis and dermatomyositis associated with malignancy: A 30-year retrospective study. Int J Dermatol 2002;41:729–34.  Back to cited text no. 1
    
2.
Yusipovitch G, Tan A, Losicco K, Manabat CG, Kannagra A, Carroll C, et al. A comparative study of clinical characteristics, work-up, treatment, and association to malignancy in dermatomyositis between two tertiary skin centers in the USA and Singapore. Int J Dermatol 2013;52:813–9.  Back to cited text no. 2
    
3.
Kooistra L, Ricotti C, Galimberti F, Gota C, Fernandea AP. Malignancy associated dermatomyositis: Retrospective case-control study from a single tertiary care center. J Am Acad Dermatol 2018;79:152–5.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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