Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor - Observation Letter
2017:83:5;606-609
doi: 10.4103/ijdvl.IJDVL_697_16
PMID: 28731019

Dermatomyositis revealing both a metastatic linitis plastica and hepatitis C virus infection

Imane Alouani, Nadia Fihmi, Nada Zizi, Siham Dikhaye
 Department of Dermatology, Mohammed VI University Hospital of Oujda, Medical School of Oujda, Mohammed First University of Oujda, Oujda, Morocco

Correspondence Address:
Imane Alouani
Department of Dermatology, Mohammed VI University Hospital of Oujda, Oujda
Morocco
Published: 20-Jul-2017
How to cite this article:
Alouani I, Fihmi N, Zizi N, Dikhaye S. Dermatomyositis revealing both a metastatic linitis plastica and hepatitis C virus infection. Indian J Dermatol Venereol Leprol 2017;83:606-609
Copyright: (C)2017 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Dermatomyositis is a rare disease, characterized by an inflammatory myopathy and typical cutaneous lesions. Its relationship with cancer is well known.[1] However, its association with hepatitis C virus has rarely been reported in the literature.[2] We report a patient with dermatomyositis revealing a metastatic linitis plastica and hepatitis C virus infection.

An 80-year-old woman with no preceding history of autoimmune disease, malignancy or jaundice was admitted for a periorbital heliotrope rash [Figure - 1] with muscle weakness. She reported having dysphagia with episodes of accidental liquid aspiration, including saliva. Physical examination revealed discrete erythematous papules overlying the interphalangeal joints (Gottron's papules) [Figure - 2] and prominent periungual erythema with ragged cuticles (manicure sign) [Figure - 3]. Manual muscle testing showed proximal muscle weakness (2/5 according to Medical Research Council Scale). Elevated serum muscle enzymes were observed with increased creatine kinase to 426 U/L (normal <170 U/L); the aspartate aminotransferase and lactic dehydrogenase levels were 87 and 546 U/L, respectively (normal aspartate aminotransferase <35 U/L and lactic dehydrogenase 190–400 U/L). Antinuclear antibody, anti-aminoacyl transfer ribonucleic acid synthetase, anti-signal recognition particle, anti-Mi-2 antibody and anti-PM/SCL antibody were all negative. The electromyography revealed proximal muscle-predominant myogenic change with fibrillation waves and abnormal low amplitude and short duration of motor unit action potential. The muscle biopsy showed perifascicular muscle atrophy associated with interstitial fibrosis and perivascular inflammatory cell collections [Figure - 4], [Figure - 5], [Figure - 6]. Based on these findings and on Bohan and Peter's criteria (the five criteria are: symmetric proximal muscle weakness, elevation of serum skeletal muscle enzymes, the electromyographic triad, muscle biopsy abnormalities and typical skin rash of dermatomyositis), a diagnosis of dermatomyositis was made. The patient was started on prednisolone 2mg/kg/day, with improvement in her weakness and dysphagia. Intravenous immunoglobulin therapy was considered, but could not be administered due to financial constraints. Laboratory workup revealed positive hepatitis C antibody with a high titer: 815,000 IU/ml.

Figure 1: Periorbital heliotrope rash
Figure 2: Gottron's papules on the left foot. Note the characteristic erythematous papules overlying the interphalangeal joints
Figure 3: Prominent periungual erythema with ragged and painful cuticles on the right hand suggestive of manicure sign
Figure 4: Muscle biopsy showing perifascicular muscle atrophy (Masson's Trichrome, ×100)
Figure 5: Muscle biopsy showing interstitial fibrosis (Masson's Trichrome, ×200)
Figure 6: Muscle biopsy showing perivascular inflammatory cell infiltrate (Masson's Trichrome, ×400)

Cancer screening using thoracic, abdominal and pelvic computed tomography revealed metastatic lesions within the spine and the liver, ascites and peritoneal nodules [Figure - 7]. Digestive endoscopy showed a macroscopically normal gastric mucosa. However, there was a malignant glandular proliferation of independent cells (signet ring cells) in the fibrous stroma histologically [Figure - 8] and [Figure - 9]. Due to the poor prognosis of her metastatic carcinoma (stage T4bN3M1), interferon-based therapy for her hepatitis C could not be started.

Figure 7: Abdominal computed tomography scan showing hypoattenuating lesions suggestive of liver metastases (arrows)
Figure 8: Gastric mucosa biopsy showing tumor proliferation in gastric mucosa (HES, ×10)
Figure 9: Gastric mucosa biopsy showing signet ring cells (arrows) (HES, ×50)

Chronic hepatitis C virus infection has been known to be associated with the presence of autoantibodies and various types of autoimmune diseases, leading to the hypothesis that chronic hepatitis C virus infection could cause dermatomyositis through an autoimmune mechanism.[3] However, this coexistence could be coincidental. Studies have been published on the outcome of corticosteroid therapy in patients with chronic hepatitis C virus infection. Some studies have described a rapid progression of the liver disease in immunosuppressed patients with chronic hepatitis C virus infection.[4] In our case, steroids could not be stopped because of the life-threatening consequences of her severe dermatomyositis. During the initial two months of follow-up, the patient did not show any sign of reactivation of her hepatitis. Liver enzymes' status was normal. The patient was then lost to follow-up. The alternative treatment considered was intravenous immunoglobulin therapy. It can be tried in patients with contraindication for corticosteroids.[5] Furthermore, intravenous immunoglobulin has been shown to be effective in most dermatomyositis patients with esophageal involvement.[5]

On the other hand, the relationship between cancer and dermatomyositis has been widely reported in the literature. Recent studies demonstrated that 15%–32% of patients with dermatomyositis are associated with various internal malignancies.[1] Ovarian and breast cancers are the most frequent neoplasms in women.[1] Linitis plastica is a rare, malignant gastric adenocarcinoma characterized by glandular proliferation of signet ring cells in the fibrous stroma.[6] Due to its metastatic stage in the present case, curative surgery could not be proposed. The patient refused the palliative chemotherapy. Terada described in 2013 a patient with dermatomyositis associated with a signet ring cell carcinoma of esophagus.[7] However, after a literature review, no previous association has been noted between dermatomyositis and gastric signet ring cell carcinoma.

This is a rare case of a patient with dermatomyositis, revealing both a metastatic linitis plastica and hepatitis C virus infection. This case emphasizes again the need for tumor screening, when dermatomyositis is diagnosed in advanced adulthood. It raises a discussion about the risk–benefit profile of corticosteroid therapy in the presence of chronic infection, particularly in life-threatening situations such as a severe dermatomyositis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgment

The authors wish to thank Dr. Mouna Rimani, Dr. Amal Bennani and Dr. Nawal Harchichi, for their valuable assistance to the histopathology photographs.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: A population-based study. Lancet 2001;357:96-100.
[Google Scholar]
2.
Kee KM, Wang JH, Lee CM, Changchien CS, Eng HL. Chronic hepatitis C virus infection associated with dermatomyositis and hepatocellular carcinoma. Chang Gung Med J 2004;27:834-9.
[Google Scholar]
3.
McMurray RW, Elbourne K. Hepatitis C virus infection and autoimmunity. Semin Arthritis Rheum 1997;26:689-701.
[Google Scholar]
4.
Fong TL, Valinluck B, Govindarajan S, Charboneau F, Adkins RH, Redeker AG. Short-term prednisone therapy affects aminotransferase activity and hepatitis C virus RNA levels in chronic hepatitis C. Gastroenterology 1994;107:196-9.
[Google Scholar]
5.
Marie I, Menard JF, Hatron PY, Hachulla E, Mouthon L, Tiev K, et al. Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: A series of 73 patients. Arthritis Care Res (Hoboken) 2010;62:1748-55.
[Google Scholar]
6.
Issam Beyrouti M, Beyrouti R, Ben Amar M, Frikha F, Beyrouti R, Abid M, et al. Linitis plastica. Presse Med 2007;36(12 Pt 2):1782-6.
[Google Scholar]
7.
Terada T. Signet-ring cell carcinoma of the esophagus in dermatomyositis: A case report with immunohistochemical study. J Gastrointest Cancer 2013;44:489-90.
[Google Scholar]

Fulltext Views
510

PDF downloads
268
Show Sections