|LETTER TO THE EDITOR
|Ahead of print publication
Dermatomyositis: Look beyond skin
Karthik K Prasad1, Manish S Bhatia1, Nagesh T Sirsath2
1 Department of Surgical Oncology, Inlaks and Budhrani Hospital, Pune, Maharashtra, India
2 Department of Medical Oncology, Inlaks and Budhrani Hospital, Pune, Maharashtra, India
Karthik K Prasad,
Inlaks and Budhrani Hospital, 7-9, Koregaon Park, Pune - 411 001, Maharashtra
Source of Support: None, Conflict of Interest: None
Cancer is a multifaceted evil that may be lurking behind a variety of relatively benign faces. The initial diagnosis of cancer requires a good clinical acumen and a suspicious mind. The presentation of cancer as a paraneoplastic syndrome is not unknown yet often misleads the clinician. As these syndromes antedate the clinical manifestation of cancer, proper evaluation may lead to early diagnosis of cancer. We report a case of ovarian carcinoma presenting as dermatomyositis.
A 49-year-old postmenopausal woman presented with scaly lesions over the trunk and upper limb of 3-month duration with generalized weakness and dysphagia of 1-month duration. On examination, scaly maculopapular lesions were seen on the neck, trunk, and upper limbs. Neurological examination revealed a low lower limb power of 2/5 and weak gag reflex. On examination of the abdomen, a mass was palpable in the right iliac fossa. CA-125 level was elevated (990 IU/ml).
On further evaluation, computed tomography (CT) abdomen and pelvis showed a well-defined moderately enhancing lesion measuring 28 mm × 27 mm in the right ovary with multiple peritoneal deposits. Fine-needle aspiration cytology of the palpable peritoneal deposits in the right iliac fossa was suggestive of metastatic adenocarcinoma.
Creatine kinase (CK) levels were normal. Electromyography of proximal muscles was suggestive of myopathy. Hence, a diagnosis of ovarian adenocarcinoma with paraneoplastic dermatomyositis (DM) was made. Interval cytoreduction was planned, and the patient was started on neoadjuvant chemotherapy with weekly paclitaxel and carboplatin. The muscle weakness improved during therapy. The patient underwent complete pelvic peritonectomy with the removal of the right diaphragmatic peritoneum and ovarian cytoreductive surgery. Postoperative stay was uneventful. Histopathologic examination showed residual high-grade serous papillary adenocarcinoma, and multiple peritoneal nodules show viable metastatic deposits with chemotherapy-related changes. The patient received adjuvant chemotherapy and is currently doing well on regular follow up and there is regression of DM features.
Dermatomyositis is an idiopathic, inflammatory myopathy characterized by progressive symmetrical proximal muscle weakness associated with a characteristic rash. It has a reported incidence of 0.5–0.89 per 1 lakh population. Clinical features of dermatomyositis include photosensitivity and rashes affecting the upper back, chest, and shoulders (shawl sign), face, ears, and hands. Gottron's papules and heliotropic rash are characteristic. It is a systemic disease with frequent manifestations in the gastrointestinal tract and pulmonary system. CK levels are commonly elevated and used to monitor disease activity but can be normal in active disease.
It has been linked with internal malignancies in 15%–25% of cases. It is more commonly associated with lung cancer in men and ovarian cancer in women. The reported incidence of ovarian carcinoma in dermatomyositis is 13.3%. Dermatomyositis usually precedes malignancies such as ovarian, breast, and lung cancer by a few months. Skin and muscle disease may occur concurrently, or skin disease may precede muscle manifestations by 3 months to 6 years.
The pathogenesis is that of a microangiopathy, characterized by complement deposition in the microvasculature of the skin and muscle and the antibody-mediated capillary destruction. Confirmation requires a muscle biopsy that shows typical perivascular inflammatory cells that include plasmacytoid dendritic cells, abnormal capillaries, and perimysial perifascicular myofibers. The most consistent histological findings of DM on a skin biopsy include increased dermal mucin, vacuolar alteration of the basal cell layer, and mild-to-moderate mononuclear cell inflammatory infiltrates.
The specific autoantibody transcriptional intermediary factor-1 gamma (anti-p155/140) currently represents the best marker of neoplasia in dermatomyositis. The antibody positivity indicates high risk of neoplasia and forces the clinician to closely monitor the patient with exhaustive screening periodically, mainly during the first 3 years. Increased incidence of malignancy is seen up to 5 years after the diagnosis of dermatomyositis. Women should be screened by ultrasonography (USG) of the pelvic region and mammography and by CT thorax/abdomen. Men should be tested by CT thorax/abdomen. Men under the age of 50 years should have an USG of the testes. All patients over 50 years old (men and women) should have a colonoscopy. Screening is to be repeated annually for 3 years.
In patients with tumor-associated myositis, it was more frequently necessary to administer other immunosuppressive drugs along with glucocorticoids. The overall survival rate was considerably worse when compared to other forms of myositis. Curth's postulate states that when an underlying malignancy is causative of DM, treatment of the malignancy generally results in regression of the DM as occurred in the present case. Recurrence of DM may herald recurrence of underlying malignancy.
Low prevalence of paraneoplastic syndromes in specialist oncology practice results in patients consulting dermatology, endocrine, neurology, and other specialties initially. This case is an example of how oncology is relevant to all branches of medicine including dermatology, neurology, and others. Inspection of the skin is an essential part of the complete physical examination. Dermatomyositis should be considered a clinical sign indicating the possibility of underlying malignancy, and detailed evaluation of the patient should follow.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| > References|| |
Zaborowski MP, Spaczynski M, Nowak-Markwitz E, Michalak S. Paraneoplastic neurological syndromes associated with ovarian tumors. J Cancer Res Clin Oncol 2015;141:99-108.
Robinson AB, Reed AM. Clinical features, pathogenesis and treatment of juvenile and adult dermatomyositis. Nat Rev Rheumatol 2011;7:664-75.
Callen JP. Dermatomyositis. Lancet 2000;355:53-7.
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.
María del RF, Ana María GL, Flora NB, Javier HG, Francisco LA, et al
. Ovarian carcinoma following dermatomyositis diagnosis: A case report and review of the literature. J Gynecol Womens Health 2016;2:555-76.
Greenberg SA. Inflammatory myopathies: Evaluation and management. Semin Neurol 2008;28:241-9.
Smith ES, Hallman JR, DeLuca AM, Goldenberg G, Jorizzo JL, Sangueza OP, et al.
Dermatomyositis: A clinicopathological study of 40 patients. Am J Dermatopathol 2009;31:61-7.
Trallero-Araguás E, Rodrigo-Pendás JÁ, Selva-O'Callaghan A, Martínez-Gómez X, Bosch X, Labrador-Horrillo M, et al.
Usefulness of anti-p155 autoantibody for diagnosing cancer-associated dermatomyositis: A systematic review and meta-analysis. Arthritis Rheum 2012;64:523-32.
Titulaer MJ, Soffietti R, Dalmau J, Gilhus NE, Giometto B, Graus F, et al.
Screening for tumours in paraneoplastic syndromes: Report of an EFNS task force. Eur J Neurol 2011;18:19-e3.
András C, Ponyi A, Constantin T, Csiki Z, Szekanecz E, Szodoray P, et al.
Dermatomyositis and polymyositis associated with malignancy: A 21-year retrospective study. J Rheumatol 2008;35:438-44.
Curth HO. Skin lesions and internal carcinoma. In: Andrade R, Gumport SL, Popkin GL, Rees TD, editors. Cancer of the Skin. Philadelphia: W.B. Saunders; 1976. p. 1308-9.
Christie A, McKay N, Nussey F. Dermatomyositis as presenting feature of ovarian cancer, treated with neo-adjuvant chemotherapy and interval debulking surgery. Gynecol Oncol Case Rep 2013;6:13-5.
Hudson CN, Curling M, Potsides P, Lowe DG. Paraneoplastic syndromes in patients with ovarian neoplasia. J R Soc Med 1993;86:202-4.
Thiers BH. Dermatologic manifestations of internal cancer. CA Cancer J Clin 1986;36:130-48.