Balabanova, Maria

Dourmishev, Lyubomir

Miteva, Ljubka

Pozharashka, Joana

Rusinova, Darena

English

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Paraneoplastic Dermatomyositis in a Patient with  Metastatic Gastric CarcinomaActa Dermatovenerol Croat                              2020;28(2):120-122                               LETTER TO THE EDITORDear Editor,Paraneoplastic dermatomyositis is a distinct clini-cal variant of dermatomyositis (DM) in which the typi-cal cutaneous features and muscle weakness appear before, simultaneously, or after the diagnosis of an internal malignancy. It occurs in approximately one-third of patients with DM, predominantly adults, after the age of 40 (1). Different neoplasms have been de-scribed in association with DM, the most common of which are lung, breast, ovarian, gastrointestinal, pros-tate, and bladder cancers. The gender distribution of cancer type corresponds roughly to that of the gen-eral population (1,2).We report the case of a 58-year-old man who pre-sented with facial heliotrope erythema, periorbital edema, Gottron’s papules over the interphalangeal and metacarpophalangeal joints, and Gottron’s sign on the elbows (Figure 1). The patient also exhibited some less frequent skin signs of DM, such as shawl sign on the upper back and shoulders and V-sign on the neck and chest. Apart from the rash, he com-plained of weight loss, adynamia, dysphagia, cough, and scant expectoration, which he reported experi-encing over a 3-month period. The muscle involve-ment consisted of proximal muscle weakness and had appeared a month after the skin rash. The histology of the skin lesion revealed epider-mal atrophy, vacuolar degeneration of the basal kera-tinocytes, and perivascular and periadnexal lympho-cytic infiltrate in the upper dermis (Figure 2). Labo-ratory examination found increased creatine kinase (2822 U/L) and liver enzymes, anemia, and leukocyto-sis. Screening for antinuclear antibodies and anti-Jo1 autoantibodies were both negative. The diagnosis of trichinosis was excluded via serologic examination. The impaired general condition of the patient led to a prompt paraneoplastic screening. Abdominal sonography detected hepatomegaly. Computed to-mography (CT) of the abdomen and pelvis visualized a mass in the distal part of the esophagus, narrowed lumen of the gastric cardia, enlarged gastric lymph nodes, lung and liver metastases, and ascites (Figure 3). The diagnosis of paraneoplastic DM in association with an advanced, metastatic, primary gastric carci-noma was established. Palliative surgery and chemo-therapy were proposed to the patient, but he refused both. A systemic therapy with methylprednisolone Figure 1. (A) Heliotrope erythema, periorbital edema, ery-thematous to livid discoloration of the face, and scaly ery-thema of the scalp; (B) Shawl sign on the upper back and shoulders; (C) Gottron’s papules over the interphalangeal and metacarpophalangeal joints; (D) Gottron’s sign over the elbows.ACTA DERMATOVENEROLOGICA CROATICA12012160 mg/daily and azathioprine 100 mg/daily was initi-ated, aiming to alleviate the progressively worsening muscle weakness, but proved ineffective. The patient died two months later of combined respiratory and heart failure. There are multiple prediction factors, such as cu-taneous signs, laboratory data, and disease progres-sion, which may direct the physician towards the pos-sibility of paraneoplastic DM. Some atypical cutaneous lesions, such as cuta-neous necrosis or vasculitis, hyperkeratotic follicular papules, vesiculo-bullous lesions, and flagellate ery-thema, are seen more frequently in cancer-associated DM (3,4). None of these were present in our patient. Pruritus is also described as a paraneoplastic sign (5). Some authors consider the increased erythrocyte sedimentation rate and C-reactive protein to be of predictive value for malignancy.Myositis-specific autoantibodies anti-TIF1-γ and anti-NXP-2, among the numerous novel serological markers for DM, are clearly associated with the pres-ence of neoplasia (6,7). Unfortunately, we were un-able to test for those autoantibodies.The symptom of dysphagia is a hallmark of para-neoplastic dermatomyositis and usually represents a manifestation of muscle weakness (8). In our case, it was rather a reflection of the endoluminal tumor, al-though it may also be a combination of both factors. In their study, Bowerman et al. investigated the risk of cancer development in different DM subtypes (9). They included 201 patients with adult-onset DM, 142 of with classic DM and 59 with the clinically amyo-pathic type. The estimated prevalence of malignancy-associated classic and clinically amyopathic DM were 9.9% and 1.7%, respectively. The authors concluded that older age and classic DM represent independent risk factors for malignancy-associated DM within 2 years of disease onset.Given that early diagnosis significantly impacts prognosis in patients with cancer-associated DM, re-cent studies support blind screening for internal ma-lignancy (10). Leatham et al. performed a retrospec-tive analysis of 400 patients with DM, finding a total of 53 cancers in 48 patients (some of the patients had two separate neoplasms). Among the group of para-neoplastic DM cases, 17 cancers were diagnosed via purely blind screening in patients with a lack of con-cerning history or physical examination. The authors Figure 3. Abdominal CT scan visualizing a mass in the distal part of esophagus, narrowed lumen of the gastric cardia, enlarged gastric lymph nodes, and lung and liver metastases.Figure 2. Hematoxylin and eosin staining (×200). Epider-mal atrophy, vacuolar degeneration of the basal keratino-cytes, perivascular and periadnexal lymphocytic infiltrate in the upper dermis.Letter to the editor Acta Dermatovenerol Croat  2020;28(2):120-122ACTA DERMATOVENEROLOGICA CROATICA122 ACTA DERMATOVENEROLOGICA CROATICAclaimed that the most informative tests were mam-mography and CT scanning. The above-mentioned predictive factors for paraneoplastic DM represent a useful tool for the clinician. Although it is gener-ally accepted that patients with DM should undergo some type of cancer screening, there is no consensus regarding methods or frequency. New data suggest that blind screening in asymptomatic patients might be of great importance for early diagnosis and treat-ment of patients with cancer-associated DM. References: 1. Dourmishev L, Draganov P. Paraneoplastic derma-tological manifestation of gastrointestinal malig-nancies. World J Gastroenterol. 2009;15:4372-9.2. Sawada T, Nakai N, Masuda K, Katoh N. Para-neoplastic dermatomyositis associated with gallbladder Ccrcinoma: A case report and mini-review of the published work. Indian J Dermatol. 2014;59:615-6. 3. Zangrilli A, Papoutsaki M, Bianchi L, Teoli M, Chi-menti S. Bullous dermatomyositis: a marker of poor prognosis and aggressive internal malig-nancy? Acta Derm Venereol. 2008;88:393-4.4. Manriquez J, Acle R, Llanos C. Flagellate erythema in a case of paraneoplastic dermatomyositis. J Clin Rheumatol. 2016;22:435.5. Rowe B, Yosipovitch G. Paraneoplastic itch mana-gement. Curr Probl Dermatol. 2016;50:149-54.6. Venalis P, Selickaja S, Lundberg K, Rugiene R, Lund-berg IE. Association of anti-transcription interme-diary factor 1γ antibodies with paraneoplastic rheumatic syndromes other than dermatomyosi-tis. Arthritis Care Res (Hoboken). 2018;70:648-51.7. Ichimura Y, Matsushita T, Hamaguchi Y, Kaji K, Ha-segawa M, Tanino Y, et al. Anti - NXP2 autoantibo-dies in adult patients with idiopathic inflammatory myopathies: possible association with malignan-cy. Ann Rheum Dis. 2012;71:710-3.8. Ofori E, Ramai D, Ona M, Reddy M. Paraneoplastic dermatomyositis syndrome presenting as dyspha-gia. Gastroenterology Res. 2017;10:251-4.9. Bowerman K, Pearson DR, Okawa J, Werth VP. Ma-lignancy in dermatomyositis: A retrospective stu-dy of 201 patients seen at the University of Penn-sylvania. J Am Acad Dermatol. 2020 Jul;83:117-22. 10. Leatham H, Schadt C, Chisolm S, Fretwell D, Chung L, Callen JP, et al. Evidence supports blind screen-ing for internal malignancy in dermatomyositis: Data from 2 large US dermatology cohorts. Medi-cine (Baltimore). 2018;97(2):e9639.Joana Pozharashka1, Lyubomir Dourmishev1, Darena Rusinova2, Maria Balabanova1, Ljubka Miteva11Department of Dermatology and Venereology, Medical University – Sofia, Bulgaria2Department of Dermatology and Venereology, MBAL “Tota Venkova”, Gabrovo, BulgariaCorresponding author: Lyubomir DourmishevAddress: 1 St. Georgi Sofiiski Blvd.Sofia 1431, Bulgarial_dourmishev@yahoo.comReceived: October 17, 2019Accepted: May 15, 2020Letter to the editor Acta Dermatovenerol Croat  2020;28(2):120-122

Paraneoplastic Dermatomyositis in a Patient with Metastatic Gastric Carcinoma

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