|Year : 2023 | Volume
| Issue : 1 | Page : 40-41
Dermatomyositis with insidious advanced renal cell carcinoma: A case report
Meng-Han Shen, Shih-Jyun Yang, Ya-Ching Chang, Ya-Hui Chuang
Department of Dermatology, Chang Gung Memorial Hospital, Keelung, Taipei, and Taoyuan; College of Medicine, Chang Gung University, Taoyuan, Taiwan
|Date of Submission||03-May-2022|
|Date of Decision||31-Aug-2022|
|Date of Acceptance||09-Oct-2022|
|Date of Web Publication||27-Mar-2023|
Dr. Ya-Hui Chuang
Department of Dermatology, Chang Gung Memorial Hospital, No. 199, Tung Hwa N. Rd., Taipei 105
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Shen MH, Yang SJ, Chang YC, Chuang YH. Dermatomyositis with insidious advanced renal cell carcinoma: A case report. Dermatol Sin 2023;41:40-1
Dermatomyositis (DM) is an inflammatory myopathy and manifests with characteristic skin findings, including Gottron's sign or papules, V sign, and shawl sign. The disease is frequently linked to underlying malignancies from the lung, stomach, and ovaries. Older age, extent and severity of cutaneous lesions, dysphagia, and antibodies to transcription intermediary factor-1 gamma (TIF-1γ), and nuclear matrix protein-2 are factors associated with a higher risk of internal malignancy and a worse prognosis.
We report a 58-year-old Taiwanese woman who presented with an acute onset of painful and pruritic skin lesions on the face, neck, trunk, and four limbs for 10 days. Informed consent was obtained for the submission. Her medical history included hepatitis B and diabetes mellitus for which she received regular treatment of sitagliptin and metformin. She also had weakness of the bilateral thighs. The patient had no fever, dyspnea, and joint pain and could not recall any past history of drug allergy or recent exposure to new medicines.
Physical examination revealed confluent, scaly erythematous patches and papules on the face [Figure 1]a, V area of the anterior neck [Figure 1]b, and back. Erythematous to violaceous papules on the knuckles (Gottron's papules) were noted [Figure 1c]. A muscle power examination demonstrated mild symmetrical proximal weakness (4/5) of the hip flexor.
|Figure 1: (a) The Heliotrope sign on the bilateral upper eyelids was not obvious in the patient. (b) Erythematous to violaceous patches with confluence on the neck. (c) Gottron's papules on the finger joints. (d) Whole-body computed tomography revealed a huge mass in the left kidney|
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On investigation, the complete blood count (CBC) and urinalysis were unremarkable. Slight elevation of alanine aminotransferase 48 (reference range: ≤36 U/L), creatinine 1.28 (normal range: 0.44–1.03 mg/dL), and blood urea nitrogen 24.9 (normal range: 6–21 mg/dL) were noted. Blood biochemistry for muscle enzymes showed a slight elevation of aspartate aminotransferase 41 (reference range: ≤34 U/L) and lactate dehydrogenase 293 (reference range: 135–260 U/L). Curiously, she had a normal level of creatine kinase 177 (reference range: 20–180 U/L). Studies for myositis-specific markers revealed positivity for anti-TIF-1γ and anti-OJ (anti-isoleucyl-tRNA synthetase) antibodies. Antinuclear antibodies and autoantibodies to double-stranded DNA, Sm, ribonucleoprotein, SSA, and SSB were all negative. Skin biopsy on the neck showed parakeratosis, vacuolar degeneration of the basal layer, melanin incontinence, and perivascular lymphocytic infiltration. The histology was compatible with poikilodermatous dermatitis.
According to the diagnostic criteria of the European League Against Rheumatism/American College of Rheumatology in 2017, the diagnosis of DM in our patient was definite based on the presentations of an onset age older than 18, objectively symmetric proximal weakness, and Gottron's papules. Electromyography and muscle biopsy were suggested but the patient refused.
A comprehensive survey for malignancy including tumor markers, rhinolaryngoscopy, chest radiography, an immune-fecal occult blood test, gynecological consultation, and computed tomography (CT) of the chest, abdomen, and pelvis was performed. Impressively, CT revealed a huge mass in the left kidney and multiple nodules in the lung [Figure 1]d, suggesting a renal tumor with lung metastasis. Radical nephrectomy was arranged, and histopathology confirmed the diagnosis of clear cell renal cell carcinoma (RCC), T3aN1M1. The patient (body weight: 55 kg) was treated with intravenous methylprednisolone (60 mg/day), oral azathioprine (100 mg/day), and sunitinib (37.5 mg/day) as target therapy of stage IV RCC. Regular follow-ups of CBC showed no evidence of bone marrow toxicity caused by azathioprine. However, symptoms of painful skin lesions and bilateral thigh weakness persisted. Unfortunately, 3 months after the diagnosis of DM, she was readmitted due to acute-onset pneumonia and disturbed consciousness. The patient's condition deteriorated progressively and expired due to respiratory failure.
Regarding the association between DM and RCC, we could find only four similar case reports from PubMed [Table 1].,,, The average age was 60 years, with a gender ratio of 2:3 (male:female). Three out of four patients did not have any genitourinary symptoms. In all patients (five out of five cases), RCC had not been detected until a diagnosis of DM or polymyositis was made.
|Table 1: Summary of four similar cases reports of dermatomyositis with RCC and the present patient|
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In conclusion, we learned from the patient that, although rare, RCC should be kept in mind when surveying an internal malignancy in DM cases, even in those without urinary symptoms. The rapid mortality seen in our patient might be explained by the advanced stage of RCC, and vulnerability to infection caused by immunosuppressive therapy for DM. In the future, we hope there will be more studies about how to manage those DM patients with advanced malignancies.
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 her name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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