Dermatomyositis as presenting feature of ovarian cancer, treated with neo-adjuvant chemotherapy and interval debulking surgery
A 57 year old Caucasian lady presented to her General Practitioner with a pruritic rash on her anterior chest, upper arms, hands and face. Two weeks later proximal myalgia and stiffness of all four limbs developed and a dermatology opinion was sought. On physical examination there was an erythematous maculopapular rash affecting the face, trunk and upper limbs which was more prominent in photo-exposed areas, with flat-topped scaly lesions on the dorsum of both hands and wrists thought likely to be Gottron's papules. Serum creatine kinase (CK) was elevated at 3012 IU/L (n = 30–135).\nMedical history included hypothyroidism, hypertension, osteoporosis, depression and previous lumbar discectomy for degenerative disease. She was prescribed levothyroxine, fluoxetine, amitriptyline, calcium carbonate, alendronic acid, lisinopril, paracetamol and omeprazole. There was a history of ovarian cancer in the patient's mother.\nDermatomyositis was suspected and a skin biopsy was performed under immunofluorescence. The skin biopsy revealed a vacuolar interface dermatitis consistent with a collagen vascular disease or dermatomyositis [].\nOne week later the patient was admitted to hospital with progressive proximal muscle weakness. CK had risen to 11,608 and erythrocyte sedimentation rate was elevated at 46. Temperature was elevated at 38.2 °C. Physical examination revealed a rash affecting her chest, back and upper arms (shawl distribution), muscle weakness predominantly affecting hip flexion and shoulder abduction, and a palpable mass in the right iliac fossa.\nPrednisolone 40 mg was commenced with a dose increase to 60 mg after 10 days due to lack of response. CK fell to 2279 but without any symptomatic improvement and she remained bedbound in hospital.\nCT scan of chest, abdomen and pelvis revealed a 9.5 cm × 8.8 cm lobulated, cystic adnexal mass with multiple peritoneal deposits. Biopsy of the pelvic mass was consistent with a high grade serous carcinoma of ovarian or primary peritoneal origin.\nNeo-adjuvant chemotherapy with Carboplatin AUC-6 and Paclitaxel 175 mg/m2 was commenced as an in-patient 1 month after presentation. Ca125 fell from 2231 KU/L to 172 after 3 cycles of chemotherapy, all given as an in-patient. CK rose to 3256 during week 1 of chemotherapy but was within normal range at 51 by cycle 3. Muscle weakness improved during chemotherapy and she was mobilising independently on the ward after 2 cycles. A relapse of her photosensitive rash responded to hydroxychloroquine. Prednisolone was gradually reduced to a dose of 30 mg.\nCT scan after 3 cycles showed a good partial response to treatment and a fourth cycle of neo-adjuvant chemotherapy was given as an out-patient.\nShe proceeded to a laparotomy, anterior resection of the rectum with re-anastomosis, bilateral salpingo-oophorectomy, hysterectomy and omentectomy without intra-operative complications. Post-operatively, a flare of the rash affecting her shoulders, back, elbows and knees, and subsequent mild proximal muscle weakness, responded after increasing prednisolone to 40 mg.\nFollowing cytoreductive surgery, a 1 cm peritoneal deposit remained in an inaccessible area beneath the right hemi-diaphragm. Her post-surgery CT scan 6 weeks later identified diffuse low volume peritoneal disease in the right and left paracolic gutters. Two further cycles of post-operative carboplatin and paclitaxel were given, with a further response but persistent low volume peritoneal disease on her end of treatment CT, and Ca125 of 54. No dose reductions or delays were required during chemotherapy and the only grade 2 toxicity experienced was alopecia. Prednisolone was gradually reduced to a maintenance dose of 10 mg.\nThe patient experienced exacerbation of her skin rash 3 months after completion of chemotherapy, but remained asymptomatic of her ovarian cancer. There was a rise in Ca125 and CK, and restaging CT scan revealed new low volume ascites and an increased volume of peritoneal disease. There was a subsequent relapse of proximal muscle weakness. Prednisolone was increased to 40 mg, with an improvement of the skin rash and fall in CK, although muscle weakness persisted.\nChemotherapy for relapsed disease with weekly carboplatin and paclitaxel and 3-weekly bevacizumab was commenced 5 months after completion of her post-operative chemotherapy. Bevacizumab was stopped after the first cycle due to haematuria. Ca125 levels were rising after 4 months of second-line chemotherapy, with disease progression confirmed on CT. Although CK levels continued to fall, disease progression coincided with worsening proximal muscle weakness and new difficulties swallowing.\nChemotherapy was stopped and the patient died from progressive disease 18 months after initial presentation.