Dermatomyositis associated with malignancy: A report of 3 cases
A 36-year-old male presented with complaints of itchy red lesions over the periorbital area, forehead and malar area associated with photosensitivity since 3 months, painless swelling around both eyes since 1 month and pain over the shoulder and hip region, nasal twang of voice and difficulty in deglutition since 2 days. Cutaneous examination of the patient revealed periorbital erythema, edema and hyperpigmentation [Heliotrope rash, Figure and ]. Similar erythema and hyperpigmentation were also present on the neck, chest, shoulders and back [Shawl sign, [Figure and ]. Erythematous hyperpigmented papules were present on the knuckles and both elbows [Gottron's papules, Figure and ]. Musculoskeletal examination revealed proximal muscle weakness of shoulder and hip girdles. Skin biopsy from the erythematous lesion on the forehead revealed atrophic epidermis, focal basal cell vacuolar degeneration, dermal edema, mild perivascular lymphocytic infiltrate and abundant mucin in the dermis [Figure and ]. Blood investigations of the patient revealed raised erythrocyte sedimentation rate (ESR), elevated titers of muscle enzymes (LDH and CPK-MM) and electromyography (EMG) showing first-degree muscle disease, confirming the diagnosis of dermatomyositis. Rhinolaryngoscopy of the patient revealed articular defects of vocal cords due to bulbar muscle palsy [detailed investigations are mentioned in Tables and ]. The patient was simultaneously investigated for underlying malignancy. Barium swallow of the patient showed esophageal motility disorder, computed tomography (CT) scan of the abdomen revealed thickening in the pyloric region, causing mild luminal compromise, esophago-gastro-duodenoscopy (OGDscopy) showed presence of gastric ulcer at the pyloric antrum and biopsy from the gastric ulcer confirmed the diagnosis of poorly differentiated adenocarcinoma of the stomach [Figure and ]. The patient was diagnosed as DMS associated with poorly differentiated gastric adenocarcinoma. The patient received oral prednisolone 1.5 mg/kg/day with low potent topical corticosteroids and sunscreen. The patient's skin lesions and muscle symptoms showed excellent response [Figure –] to the treatment with reduction in the titers of muscle enzymes []. After clinical improvement, prednisolone was tapered gradually and the patient was referred to an oncologist for management of gastric adenocarcinoma. Unfortunately, the patient was lost to follow-up.