Systemic sclerosis with keloidal nodules
A 44 years old female patient of afro-descendant origin seeks medical help with a 12\nyears history of Raynaud's phenomenon associated with painful digital ulcers. Six years\nago she developed arthralgia in both hands, alopecia and dyspnea on exertion. One year\nago she noted skin lesions "similar to keloid", of approximately 2 to 3 cm in trunk,\nback and neck that gradually increased in number. Prior treatment with local\ncorticosteroid injections was done without any improvement.\nOn physical examination she had microstomia, narrowing of hand fingertips with small\nscars and an active ulcer in 2nd right finger of 5 mm. Skin thickness was\nseen in skin of the hands forearms and face. In the regions of the neck, trunk and back\nshe had numerous nodular lesions, 2 to 3 cm in diameter, not painful (). Heart, lung and abdomen examinations were\nnormal. A periungueal capillaroscopy was performed and showed a SD pattern (SD=systemic\nsclerosis pattern). Radiographs of hands, echocardiography, barium swallow and CT of the\nchest were normal. A respiratory function test showed restrictive ventilatory defect.\nLaboratory showed a positive ANA (1/640; with fine speckled pattern) and negatives tests\nfor antiScl-70 and anti-centromere. Sedimentation rate, C reactive protein, complete\nblood count, liver and kidney function tests were normal.\nThe patient underwent biopsy of two nodular lesions in the chest, which findings are\nshown at and . In and , it can be seen that the epidermis presents with\nacanthosis and hyperpigmentation at basal layer. Superficial dermis is preserved and\nreticular dermis has increased thickness and sclerosis of collagen bundles. Collagen\nfibers exhibit parallel distribution. There is, also, a discrete perivascular\ninflammatory process. The interstices between collagen fibers is decreased and there is\nentrapment of eccrine glands and nerves. ( and ). In the elastic fibers (darker) can be seen. They do not exist in\nkeloids.\nBased on clinical and pathological report was diagnosed with scleroderma, in its variant\nnodular/keloid.\nTreatment was started with nifedipine 20 mg/day and aspirin 100 mg/day for Raynaud's\nphenomenon. To treat scleroderma nodular lesions we added systemic corticosteroids, oral\nmethotrexate and phototherapy (PUVA).