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Introduction and objectives: Perniosis manifests as inflammatory cutaneous lesions in patients exposed to nonfreezing weather during winter or early spring. Perniosis can be a primary disorder or a cutaneous presentation of a wide range of disorders. Methods: We present a 77-years-old woman with history of mastectomy for breast cancer, hypertension, atrial fibrillation and chronic obstructive pulmonary disease. She was a non-smoker and her medical familial history was unremarkable. She visited her family doctor because of severe cyanotic fingers, nose and ears, with painful and erythematous wounds, accompanied by edema and ulcerations. These were longstanding and very disabling symptoms, that revealed an intimate relation with cold exposure. No other identified triggering factors or accompanying signs/symptoms. The patient had already been treated with pentoxifylline, with a slight improvement. Patient was referenced to dermatology for further study. Blood-tests, including autoimmunity, complement, rheumatoid factor, lupus anticoagulant, VDRL, HIV, hepatitis C serologies, protein electrophoresis and thyroid function, were negative. Skin biopsy showed a lymphocytic inflammatory infiltrate in the dermis, without other relevant findings. Since the symptoms remain uncontrolled, the patient will start nifedipine. Results and conclusion: Diagnosis of perniosis is essentially clinical. Severe lesions like those found in our patient are very rare. Correlation with a wide range of extracutaneous diseases has been reported. The most recent studies suggest that sudden appearance of perniosis in adults should be carefully evaluated, as it may be a predictive sign of an associated systemic disease. Apparently, this is a case of chronic idiopathic perniosis in an elderly woman.