593 Chronic Urticaria as First Sign of Sarcoidosis

Abstract

BackgroundSarcoidosis is a granulomatous multisystemic disease of unclear etiology, which can affect any organ. The cutaneous manifestations are present in 20% to 35% of patients. Cutaneous lesions have been classified as specific and nonspecific, depending on the presence of noncaseating granulomas on histologic studies. Specific lesions include maculopapules, plaques, nodules, lupus pernio, scar infiltration, alopecia, ulcerative lesions, and hypopigmentation among others. The most common nonspecific lesion is erythema nodosum. Others include calcifications, prurigo, erythema multiforme, nail clubbing, and sweet syndrome. Urticaria does not belong to nonspecific nor specific lesions of this illness. Diagnosis is based on 3 criteria: a compatible clinical and/or radiological picture, histological evidence of noncaseating granulomas, and exclusion of other diseases. There is no standarized therapy but corticosteroids are the mainstay of treatment for sarcoidosis. We report a case of undetermined chronic urticaria which after 6 months displayed compatible symptoms with respiratory disease associated with systemic involvement.MethodsA 34-year-old man followed in our department due to a chronic urticaria, during 6 months with no good response to anti-histaminic treatment; refers non specific constitutional symptoms, including fever, weight loss, fatigue dyspnea and dry cough for 2 months. Skin prick test with standard aeroallergens and foods are done. Laboratory test (incluiding ECA levels), functional study; tuberculine test, chest x-ray, CT scan and lung biopsy were performed.ResultsSkin prick test and tuberculine test were negatives. Angiotensin-converting enzyme was high (ECA: 141). We observed a mixed process with negative bronchodilator response in pulmonary function test and a moderate disminution in diffusing capacity. Difuse and bilateral reticulo-nodular infiltration with mediastinal and hiliar lymphadenopathy was observed in X-chest. Lung biopsy: Noncaseating granulomas were observed. The diagnosis was Sarcoidosis, stage II. The patient remains asymptomatic regarding cutaneous and respiratory symptoms, after fulfilling oral corticoiteroids treatment for sarcoidosis.ConclusionsChronic urticaria can be the tip of an iceberg indicating more changes in other organs. The importance of considering cutaneous sarcoidosis in the clinical differential diagnosis of an urticaria relies on the association with systemic involvement, partial treatment response and the convenience of the skin as a tissue source for histologic analysis.