Chronic urticaria as the first sign of sarcoidosis

Case

A 34-year-old man was followed in our department for chronic urticaria that responded poorly to 6 months of antihistamine treatment (Figure 1). During the last 2 months of antihistamine treatment, the patient had nonspecific constitutional symptoms including fever, weight loss, fatigue, dyspnea, and dry cough. Skin-prick tests were done with standard aeroallergens and foods. Laboratory tests (including measurement of angiotensin-converting enzyme [ACE] level), lung function studies, a tuberculin test, a chest x-ray scan, a computed tomography scan, and a lung biopsy were performed.

Skin-prick tests were performed using the most common aeroallergens in our epidemiologic area, including molds (Alternaria, Aspergillus, Cladosporium, and Penicillium spp); negative results were obtained for all tests. The tuberculin test also had a negative result. Lung function tests revealed a mixed disease process with no response to a bronchodilator and a moderate decline in diffusing capacity. Chest x-ray and thoracic computed tomography scans showed mediastinal and hilar lymphadenopathy, as well as diffuse and bilateral reticulonodular infiltration (Figure 2). A trans-bronchial lung biopsy revealed noncaseating granulomas. Bacterial and mycobacterial cultures had negative results. For the sputum tests, bacterial culture did not have any conclusive results, and potassium hydroxide fungal staining did not reveal any hyphae from filamentous fungi. Blood tests showed an ACE level of 141 μg/L.

The changes in the lungs seen on the chest x-ray scan were consistent with stage 2 sarcoidosis. Figure 3 is a chest x-ray scan taken after 6 months of corticosteroid treatment; it shows partial improvement of the pulmonary infiltrates. After 12 months of tapered-dose treatment with oral corticosteroids, the patient had no cutaneous or respiratory symptoms, and had a normal ACE level. Of interest, 3 weeks after the end of corticosteroid treatment, the patient had increasing cough and intensification of dyspnea, which prompted re-admission to hospital owing to respiratory insufficiency and a diffuse nodular interstitial pattern on chest x-ray scan. His ACE level had increased 3-fold from the previous measurement, and cutaneous manifestations were absent.

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Figure 1.

Skin lesions and chest x-ray scan with negative results at first presentation with urticaria

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Figure 2.

Chest x-ray and computed tomography scans after 6 months of unsuccessful antihistamine treatment for urticaria: Scans show mediastinal and hilar lymphadenopathy, as well as diffuse and bilateral reticulonodular infiltration.

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Figure 3.

Chest x-ray scan after 6 months of treatment with oral corticosteroids showing partial improvement of pulmonary infiltrates