Panniculitis as Initial Manifestation of Sarcoidosis

Abstract

BACKGROUNDSarcoidosis is a disease of unknown etiology characterized by the formation of granulomas in various organs. Systemic symptoms such fatigue and weight loss are common. In more than 90% of patients, clinical sarcoidosis is manifested as intrathoracic lymphnode enlargement, pulmonary involvement, skin or ocular signs and symptoms. Approximately, 50 to 65% of patients with sarcoidosis have granulomas on liver biopsy, but only 5 to 15% are symptomatic. Skin involvement is common, occurring in 25 to 35% of patients with sarcoidosis, and includes papular sarcoidosis, lupus pernio, subcutaneous nodules, erythema nodosum and many others skin manifestations. However, panniculitis as an initial presentation is rarely reported. The diagnosis of sarcoidosis requires a compatible clinical picture, the demonstration of noncaseating granulomas on tissue biopsy, and the exclusion of other disorders. Treatment for cutaneous sarcoidosis includes glucocorticoids, methotrexate, hydroxychloroquine and tetracyclines. Tumor necrosis factor alpha inhibitors are indicated in refractory disease. CASE REPORTA 51-year-old female patient with a previous history of obesity, hepatic steatosis, dyslipidemia, and hypertension, reported the appearance of painful subcutaneous nodules, distributed in upper and lower limbs, 2 years ago. In addition, she reported proximal muscle weakness, fever, arthralgias, dyspeptic symptoms, dysphagia for solids, and flank abdominal pain. She also reported coughing, recurrent oral ulcers, bleeding gums, and tooth loss. On physical examination, she had hyperchromic and painful nodular lesions, diffusely distributed over the lower and upper limbs. During hospitalization, laboratory tests showed increased evidence of inflammatory activity and cholestasis, and liver biopsy was indicated. Computed tomography of the chest and abdomen showed multiple pulmonary nodules and mediastinal, hilar, and abdominal lymph node enlargement. Skin and liver biopsies were performed, with the finding of septal panniculitis and non-caseating granulomatous hepatitis, respectively. Clinical, imaging and anatomopathological findings were compatible with the diagnosis of sarcoidosis. She started treatment with prednisone 40 mg/day and methotrexate 10 mg/week during hospitalization, with improvement of the skin lesions. CONCLUSIONWe report a case of sarcoidosis with initial presentation of panniculitis in a 51-year-old female patient.