Sarcoidosis in Children: Differentiation from Juvenile Rheumatoid Arthritis

Abstract

Sarcoidosis is rare in young children, and is characterized by skin, joint, and eye changes. Differentiated clinically from juvenile rheumatoid arthritis (JRA) by milder constitutional symptoms and characteristic joint abnormalities, sarcoidosis is confirmed by demonstrating noncaseating granulomas in skin, conjunctival, or synovial biopsies. Recent reports have shown children with features of both sarcoidosis and juvenile rheumatoid arthritis, some with similarly affected family members. We cared for four children with sarcoidosis and severe joint manifestations. Two had a personal or family history of JRA. Three of the four children had ichthyosiform cutaneous manifestations, which may suggest an association between severe joint disease and ichthyosiform changes. Because of the difficulty in making a diagnosis on clinical grounds alone, biopsy of cutaneous lesions is recommended in children with these symptoms.

Widespread Granulomatous Dermatitis of Infancy

“…12,13 Of note, the latter can also occur in the setting of long-standing joint disease due to sporadic EOS. 3,4,14 The age at onset, organ systems involved, and severity of BS vary substantially, even within affected families. 12 The disease becomes evident at a median age of 2 years (range, 4 months to 27 years).…”

“…52 They tend to be arranged in clusters or linear arrays but can become confluent, and a perifollicular distribution may be evident. 3,4,14,24,27,32,53,54 Intermittent episodes of widespread skin disease with spontaneous resolution usually occur during a period of years (in contrast to the more persistent skin lesions of sarcoidosis). Poikiloderma has been observed in older patients, 13,27 and pitted scars due to follicular atrophoderma are sometimes apparent at sites of previous inflammatory papules.…”

“…2,3,27,[54][55][56][57] Ichthyosiform skin changes characterized histologically by a hyperkeratotic epidermis with underlying dermal granulomas have also been described in EOS/BS. 2,4,27,30,34,55 Histologically, the naked noncaseating granulomas of EOS/BS involve primarily the upper dermis and often have a perifollicular distribution. 24,54 This histologic pattern is identical to that of lichen scrofulosorum, a tuberculid that is usually seen in children with tuberculosis that involves the lymph nodes and bones.…”

“…Because the granulomatous inflammation is prone to relapse on medication withdrawal, steroid-sparing agents such as tumor necrosis factor inhibitors (eg, infliximab), methotrexate, cyclosporine, and other immunosuppressive medications may be required. 4,14,32,35,36,38 Immunomodulatory agents that target IL-1 (a proinflammatory cytokine that is up-regulated by NF-B) may also have therapeutic potential in EOS/BS. 35 In our patient, azithromycin (which has anti-inflammatory properties that include inhibition of tumor necrosis factor ␣ production) was beneficial for the skin lesions but not the arthritis.…”

A case of ichthyosiform sarcoidosis with unusual localization

“…In addition, in children, juvenile rheumatoid arthritis must be considered in the clinical differential diagnosis for acquired ichthyosis [7,8].…”