Juvenile primary Sjogren's syndrome with cutaneous involvement

Objective: This study aims to analyze the clinical characteristics of juvenile primary Sjogren's syndrome (pSS) with cutaneous involvement.

Methods: We investigated the clinical and immunological characteristics of 37 children with pSS. All the patients met the 2012 American College of Rheumatology Classification Criteria for Sjogren's syndrome.

Results: The cutaneous involvement presented in 19 children (excluding drug eruption), 16 (84.2%) female patients, and 3 (15.8%) male patients, with a mean age of 11 ± 2.68 years, 17 of whom (89.5%) had cutaneous lesions as the first symptom, with a median time of 12 months (1 day to 4 years) before the diagnosis of pSS. The cutaneous lesions included 12 cases of palpable purpura (63.2%), 5 cases of urticaria (26.3%), 2 cases of xeroderma (10.5%), 1 case of skin ulcer, 1 case of erythema nodosum, 1 case of livedo reticularis, 1 case of Raynaud's phenomenon, and 1 case of hard erythema. Children with cutaneous lesions had a higher prevalence of articular involvement (42.1% vs. 11.1%, P = 0.016), fever (47.4% vs. 5.6%, P = 0.004), ESR > 50 mm/h (47.4% vs. 11.1%, P = 0.016), and a lower prevalence of thrombocytopenia (0% vs. 27.8%, P = 0.013) and methylprednisolone pulse treatment (0% vs. 13.5%, P = 0.013), compared with pSS without cutaneous involvement.

Conclusion: More than half (51.3%) of the children with juvenile pSS presented with cutaneous lesions; the main cutaneous involvement was palpable purpura. Children with cutaneous lesions were more likely to have fever and arthritis, were more likely to have stronger inflammatory response, and were less likely to have serious complications. In many cases, the cutaneous lesion could be the first symptom of juvenile pSS, which could easily lead to a misdiagnosis. The possibility of pSS should be considered for children with skin lesions such as palpable purpura and urticaria, and further examinations should be carried out. Key Points • Cutaneous lesions of juvenile pSS are not uncommon and often present as the first symptom. • Palpable purpura was the most common skin lesion in juvenile pSS, followed by urticaria. • Juvenile pSS with skin lesions does not increase the risk of serious complications such as blood system damage. • Skin lesions in juvenile pSS patients may easily cause misdiagnosis.