Papulonodular mucinosis with or without systemic lupus erythematosus: a retrospective study

Lupus erythematosus-associated skin disease - papulonodular mucinosis (PNM) is characterized by a diffuse deposition of mucin in the dermis. The relationship between PNM and lupus erythematosus and appropriate treatment is crucial for PNM patients.

To investigate the relationship between systemic lupus erythematosus (SLE) and PNM.

We collected clinicopathological, treatment and follow-up data of 13 patients with PNM of the Chinese Academy of Medical Sciences and Peking Union Medical College from 2004 to 2024. According to the diagnostic criteria for SLE, the patients were divided into two groups: the SLE group and non-SLE group who presented with PNM as major manifestations. The similarities and differences between the two groups were summarised and compared.

Thirteen patients included 8 (61.5%) males and 5 (38.5%) females, who presented with generalized (61.5%) or localized (38.5%) nodules/plaques with a mean onset age of 39.8 years. Among them, 4 (33.3%) cases accorded with the diagnostic criteria for SLE. As to treatment, the SLE group adopted the regimen of systemic prednisolone, hydroxychloroquine and intralesional triamcinolone acetonide. The rest of patients all received systemic prednisolone, together with some other adjuvant therapy. They all gained improvement of varying degree and to date, no SLE symptoms have developed in 11 patients, while 2 patients have developed arthralgia, oral ulcer and alopecia.

Although the clinical presentation varies, the SLE and non-SLE groups of PNM have similar histological features and outcomes, suggesting that these two situations are highly associated.