Anti-RO 52-positive systemic sclerosis sine scleroderma with multisystem involvement and recurrent vasculitis

Abstract

The atrophic cutaneous lesions with shiny surface prompted us to consider the diagnosis of Dego’s disease, but the absence of clinical and imagological evidence of involvement of gastrointestinal and central nervous system could not support the diagnosis. [...]the absence of wedge-shaped connective tissue necrosis and lack of mucin deposition on histopathological examination characteristic of Dego’s disease could not substantiate the diagnosis. Interestingly, in a study by Poormoghim et al., 18 cases grouped as systemic sclerosis sine scleroderma, subsequently, developed sclerodactyly during the follow-up period of 3.9 years. [...]they consider systemic sclerosis sine scleroderma to be part of the continuance of limited systemic sclerosis rather than a separate entity. [...]a Brazilian study on systemic sclerosis sine scleroderma reported pulmonary involvement in 63.2% of cases. [...]the same study revealed esophageal involvement in 83.1% of cases.