Selma ABDELLAOUI, Merzak GHARNAOUT, Sonia NOUIOUA
Rheumatology Quarterly - 2026;4(2):124-127
Aim: In this case report, we describe a severe acrosyndrome occurring in a young girl, leading to major functional impairment and significant cosmetic sequelae. We report the case of an 18 year old young woman whose quality of life has been severely impaired since 2023. Her initial symptoms consisted of a burning sensation and heat in the distal parts of the lower limbs, occurring in the absence of any trauma. Soon after immersing her feet in ice water, she developed rapid onset erythema and edema. Despite multiple topical treatments, her condition progressed, and she underwent a skin graft, which failed to achieve satisfactory healing. Angio-tomoscintigraphy revealed an active bilateral osteoblastic pattern with significant bone and soft tissue involvement, as well as joint space alterations compatible with complex regional pain syndrome (algodystrophy). Conversely, lymphoscintigraphy showed moderate lymphatic insufficiency. In the absence of effective corrective management, her clinical condition continued to worsen, raising significant diagnostic and therapeutic challenges. Two years later, the presentation was suggestive of a critical, permanent idiopathic erythromelalgia, associated with perilesional dyspigmentation following grafting, leading to severe cosmetic disfigurement, discontinuation of physical activity, and a profound psychological impact. This severe and progressive acrosyndrome required extensive etiological investigation and posed a major diagnostic and therapeutic challenge. Early recognition is essential in order to limit trophic complications and improve functional and cosmetic outcomes.
