Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts

• DOI: 10.3389/fimmu.2021.656407
• Publication Date: 2021-03-09T06:17:11Z
• Authors: Sarah Ventéjou; Agnes Schwieger-B...; Rebecca Nicolai; Stephanie Christe...; Caroline Schnider; Michael Hofer; Sofia Bogiatzi; Daniel Hohl; Fabrizio De Bened...; Marie-Anne Morren
• Keywords: 2403 Immunology; pansclerotic morphea; IL-6; Immunology; stiff skin; 610 Medicine & health; RC581-607; 3. Good health; IL-6; case report; pansclerotic morphea; scleroderma; stiff skin; tocilizumab; tocilizumab; 03 medical and health sciences; 0302 clinical medicine; 2723 Immunology and Allergy; Immunology and Allergy; case report; scleroderma; 10220 Clinic for Surgery; Immunologic diseases. Allergy

Pansclerotic morphea (PSM) is a rare skin disease characterized by progressive stiffening of the skin with or without the typical superficial skin changes usually seen in morphea (localized scleroderma). Standard therapy, consisting of a combination of systemic glucocorticoids and methotrexate or mycophenolate mofetil, does rarely stop disease progression, which may lead to severe cutaneous sclerosis and secondary contractures. Little is known about the efficacy of newer biologicals such as abatacept, a fusion protein antibody against CTLA-4, or tocilizumab, a fully humanized IL-6R antibody, in the treatment of this pathology. We present the case of an 8 years old girl with an unusual, progressive stiffening of the skin, which was eventually diagnosed as pansclerotic morphea. A treatment with systemic glucocorticoids and methotrexate combined with tocilizumab led to a good clinical response within 2 months after initiation. In this paper, we discuss differential diagnoses to be considered and this new promising treatment option based on a case review of the literature.