Update of the guidelines for sclerosing skin diseases

The present consensus on sclerosing diseases of the skin provides an update to the 2017 European Dermatology Forum Guidelines on scleroderma.1, 2 Scleroderma or sclerosing diseases of the skin encompasses a variety of diseases and clinical subtypes. This consensus is focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes. Part 2 of this consensus provides an additional overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke). As the diseases described in this consensus are rare, the current update of the previous guidelines will be very important for the treating dermatologist. One of the most important challenges regarding patients with sclerosing skin diseases is to distinguish timely and accurately the various manifestations of scleroderma and to install the best effective therapy for the patient. An important issue is not only to establish the superficial extent of skin involvement but also the degree of involvement of deeper structures such as subcutaneous tissue, fascia and muscle. When diagnosed in an early stage, the possibility of preventing extension of the skin lesions and irreversible damage is still present. If treated late, patients can be confronted with loss of function such as developing contractures or atrophy, especially in the severe subtypes. Collaboration with other specialists such as rheumatologists, paediatricians, ophthalmologists and neurologists will often be important depending on the subtype of sclerosing disease. The well-known group of authors has succeeded again in providing a beautiful and clear update of the available body of evidence regarding diagnostic and therapeutic possibilities of sclerosing skin diseases combined with the consensus of experts. Especially the flow chart addressing the treatment of localized scleroderma subtypes with limited skin involvement (reaching to the dermis vs.) versus subtypes with severe skin and/or musculoskeletal involvement (affecting fat tissue, fascia, muscle, joints and bones, or widespread skin involvement), in adults as well as children will be very helpful for treating physicians. Clear recommendations are presented in each subchapter of the consensus, which facilitates the use of the consensus in daily practice of dermatology. The paucity of new evidence from randomized controlled trials and the scarcity of new treatment modalities designed for localized scleroderma and systemic sclerosis is unfortunate. The same holds true for scleromyxoedema and scleroedema, where the available data consist primarily of case reports and case series. The authors rightly advocate that more randomized controlled trials should be performed in the future to identify the best treatment regimens, optimum dose and long-term efficacy of the therapy. There is however a small silver lining for immune-mediated sclerosing skin diseases. Efficacy of imatinib, an oral tyrosine kinase inhibitor was described in systemic sclerosis in a recent systematic review and meta-analysis, where a significant change was shown within the range of clinical relevance of the modified Rodnan Skin Score (mRSS).3 In addition, recent advantages have been shown for chronic graft versus host disease, a disease that shares similarities with the above described sclerosing skin diseases, for which ruxolitinib (Jakavi), an oral tyrosine kinase inhibitor which effect is exerted via the JAK/STAT pathway, has been registered since 2022.4 In addition, ruxolitinib cream (Opzelura) has been registered for vitiligo and is currently investigated in clinical trials for various skin diseases such as hidradenitis suppurativa and atopic dermatitis.5 This might be the prelude to the introduction of more effective treatment options for patients with immune-mediated sclerosing skin disease. The author has no conflict of interest to declare. Data sharing not applicable to this article as no datasets were generated or analysed during the current study.