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Localized scleroderma — Clinical Guidelines

Overview

Localized scleroderma (LoS) encompasses a spectrum of sclerotic skin diseases affecting skin and potentially adjacent structures like muscles, joints, and bones, without systemic involvement or progression to systemic sclerosis 1 3.

Diagnosis

Diagnostic Criteria: Established criteria based on clinical presentation and histopathology 2.

Recommended Tests: Laboratory workup, histopathology, and imaging studies (e.g., MRI) to assess involvement of adjacent structures 3.

Severity Classification: Clinical scoring systems to grade disease severity 3.

Management

First-Line Treatments:

- Limited Skin Involvement: Topical corticosteroids 1. - Mild to Moderate Cases: UV therapy can be considered 1.

Adjunctive Treatments:

- Severe Skin or Musculoskeletal Involvement: Systemic methotrexate 1. - Active Phase: Systemic glucocorticosteroids may be used additionally 1.

Second-Line Therapies:

- Refractory Cases: Mycophenolate mofetil, mycophenolic acid, or abatacept 1. - Linear LoS: Autologous adipose-derived stem cell transplantation for soft tissue defects 1.

Special Populations

Pediatrics: Methotrexate and corticosteroids are commonly used, with intensified treatment for facial lesions or recent onset (<6 months) 5.

Misdiagnosis: Often delayed, frequently misdiagnosed initially as atopic eczema, fungal infections, or other conditions; early referral to specialists is crucial 4.

Key Recommendations

Use Topical Corticosteroids for Limited Skin Involvement (Evidence: Strong 1).

Consider Systemic Methotrexate for Severe Cases (Evidence: Strong 1).

Intensify Treatment for Facial Lesions or Recent Onset in Pediatric Patients (Evidence: Moderate 5).

Early Specialist Referral to Avoid Diagnostic Delays (Evidence: Weak 4).

Explore Second-Line Therapies for Refractory Cases (Evidence: Expert opinion 1).

References

1 Kreuter A, Moinzadeh P, Kinberger M, Horneff G, Worm M, Werner RN et al.. S2k guideline: Diagnosis and therapy of localized scleroderma. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2024. link 2 Asano Y, Fujimoto M, Ishikawa O, Sato S, Jinnin M, Takehara K et al.. Diagnostic criteria, severity classification and guidelines of localized scleroderma. The Journal of dermatology 2018. link 3 Kreuter A, Krieg T, Worm M, Wenzel J, Moinzadeh P, Kuhn A et al.. German guidelines for the diagnosis and therapy of localized scleroderma. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2016. link 4 Weibel L, Laguda B, Atherton D, Harper JI. Misdiagnosis and delay in referral of children with localized scleroderma. The British journal of dermatology 2011. link 5 Li SC, Feldman BM, Higgins GC, Haines KA, Punaro MG, O'Neil KM. Treatment of pediatric localized scleroderma: results of a survey of North American pediatric rheumatologists. The Journal of rheumatology 2010. link 6 Kuto F, Sakaguchi T, Horasawa Y, Hayashi M, Hirasawa Y, Tokuhiro H. Total hemiatrophy. Association with localized scleroderma, Schönlein-Henoch nephritis, and paroxysmal nocturnal hemoglobinuria. Archives of internal medicine 1985. link

Localized scleroderma