Table of contents
Localized scleroderma
What's new
Updated 2024 DDG/DGMKG guidelines for the diagnosis and management of localized scleroderma.
Background
Overview
Definition
LoS, also known as morphea, is a chronic autoimmune disorder characterized by inflammation and fibrosis of the skin and underlying tissues due to excessive collagen deposition.
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Pathophysiology
The pathophysiology of LoS involves an imbalance between T-helper cell subsets, with Th1 and Th17 cells driving inflammation in the early stages of the disease and Th2 cells contributing to fibrosis in the later stages.
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Epidemiology
The incidence of LoS is estimated at 0.4-2.7 per 100,000 person-years in the general population, and at 34 per 100,000 person-years in children.
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Risk factors
Risk factors for LoS are not well understood. However, it has been suggested that a combination of genetic predisposition and environmental triggers, such as infections or trauma, may contribute to the development of the disease.
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Disease course
Clinically, LoS presents with skin lesions that can be classified into various types, including plaque morphea and linear scleroderma. These lesions can lead to functional impairment and cosmetic disfigurement, particularly when they affect the extremities or the face. In some cases, extracutaneous manifestations such as arthritis, myositis, and uveitis may also be present.
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Prognosis and risk of recurrence
LoS can lead to significant morbidity, particularly in cases with severe skin involvement or musculoskeletal complications.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of localized scleroderma are prepared by our editorial team based on guidelines from the German Society of Dermatology (DDG/GKJR/DGMKG/SGDV/ÖGDV 2024), the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019), the Pediatric Rheumatology European Society (PReS 2018), and the European Dermatology Forum (EDF 2017).
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Classification and risk stratification
Classification
As per DDG/DGMKG/GKJR/ÖGDV/SGDV 2024 guidelines:
Classify LoS into the following main types:
limited
generalized
linear
mixed
B
Recognize that the following subtypes may also exist:
Situation
Guidance
Limited
Morphea (plaque type)
Guttate morphea (special type of morphea)
Atrophodermia Pierini-Pasini (special type of morphea)
Deep morphea
Generalized
Generalized LoS (involvement of at least 3 anatomical areas)
Disabling pansclerotic morphea (severe variant)
Linear
Linear LoS (mostly affecting the extremities)
Linear LoS, en coup de sabre type
Progressive facial hemiatrophy (Parry-Romberg syndrome)
Eosinophilic fasciitis (special type with predominant fascial involvement)
Mixed
Mixed phenotype
B
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Severity assessment
Diagnostic investigations
History and physical examination
As per DDG/DGMKG/GKJR/ÖGDV/SGDV 2024 guidelines:
Elicit targeted medical history and perform a physical examination for other autoimmune diseases and rheumatic conditions in patients with LoS.
B
Assess the anogenital region, especially in patients with morphea (plaque type) and generalized LoS, to screen for the presence of genital lichen sclerosus.
B
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Laboratory testing (baseline blood tests)
Laboratory testing (Lyme disease testing)
Laboratory testing (ENA)
Laboratory testing (systemic evaluation)
Diagnostic imaging
Diagnostic procedures
Medical management
Setting of care: as per DDG/DGMKG/GKJR/ÖGDV/SGDV 2024 guidelines, diagnose and manage LoS in collaboration with experts in LoS, including general and pediatric dermatologists and/or rheumatologists.
B
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Topical corticosteroids
Topical vitamin D analogs
Topical calcineurin inhibitors
DMARDs
Systemic corticosteroids
Nonpharmacologic interventions
Physical and manual therapy
As per DDG/DGMKG/GKJR/ÖGDV/SGDV 2024 guidelines:
Offer physical therapy and manual therapy in all subtypes of LoS with restricted mobility (joint contracture, muscle atrophy/hypotrophy) and for the prevention of joint contracture in cases of skin involvement spanning the joint.
B
Offer connective tissue massage and manual lymphatic drainage concurrently or following a therapy in the sclerotic stage.
B
Therapeutic procedures
Phototherapy: as per DDG/DGMKG/GKJR/ÖGDV/SGDV 2024 guidelines, offer medium-dose UVA1 phototherapy as first-line phototherapy in patients with limited subtypes of LoS.
B
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Laser therapy
Stem cell fat grafting
Surgical interventions
Indications for surgery
As per DDG/DGMKG/GKJR/ÖGDV/SGDV 2024 guidelines:
Consider performing functionally necessary surgical interventions for the linear type of LoS primarily during the inactive phase.
C
Consider performing plastic surgical interventions for linear LoS en coup de sabre or progressive facial hemiatrophy during the inactive phase.
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Specific circumstances
Pediatric patients, physical examination: as per SHARE initiative 2019 guidelines, obtain a careful evaluation with a complete joint examination, including the TMJ, at diagnosis and during follow-up in all patients with juvenile LoS.
B
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Pediatric patients (diagnostic imaging)
Pediatric patients (severity assessment)
Pediatric patients (setting of care)
Pediatric patients (topical therapy)
Pediatric patients (DMARDs)
Pediatric patients (systemic corticosteroids)
Pediatric patients (phototherapy)
Pediatric patients (clinical follow-up)