1.2.3 Localized scleroderma

Grading & Level of Importance: C

Review:

2026

W. Burgdorf, Munich; J. McGrath, London
Revised by G. Girolomoni, Verona; P. Gisondi, Verona; L. Martos-Cabrera, Madrid; M. Maurelli, Verona

Overview:

ICD-11
Synonyms
Epidemiology
Definition
Aetiology & Pathogenesis
Signs & Symptoms
Localisation
Classification
Clinical presentation
Laboratory & other workups
Dermatopathology
Course
Complications
Diagnosis
Differential Diagnosis
Prevention & Therapy
Special

ICD-11

EB61.0

Synonyms

Morphea, scleroderma circumscriptum.

Epidemiology

Where present, most frequently between 20-40 years, 15%

Localized scleroderma is most common between 20-40 years, with a prevalence of 500 and 2200 per million at ages of 18 and 80 years. The F:M = 2.6:1.

Definition

Uncommon localized connective tissue disease with inflammatory border (lilac ring).

Localized inflammatory connective tissue disease of the skin and subcutaneous fat that leads to a scar- like sclerosis.

Aetiology & Pathogenesis

Unknown. In some cases tick bite induction proven.

Localized scleroderma is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Although the etiopathogenesis of localized scleroderma is not fully understood, it is characterized by vascular damage, infiltration of activated T-cells, and by an altered production of connective tissue produced by fibroblasts. Vascular changes are characterized by a reduction of the number of capillaries and hypoxia caused by microvascular injury, and by the endothelial cell activation. T-cells, which are present in the perivascular sites, have the capacity to modify collagen synthesis by fibroblasts, so they contribute to develop sclerosis. Th2 cytokines such as IL-4 and IL-13 directly enhance TGF-β production, and this in turn enhances the production of the pathologic collagen by fibroblasts and induces the recruitment of eosinophils.

Triggering factors include mechanical trauma (including injections), infections (Borrelia burgdoferi), drugs and irradiation by X-rays.

Signs & Symptoms

Centrifugally spreading erythema with progressive central white-yellow induration with loss of adnexal structures. Border often with purple tones (lilac ring) as sign of disease activity.

Severe course with involvement of deeper structures possible, especially with linear scleroderma ("en coup de sabre" on scalp), facial hemiatrophy.

Localisation

Varies with classification.

Classification

1. Limited forms: plaque-type morphea, guttate morphea, atrophoderma of Pierini-Pasini
2.Generalized forms: generalized circumscribed scleroderma, disabling pansclerotic morphea, eosinophilic fasciitis
3. Linear forms: linear circumscribed scleroderma including type "en coup de sabre", Parry-Romberg syndrome
4. Deep morphea (see www.AWMF guidelines).

Clinical presentation

Localized scleroderma can be distinguished into subgroups: plaque-type morphea, linear morphea, generalized morphea, and the less common variants that include the deep, the guttate and the nodular forms.

The plaque-type morphea is the most prevalent form, and is characterized by slightly edematous, erythematous plaques with a centrifugal expansion (lilac ring), usually localized asymmetrically at the trunk, 2-15 cm of diameter. The lesions can be solitary or multiple, are usually asymptomatic, but in some cases are itchy, usually in the case of sclerosis. At the early stages, the central area of the lesions takes a shiny white colour and shows a sclerotic and scar-like tissue appearance, becoming progressively indurated. The lilac ring is a sign of disease activity. A white sclerosis with a post-inflammatory brown hyperpigmentation characterizes the late lesions. The adnexal structures, such as hairs follicle and sweat glands are usually absent.
Linear scleroderma is typically the ‘en coup de sabre’ form, with a longitudinal scar-like whitish band localized on the forehead and the frontal scalp. It usually starts either as a linear streak, which may at the early stages mimic a port-wine stain, or as a row of small plaques that tend to coalesce. It may begin as plaque-morphea but extends longitudinally and may involve the scalp (cicatricial alopecia), eyebrows, nose, and cheeks. Sclerosis is associated with a progressive loss of subcutaneous fat. The less frequent forms are hemifacial atrophy, called Parry-Romberg syndrome, which is a severe variant of linear morphea with involvement of subcutaneous fat and the underlying bone giving rise to facial disfiguration. The entire distribution at the trigeminal nerve can be affected. Linear morphea may also affect the extremities (arms or legs) presenting as linear sclerotic band with hyper- and hypo-pigmentation. This form tends to involve the underlying fascia, muscles, and tendons, and may be associated with hypoplasia and impaired mobility of the affected joints, and muscle weakness.
The generalized forms of localized scleroderma include: generalized circumscribed scleroderma, disabling pansclerotic morphea, and the eosinophilic fasciitis. These forms are characterized by involvement of the trunk at the early stages, but the distinctive feature is that this form does not stop his expansion. Usually, the plaques tend to coalescence rapidly, resulting in a disabling thoracic constriction and inflammation of intercostal muscle with breathing difficulty.
The variants include guttate morphea that presents as multiple, rather superficial, and nummular plaques. Usually, they are small and become deeply sclerotic. The atrophoderma of Pasini and Pierini is considered as a very superficial variant of plaque-type morphea and is also considered a separate entity in the differential diagnosis of ‘burnt-out’ morphea. The hyperpigmented patches are usually present at the posterior trunk, and occasionally they follow the lines of Blaschko. Deep morphea is characterized by a small number of hard and deep plaques with inflammation and sclerosis that involves primarily the deep dermis and subcutaneous fat, and sometimes the underlying structures. These plaques may impair the motility of the skin and tend to calcify, leading to dystrophic calcinosis cutis. The nodular/keloidal morphea is characterized by inflammation within the dermis that leads to thick, keloid-like nodules or bands. Clinically is very similar to keloids. Bullous morphea is a form that usually develops latter with the presence of bullae. Generalized morphea and sclerodermoid/morpheiform GVHD are characterized by sclerosis of the skin with diffuse edema and stasis of lymphatic fluid. Morphea (in particular the guttate type) may coexist with lichen sclerosus et athropicus.

Laboratory & other workups

1. General

routine laboratory tests
Borrelia serology
ANA

2. In children with severe linear forms: rheumatoid factor.

In localized scleroderma, eosinophilia can occur, especially at early phases. High titers of antinuclear antibodies (ANA) or anti-single strand DNA (ssDNA) and anti-histone antibodies (AHA) are present in 40-80% of patients with linear and generalized morphea. Borrelia burgdoferi test can be performed in the case of the suspected infection.

Dermatopathology

Interface dermatitis in early phases with varying intensity of inflammatory cellular infiltrates and changes of collagen structure depending on time of the disease course. Identical to progressive systemic sclerosis (see there).

The histopathology of localized scleroderma is identical to progressive systemic sclerosis. For a correct diagnosis, the specimen for biopsy must include the subcutaneous fat. At the inflammatory border, the vessel walls show edema and endothelial swelling. The capillaries and the small arterioles are surrounded by an infiltrate rich of CD4+ T-cells and sparse eosinophils, plasma cells and mast cells. At the later stages, the inflammatory infiltrate usually wanes or disappears, except in some areas of subcutaneous fat. The epidermis can show interface dermatitis in early phases, with flattening of the rete ridges in later phases. The capillaries and the small vessels are reduced in number. In the sclerotic phase collagen bundles are packed, with a hyalinised subcutis. In deep morphea, there is sclerosis of the underlying fascia.

Course

Plaque-type morphea: usually self-limited within years leaving sclerotic, pigmented scars. Linear circumscribed scleroderma and hemifacial atrophy: chronic with significant impairment of growth and function.

The course of localized scleroderma is characterized by a progression in 3-5 years, with a subsequent arrest. Atrophy and the depigmentation persist. Linear scleroderma and chronic hemifacial atrophy may be associated with significant impairment.

Complications

Depends on localization (face, overlying joints), depth and extension (pansclerotic morphea).

Generally, no systemic involvement (in contrast to progressive systemic sclerosis). In patients with linear type "en coup de sabre" or Parry-Romberg syndrome, possible underlying bone and CNS involvement.

The complications depend on the localization (face and overlying joints) and degree (panmorphea). Generally, there is no systemic involvement. In patients with linear type ‘en coup de sabre’ or Parry-Romberg syndrome, cranial magnetic resonance imaging (MRI) and high-resolution CT scan should be performed to evaluate deep tissue involvement.

Diagnosis

Clinical features, histology. In patients with linear type "en coup de sabre" or Parry-Romberg syndrome, cranial magnetic resonance imaging (MRI) and high resolution CT scan should be performed to document bone involvement and exclude CNS involvement.

Differential Diagnosis

Lichen sclerosus (et atrophicus), acrodermatitis chronica atrophicans (borreliosis), dermatosclerosis associated to severe chronic venous insufficiency , progressive systemic sclerosis, eosinophilic fasciitis.

The most important differential diagnoses are the other morpheiform and sclerodermoid conditions and systemic sclerosis. Other differential diagnoses are lichen sclerosus et atrophicus (which may coexist with morphea) keloids, dermatosclerosis (with severe chronic venous insufficiency). Also, Dupuytren and campodactyly (a benign ulnar deviation of the fourth and the fifth finger). Acrodermatitis chronica atrophicans can also be included within the differential diagnosis.

Prevention & Therapy

No causal therapy available.

Topical therapy: corticosteroids; calcipotriol
Intralesional corticosteroids
Systemic therapy: methotrexate; corticosteroids; hydroxychloroquine
Phototherapy: PUVA; bath PUVA; UVA-1
Physical therapy: range of motion exercises; manual lymphatic drainage

Treatment of localized scleroderma can be divided in topical, phototherapy, intralesional corticosteroids, systemic therapy, and physical therapy.

Topical therapy is based on corticosteroids, usually of high potency, such as clobetasol propionate, and they are useful for reducing inflammation in the active and superficial lesions, and to prevent the progression. A useful alternative to topical corticosteroids is the intralesional injection of corticosteroids. Calcineurin inhibitors (pimecrolimus and tacrolimus) are also employed. In some cases, vitamin D derivatives, such as calcipotriol, and vitamin A derivatives are used.
PUVA therapy or UVA1 phototherapy are successfully employed in localized scleroderma and may block its progression.
Systemic therapies are based on immunosuppressive agents and hydroxychloroquine. Methotrexate is the most helpful immunosuppressive agent in the treatment of all forms of localized scleroderma. Sometimes, a combination therapy with methotrexate and pulse corticosteroids led to a marked improvement of lesions. Oral corticosteroids alone (methylprednisolone and prednisone) are helpful in the treatment of the inflammatory phases.
Physiotherapy and lymphatic drainage are useful in cases of limb involvement associated with mobility impairment.