Morphea at a Glance
- Occurs in children and adults.
- Linear subtype predominates in children.
- Circumscribed and generalized predominate in adults.
- A self-limited or chronically relapsing autoimmune disorder targeting the skin with the following major features:
- Inflammatory, sclerotic, atrophic phases.
- Thickened sclerotic skin.
- Systemic disease including arthritis and neurological disorders.
- Differentiated from scleroderma by lack of acrosclerosis/sclerodactyly.
- Complications may cause significant irreversible cosmetic and functional impairment including the following:
- Atrophy of dermis, fat, and subcutaneous structures.
- Limb length discrepancy.
- Bony abnormalities.
- Treatment based on the following:
- Disease subtype.
- Depth of involvement.
- Stage (inflammatory, sclerotic, atrophic).
- Potential for complications.
Morphea is a chronic autoimmune disease characterized by sclerosis of the skin. The term “localized scleroderma” is also used in an attempt to highlight the systemic features of morphea. This causes confusion with systemic sclerosis (scleroderma) often resulting in unnecessary evaluation and anxiety. It is the opinion of the authors this term should be avoided. Morphea itself has a spectrum of manifestations ranging from skin only to multiple organ involvement. Of note, organ involvement in morphea is distinctly different from systemic sclerosis (Box 64-1).
Box 64-1 Differential Diagnosis of Morphea |Favorite Table|Download (.pdf)
Box 64-1 Differential Diagnosis of Morphea
Scleroderma (systemic sclerosis)
Trauma-induced fat necrosis (intramuscular injections)
Nephrogenic systemic fibrosis
Chronic graft-versus-host disease
Connective tissue nevi
Morpheaform basal cell carcinoma
Chemical mediated sclerosing skin conditions (toxic oil syndrome, rapeseed oil, etc.)
Lyme disease (acrodermatitis atrophicans)
Scleromyxedema, scleroderma chronica, pretibial myxedema
Always Rule Out
Carcinoma of the breast metastatic to skin (carcinoma en cuirase)
Porphyria cutanea tarda
Morphea has an estimated incidence of 2.7 per 100,000 with a female:male ratio of 2 to 3:1.1 Morphea is more common in Caucasians.2–5 The relative frequency of the different subtypes varies between studies. This is likely due to use of different classification systems. Twenty to thirty percent of morphea begins in childhood, but it can occur at any age.2–5 Linear morphea is the most common pediatric subtype (although all subtypes occur at any age).1,2,6 Twenty-five to eighty-seven percent of pediatric cases are linear morphea, with limb or trunk involvement in approximately 70%–80% and en coup de sabre (ECDS) or progressive facial hemiatrophy (PHA; formerly described as Parry–Rhomberg) in 22%–30%.1,2,4,5,7,8 In adults, circumscribed and generalized subtypes predominate. Deep morphea/morphea profunda is uncommon in both adults and children with a frequency of 2%–4%.1,2,4,7,8
Periods of disease activity vary from 3 to 6 years, but reactivation after periods of remission occurs in 20%.2,8 (eFig. 64-0.1). Others have ...