EpistemisLocalized scleroderma / Morphea
Specialty: Skin.
Why it occurs
- Idiopathic anomalous fibroblast activation (excessive and disorganized production of type I and III collagen limited to the dermis and subcutaneous tissue)
- Repeated local microtrauma or friction in vulnerable anatomical areas
- Previous suspected triggering infections (such as borreliosis in endemic areas)
- Genetic factors and local immune deregulation with overproduction of profibrotic cytokines (TGF-beta, IL-4)
- Previous exposure to localized radiotherapy for breast cancer.
Initial workup
Clinical diagnosis of plaque with active violet border ("lilac ring") and ivory-colored central induration. Confirmation by deep skin biopsy (4-6 mm punch) demonstrating thickened, homogenized collagen bundles and loss of skin adnexa. Basic autoimmune panel (ANA, anti-ssDNA antibodies).
red flags
Distribution of plaques that follow a linear trajectory on the face or scalp ("saber blow" morphea with risk of neurological and ocular compromise), linear lesions that cross major joints with strict functional limitation or risk of permanent fixed contractures, or involvement in children that compromises the normal growth of the limb.
Standard management
- Clobetasol propionate 0.05% cream — applied to the active violaceous edge to stop the initial inflammatory progression
- Topical tacrolimus 0.1% ointment — non-steroidal anti-inflammatory alternative useful in facial or fold injuries
- Methotrexate — immunosuppressant of choice in linear, generalized or deep forms to prevent the progression of sclerosis, doses of 15 to 25 mg once a week orally or subcutaneously, associated with folic acid
- Phototherapy with UVA-1 or narrow band PUVA (physical therapy of choice to soften sclerosed collagen).
Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.
- Area
- Skin
- Listed causes
- 5
- Treatment options
- 4