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Morphea (localised scleroderma) and lichen sclerosus
Morphea is sclerosis of the skin and underlying tissue without internal organ involvement and without Raynaud phenomenon — unlike systemic sclerosis; lichen sclerosus is a related disease that mainly affects the genital area and raises the risk of SCC.
Why it matters in the exam
Exam stems show a firm, shiny, whitish plaque with a lilac ring on the trunk of a woman, a sclerotic band along a child's limb, or a midline band on the forehead — en coup de sabre. The question separates morphea from systemic sclerosis (no Raynaud, no nailfold capillary changes, no Scl-70 or centromere antibodies), and asks about treatment: topical steroids or calcineurin inhibitors in superficial disease, and methotrexate with steroids in linear disease in children, which can cause limb-length discrepancy and joint contractures. Lichen sclerosus appears as a white "cigarette-paper" lesion on the vulva, with itch and an SCC risk, and is treated with a very potent topical steroid. Remember that the linear facial form can also involve the brain and eye, and that follow-up in children includes limb measurements and eye checks.
Key points
- Morphea is localised skin sclerosis without Raynaud and without internal organ involvement, so it differs from systemic sclerosis.
- An active plaque looks firm and whitish with a lilac ring, and later a hard pigmented area remains.
- Linear morphea in children can cause limb-length discrepancy and joint contractures, and is treated with methotrexate and steroids.
- En coup de sabre is linear morphea of the forehead that can be linked to eye and brain involvement.
- Lichen sclerosus mainly affects the vulva, causes itch and narrowing, raises the SCC risk and is treated with clobetasol.
Summary table
| Form | Feature | Main risk | Treatment |
|---|---|---|---|
| Plaque morphea | Firm, shiny whitish plaque with a lilac ring on the trunk | Mainly cosmetic; usually settles over years | Potent topical steroids, tacrolimus, UVA1 |
| Linear morphea of a limb | Sclerotic band along a limb, mainly in children | Limb-length discrepancy, joint contractures | Methotrexate with initial systemic steroids, physiotherapy |
| En coup de sabre and Parry-Romberg | Depressed band on the forehead or hemifacial atrophy | Eye and brain involvement, seizures | Methotrexate; eye examination and brain MRI as symptoms require |
| Generalised morphea | Many plaques over the trunk and limbs | Restricted movement and breathing in the chest | Methotrexate or phototherapy |
| Lichen sclerosus | White, atrophic, paper-like lesions on the vulva; itch | Narrowing, adhesions; vulvar SCC | Very potent steroid (clobetasol) and lifelong follow-up |
A free exam question
מה מהבאים נכון לגבי טיפול ב- MORPHEA ? (the question as examined, in Hebrew)
- א. METHOTREXATE לא נבדק בילדים
- ב. שילוב של METHOTREXATE ו- PULSE CORTICOSTEROIDS הראה יעילות במחקרים קליניים
- ג. CYCLOSPORIN בשילוב PUVA מהווה טיפול הבחירה
- ד. NB-UVB מהווה טיפול הבחירה בין הסוגים השונים של פוטותרפיה
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Sources
- European Dermatology Forum S2k guideline on localized scleroderma, 2017 — Classification and treatment
- Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans for juvenile localized scleroderma, 2012 — Methotrexate and corticosteroids
- Fitzpatrick's Dermatology, 9th edition (2019) — Morphea and lichen sclerosus
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.