Dermatology
Morphea and Lichen Sclerosus
Built from Dermatology, 5th Edition
What’s inside
15 sections · 146 slides
Overview
Two separate diseases that both end in scarred, sclerotic skin — why they are studied together, and what tells them apart.
- Two disorders of skin sclerosis
- Key features of morphea
- Key features of lichen sclerosus
History and epidemiology of morphea
From an ancient Greek description of “hard skin” to a modern population study of how common morphea really is.
- Origins of the name “scleroderma”
- How the modern picture emerged
- How common is morphea
- Who gets morphea
Pathogenesis of morphea
Vascular injury, misdirected T cells, and overactive fibroblasts — the three-part chain that turns skin into scar.
- Morphea and systemic sclerosis: related but distinct
- Telling morphea from systemic sclerosis by pattern
- The real-world impact of morphea
- The three components of skin sclerosis
- Reading the pathogenesis diagram
- Vascular damage: the earliest change
- T cells direct the fibroblasts
- The cytokine signature behind fibrosis
- Genetic clues to susceptibility
- CXCL4: a possible blood biomarker
- Why simple immune-redirection treatment failed
- The tight-skin mouse model
- What the tight-skin (TSK) mouse teaches
- From mouse genetics to human disease and drug targets
- What actually triggers morphea
Classification and plaque-type morphea
How morphea is grouped into subtypes, and a close look at its most common form.
- How morphea is classified
- The subtypes of morphea at a glance
- Four published classification schemes
- Morphea classification schemes and their subtypes
- Which scheme classifies patients best
- Plaque-type morphea: the most common form
- Plaque-type morphea: an early lesion
- How the plaque matures
- The lilac ring: a marker of activity
- Size, course, and long-term outcome
- Plaque-type morphea: more clinical examples
- Guttate morphea and atrophoderma of Pasini and Pierini
- Deep morphea and its main mimic
- Nodular (keloidal) morphea
- Nodular morphea: a second example
- Bullous morphea
Linear morphea and Parry–Romberg syndrome
A band-like pattern with its own age of onset, course, and risk of lasting disability.
- Linear morphea: a different disease pattern
- Parry–Romberg syndrome
- Parry–Romberg syndrome: a second example
- Linear morphea of a limb
- How linear morphea behaves on a limb
- Morphea en coup de sabre: the full picture
- En coup de sabre: how the band develops
- Morphea en coup de sabre: a second example
- When en coup de sabre goes deeper
Generalized morphea and morphea in childhood
When plaques stop staying put — and why childhood-onset disease carries its own risks.
- Generalized morphea
- How generalized morphea behaves
- Generalized morphea: a second example
- Morphea in children
- Extensive childhood linear morphea
- Linear morphea of the leg in an adolescent
- Pansclerotic morphea of children
- Extracutaneous findings in childhood morphea
Laboratory findings, pathology, and look-alike syndromes
What blood tests and biopsies show, and the wider family of conditions that can mimic morphea.
- Laboratory findings in morphea
- What a skin biopsy shows depends on two things
- Morphea under the microscope
- Early versus late microscopic changes
- Deep morphea under the microscope
- Two patterns of look-alike sclerosis
- Two patterns of pseudoscleroderma
- A wide range of conditions can mimic morphea
- Differential diagnosis of morpheaform skin lesions
- Lipodermatosclerosis and injection-site sclerosis
- Implants and metabolic causes
- Radiation-induced morphea
- Radiation-induced morphea: further examples
- When radiation triggers both diseases at once
- Chronic graft-versus-host disease
Differential diagnosis of morphea
Sorting morphea from its two most important mimics, systemic sclerosis and lichen sclerosus, and a shortlist of rarer ones.
- Morphea versus systemic sclerosis
- Key mimics of morphea
- Other conditions in the differential diagnosis
Treatment of morphea
Matching the therapy to how active, how deep, and how disabling the disease is.
- General approach to treating morphea
- What determines the treatment plan
- When systemic therapy is warranted
- The overall treatment ladder
- Approach to the treatment of morphea
- Notes on the treatment ladder
- Phototherapy for morphea
- Phototherapy: what the evidence shows
- How well phototherapy works
- Measuring response with high-frequency ultrasound
- Systemic drug therapy
- Second-line and newer systemic options
- Emerging biologic approaches
- Surgery and rehabilitation
Lichen sclerosus — introduction, history, and epidemiology
An ivory-white, scarring disease that mainly targets the genital skin.
- What lichen sclerosus is
- Cancer risk if left untreated
- How the name settled
- How common is lichen sclerosus
- Who gets it, and when
- A link between lichen sclerosus and morphea
Pathogenesis and clinical features
How lichen sclerosus develops, and how it looks across extragenital and genital sites.
- Why lichen sclerosus develops
- Autoantibodies and oxidative stress
- Where lichen sclerosus appears
- Lichen sclerosus of the trunk
- Extragenital LS: typical distribution
- How an extragenital lesion evolves
- Advanced extragenital changes
- Extragenital LS: two further examples
- Fragility of extragenital lichen sclerosus
- Genital lichen sclerosus in women
- Anogenital lichen sclerosus
- How vulvar LS progresses
- Pregnancy, and a diagnostic pitfall
- Is genital lichen sclerosus precancerous?
- Anogenital lichen sclerosus: further examples
- Genital lichen sclerosus in men
- How genital LS presents in boys and men
- Circumcision, recurrence, and a diagnostic quirk
- Lichen sclerosus of the glans and shaft
- Moles that grow within lichen sclerosus
Pathology and differential diagnosis
The specific microscopic pattern of lichen sclerosus, and how it is told apart from its mimics.
- What lichen sclerosus looks like under the microscope
- Lichen sclerosus under the microscope
- Loss of elastic fibers: the key distinguishing clue
- How the infiltrate changes over time
- Telling lichen sclerosus apart from its mimics
- Lichen sclerosus-like changes in chronic GVHD
- A cancer look-alike on biopsy
Treatment of lichen sclerosus
Ultrapotent topical corticosteroids as the gold standard, and what to do when they are not enough.
- Goals of treating genital lichen sclerosus
- First-line treatment: topical corticosteroids
- Other topical options
- When treatment fails, and second-line options
- Long-term maintenance is essential
- Phototherapy and surgery for genital LS
- Ultrapotent topical corticosteroids in practice
- Treating extragenital lichen sclerosus
- Evidence for extragenital treatment
Comparing morphea and lichen sclerosus
Bringing the two conditions side by side, and the key takeaways to carry forward.
- Morphea and lichen sclerosus, side by side
- Comparing key features at a glance
- Key takeaways
References
Sources cited in this topic, from Bolognia's Dermatology, 5th Edition.
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- Dermatology, 5th Edition (2-Volume Set)