64: Morphea and Lichen Sclerosus Flashcards
1
Q
What are the clinical features of Circumscribed morphea?
A
Superficial: Single or multiple oval/round lesions limited to epidermis and dermis; Deep: Involves subcutaneous tissue, fascia, or muscle.
2
Q
What are the clinical features of Linear morphea?
A
Involves dermis and subcutaneous tissue without skin, dermis, subcutaneous tissue, muscle, or bone involvement; can affect the face and underlying structures.
3
Q
What are the clinical features of Generalized morphea?
A
More than or equal to 4 plaques in at least 2 of 7 anatomic sites; may involve isomorphic patterns and circumferential plaques.
4
Q
What are the clinical features of Panclerotic morphea?
A
Circumferential involvement of majority of body surface area; no internal organ involvement.
5
Q
What are the clinical features of Mixed morphea?
A
Combination of any subtype, e.g., linear-circumscribed.
6
Q
What is the female-to-male ratio in the epidemiology of morphea?
A
2 to 3:1.
7
Q
What demographic is more prevalent in morphea cases?
A
More prevalent in Whites.
8
Q
What percentage of morphea cases begin in childhood?
A
20-30%.
9
Q
What is the most common pediatric subtype of morphea?
A
Linear morphea, with 25-87% of pediatric cases being linear morphea.
10
Q
What is the typical duration of disease activity in morphea?
A
Typically lasts from 3 to 6 years.
11
Q
What is the recurrence rate of morphea?
A
Approximately 20% of cases or more.
12
Q
What are the clinical implications of Circumscribed and Generalized morphea?
A
Predominate in adults, with potential for significant cosmetic and functional impairment.
13
Q
What complications can arise from Linear morphea?
A
Can lead to limb-length discrepancies and bony abnormalities.
14
Q
What is the significance of Deep Morphea/Morphea Profunda?
A
Uncommon in both adults and children, but can cause significant complications due to deeper tissue involvement.
15
Q
What are the stages of cutaneous lesions in morphea?
A
- Inflammatory Stage: Begins as erythematous plaques or patches with a reticulated appearance; hypopigmented sclerotic plaques develop at the center, surrounded by an erythematous or violaceous border. Pain and/or itching can occur.
- Sclerotic Stage: Sclerosis develops centrally, has a shiny white color with surrounding hyperpigmentation, and loss of hair follicles (alopecia).
- Atrophic Stage: Months to years later, the sclerotic plaque softens and becomes atrophic with hypopigmentation or hyperpigmentation, characterized by cigarette paper wrinkling and cliff drop appearance.
16
Q
What are the noncutaneous findings associated with morphea?
A
- Musculoskeletal Involvement: Most common finding (12%), includes arthritis, myalgias, neuropathies, and carpal tunnel syndrome.
- Neurologic and Ocular Complications: Seizures, headaches, adnexal abnormalities, uveitis, and episcleritis. Rare complication: CNS vasculitis (emergency!).
- Facial Morphea: Dental malocclusion, altered dentition, gingivitis, deviation of the uvula, and atrophy of the tongue and salivary glands.
- Children: Significant morbidity affecting growth, function, and quality of life.
17
Q
What are the complications associated with morphea?
A
- Limited range of motion, limb-length discrepancy, joint deformity, and contracture (45% to 56% in linear morphea).
- Muscle weakness in affected extremities or face.
- Behavioral changes, learning disabilities, and seizures with (and without) facial involvement.
- Increased risk of squamous cell carcinoma in pansclerotic morphea due to chronic ulcers.
- Morphea can coexist with autoimmune diseases such as lichen sclerosus, systemic lupus erythematosus, and others.
18
Q
What genetic factors are associated with an increased risk for morphea?
A
- HLA-DRB1*04:04 and HLA-B37 confer an increased risk for morphea.
- Trauma: 16% develop initial lesions at sites of trauma from chronic friction or surgery (e.g., radiation, insect bites).
- Autoimmune Mediated Inflammation: Early lesions characterized by large amounts of activated T lymphocytes, plasma cells, and eosinophils.
19
Q
What are the key serum autoantibodies associated with morphea?
A
Key serum autoantibodies associated with morphea include:
- ANA (Antinuclear Antibodies)
- Anti-double-stranded DNA
- Anti-single-stranded DNA
- Anti-histone
- Antitopoisomerase IIα
- Antiphospholipid
- Anticentromere
- Anti-Scl-70
- Rheumatoid factor
- Matrix metalloproteinase-1
ANAs occur in 34-80% of patients with linear or generalized disease, with 81% showing a speckled pattern.
20
Q
What imaging techniques are used to assess morphea?
A
- MRI: Complete assessment of the extent of disease, depth of involvement, and disease activity.
- Ultrasonography: Evaluates or monitors tissue thickness, loss of subcutaneous fat and muscle, or other architectural alterations.
21
Q
What are the clinical features and prognosis of morphea?
A
Clinical Course: Self-limited or remitting-relapsing/chronic course. 25% of patients may experience recurrences. Indicators of Active Disease: New lesions or extension of existing lesions; Erythema/induration of the advancing edge of the lesion; Symptoms such as itching or tingling.
22
Q
What are the therapeutic goals in the management of morphea?
A
- Resolution of erythema (2 to 3 months)
- Lesion softening (12 months or more)
- Cessation of lesion growth and no new lesion development.
23
Q
What are the pathological findings in morphea biopsies?
A
- Inflammatory or indurated border
- Sclerotic center that includes subcutaneous fat
- Lesions with minimal clinical change: biopsy of site-matched unaffected skin may be performed.
24
Q
What cytokine profile is associated with the later sclerotic stage of morphea?
A
Elevated Th2-related cytokines, including IL-4 and IL-13.
25
What does elevated creatine kinase and aldolase levels indicate in morphea?
This indicates muscle involvement in the disease.
26
What phase of morphea does interstitial and perivascular inflammatory cell infiltrate in the dermis represent?
This represents the inflammatory phase of morphea.
27
What phase of morphea does loss of appendageal structures and telangiectasia represent?
This represents the atrophic phase of morphea.
28
What phase of morphea does thickened collagen bundles and severe sclerosis represent?
This represents the sclerotic phase of morphea.
29
What phase of morphea does eosinophils, mast cells, and macrophages represent?
This represents the inflammatory phase of morphea.
30
What phase of morphea does homogenization of the papillary dermis represent?
This represents the sclerotic phase of morphea.
31
What phase of morphea is indicated by a biopsy showing thickened collagen bundles and severe sclerosis?
This represents the sclerotic phase of morphea.
32
What phase of morphea is indicated by a biopsy showing eosinophils, mast cells, and macrophages?
This represents the inflammatory phase of morphea.
33
What phase of morphea is indicated by a biopsy showing homogenization of the papillary dermis?
This represents the sclerotic phase of morphea.
34
What phase of morphea is indicated by a biopsy showing lessening of sclerosis and absence of appendageal structures?
This represents the atrophic phase of morphea.
35
What phase of morphea is indicated by a biopsy showing enlarged tortuous vessels and thickened collagen bundles?
This represents the inflammatory phase of morphea.
36
What phase of morphea is indicated by a biopsy showing compression and loss of appendageal structures?
This represents the sclerotic phase of morphea.
37
What phase of morphea is indicated by a biopsy showing telangiectasia and loss of inflammatory cell infiltrate?
This represents the atrophic phase of morphea.
38
What do elevated CXCL-9 levels indicate about morphea?
Elevated CXCL-9 levels correlate with disease severity and are a useful biomarker in morphea.
39
What phase of morphea is indicated by a biopsy showing homogenization of the papillary dermis and sclerosis extending to the reticular dermis?
This represents the sclerotic phase of morphea.
40
What does fascial thickening and enhancement on MRI suggest in a patient with morphea?
This finding suggests active disease and deep involvement.
41
What does new lesions and erythema at the advancing edge indicate in morphea?
This indicates active disease, which is most responsive to treatment.
42
What are the key indicators of active disease in morphea?
Key indicators of active disease in morphea include:
1. New lesions or extension of existing lesions
2. Erythema/induration of the advancing edge of the lesion
3. Symptoms such as itch or tingling
43
How does the inflammatory phase of morphea differ from the sclerotic phase in terms of histopathological findings?
The inflammatory phase is characterized by interstitial and perivascular inflammatory cell infiltrate, primarily consisting of lymphocytes and plasma cells, along with eosinophils, mast cells, and macrophages. The sclerotic phase shows homogenization of the papillary dermis and sclerosis extending to the reticular dermis.
44
What imaging techniques are utilized in the assessment of morphea?
Imaging techniques include MRI and ultrasonography, which help assess disease extent, activity, and response to treatment.
45
What is the significance of the LoSCAT tool in the management of morphea?
The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) provides a standardized method to assess disease activity and damage.
46
What is the recommended treatment approach for patients with superficial to mid-dermis involvement of morphea?
Patients are amenable to topical therapy or phototherapy.
47
What are the indications for systemic immunosuppressive therapy in morphea patients?
Systemic immunosuppressive therapy is indicated for patients with systemic involvement, particularly those with linear and generalized forms.
48
How does phototherapy affect the treatment of morphea?
Phototherapy, particularly Narrowband UVB, is effective for lesions affecting the superficial dermis.
49
What is the role of Methotrexate in the treatment of morphea?
Methotrexate is considered a first-line systemic treatment for deep morphea and rapidly progressive forms.
50
What is the expected duration of treatment with Methotrexate for morphea?
The average duration of treatment is 21 to 27 months, with a 15% relapse rate at two years.
51
What is the significance of Vitamin D derivatives in the treatment of morphea?
Vitamin D derivatives have shown improvement in most patients over several months of therapy.
52
What is the recommended treatment for patients with active, inflammatory superficial plaque-type morphea?
Occluded topical tacrolimus 0.1% ointment might be effective.
53
What type of phototherapy is recommended for superficial dermal lesions?
Narrowband UVB phototherapy is recommended.
54
What type of phototherapy is most effective for deeper dermal lesions?
UVA-based therapies, particularly UVA-1, are most effective.
55
What is the first-line systemic treatment for rapidly progressive morphea?
Methotrexate is considered the first-line systemic treatment.
56
What is the second-line systemic treatment for a patient with morphea refractory to methotrexate?
Mycophenolate mofetil is the second-line systemic treatment.
57
For what type of morphea is occluded topical tacrolimus 0.1% effective?
This treatment is effective for active, inflammatory superficial plaque-type morphea.
58
What is the recommended treatment approach for patients with active morphea disease and deep dermis involvement?
Patients should be treated systemically, often requiring aggressive therapy.
59
How should patients with generalized and linear morphea be monitored and treated?
They should be closely followed and treated aggressively if rapid onset of confluent plaques occurs.
60
What is the role of phototherapy in the treatment of morphea for deep involvement?
Phototherapy is likely ineffective for deep involvement and should not be considered primary therapy.
61
What are the first-line systemic treatments for deep morphea?
Methotrexate is considered the first-line systemic treatment.
62
What is the expected clinical improvement timeline for patients treated with mycophenolate mofetil?
Patients typically experience improvement on average 3.5 months after starting treatment.
63
What is the female-to-male ratio in the epidemiology of Lichen Sclerosus?
The female-to-male ratio is 5:1.
64
What are the common clinical features of vulvar Lichen Sclerosus?
Common features include porcelain-white atrophic papules coalescing into plaques, fissures, erosions, and a classical figure-8 pattern.
65
What complications are associated with female patients suffering from Lichen Sclerosus?
Complications include dyspareunia, urinary obstruction, and increased risk of squamous cell carcinoma.
66
What are the male genital manifestations of Lichen Sclerosus?
Manifestations include involvement of the glans penis, painful erections, and urethral strictures.
67
What is the recommended adjunctive therapy for patients with Lichen Sclerosus?
Adjunctive therapy includes consulting with various specialists to maximize cosmesis and function.
68
What is the classical pattern of involvement in genital lichen sclerosus?
The classical pattern is the figure-8 pattern involving the vulva and anus.
69
What complication should be considered in a male patient with genital lichen sclerosus who has painful erections?
Urethral strictures should be considered.
70
What are the common symptoms of extragenital Lichen Sclerosus?
Common symptoms include pruritus, burning, or asymptomatic lesions.
71
What does the 5:1 female-to-male ratio indicate in relation to Lichen Sclerosus?
It indicates that Lichen Sclerosus preferentially affects women, especially in their 5th to 6th decade of life.
72
How do the clinical features of vulvar Lichen Sclerosus differ from those of anogenital Lichen Sclerosus?
Vulvar Lichen Sclerosus features porcelain-white atrophic papules, while anogenital Lichen Sclerosus presents with intractable itching and discomfort.
73
What are the potential complications associated with Lichen Sclerosus in females?
Complications include dyspareunia, urinary obstruction, and a 4-6% risk of developing squamous cell carcinoma.
74
What adjunctive therapies are recommended for patients with Lichen Sclerosus?
Recommended therapies include consultations with rheumatology, physical therapy, and plastic surgery.
75
What are the key factors contributing to the etiology and pathogenesis of Lichen Sclerosus?
Key factors include genetic predisposition, local irritation, autoimmune diseases, and immune response.
76
What is the clinical course and prognosis for patients with Lichen Sclerosus?
The course is chronic and relapsing, with a favorable prognosis if diagnosed and treated early.
77
What are the recommended management strategies for Lichen Sclerosus?
Management strategies include topical treatments and ultrapotent topical corticosteroids.
78
What are the results of Lichen Sclerosus?
Results in scarring, resorption of labia, alopecia, and altered vulvar anatomy.
79
What are the recommended management strategies for Lichen Sclerosus?
Management strategies include:
- **Topical treatments**: Aim to improve symptoms and prevent long-term complications.
- **Ultrapotent topical corticosteroids**: Clobetasol propionate 0.05% is the first-line treatment for genital LS.
- **Tapering regimen**: Clobetasol 1x/day for 4 weeks, every other day for 4 weeks, then 2x a week for 4 weeks.
- **Adjunctive treatments**: Barrier creams containing zinc oxide and referrals to specialists such as urogyn ecology and gastroenterology.
80
What are the pathological features associated with Classical Lichen Sclerosus?
Pathological features include:
- **Atrophic epidermis**: A lichenoid infiltrate at the dermal-epidermal junction.
- **Papillary edema**: Present in early LS, replaced by fibrosis as the lesion matures.
- **Epidermal hyperplasia/dysplasia**: Associated with LS on vulvar specimens, linked to an increased risk of malignant transformation, especially with high-risk human papillomaviruses.
81
What stage of Lichen Sclerosus is represented by a biopsy showing atrophic epidermis and a lichenoid infiltrate?
This represents classical lichen sclerosus.
82
What is the typical duration of initial treatment for vulvar lichen sclerosus with clobetasol propionate 0.05% ointment?
The typical duration is 2 to 3 months until remission.
83
What genetic component is implicated in the etiology of lichen sclerosus?
A genetic component is implicated, with a possible common genetic predisposition to autoimmune diseases.
84
What are the key factors contributing to the etiology and pathogenesis of Lichen Sclerosus?
Key factors include:
- **Genetic predisposition**: Increased risk in individuals with a family history of LS.
- **Local irritation and trauma**: Such as the Koebner phenomenon.
- **Autoimmune diseases**: Increased frequency suggests a common genetic predisposition.
- **Immune response**: Infiltration of T cells, B cells, and antigen-presenting dendritic cells, with a predominance of CD8 cytotoxic T cells.
- **Cytokine and chemokine regulation**: Upregulation of Th1 and Type I IFN-regulated cytokines and chemokines, including IFN-γ, CXCL-9, CXCL-10, and CXCL-11.
85
How does the clinical course and prognosis of Lichen Sclerosus vary based on the timing of diagnosis and treatment?
The clinical course and prognosis of Lichen Sclerosus (LS) vary as follows:
- **Early diagnosis and treatment**: Favorable prognosis in patients diagnosed and treated in early nonscarring stages.
- **Childhood-onset LS**: May not resolve at puberty and can remain persistent.
- **Recalcitrant chronic LS**: Can lead to erosions and progressive scarring, resulting in severe dysfunction of urination, sexual function, and defecation.
- **Advanced LS**: Scarring can lead to resorption of the labia, alopecia, and altered anatomical structure of the vulva.
86
What role does phototherapy play in the treatment of Lichen Sclerosus?
Phototherapy plays a role as an adjunct treatment for Lichen Sclerosus (LS) in the following ways:
- **Indication**: Recommended when topical corticosteroids are ineffective or not tolerated.
- **Types of phototherapy**:
- **Photodynamic therapy with 5-aminolevulinic acid**: Shown efficacy in treating vulvar LS.
- **UVA-1 therapy**: Considered a second-line treatment option.
87
What is the role of topical corticosteroids in the maintenance therapy for vulvar lichen sclerosus?
Topical corticosteroids are used consistently as maintenance therapy, reducing the risk of relapse of vulvar lichen sclerosus. Mometasone furoate applied 2 times a week is effective in reducing relapse rates.
88
What treatment options are available for male genital lichen sclerosus?
Treatment options include circumcision to resolve male genital lichen sclerosus and associated phimosis, as well as potent topical steroids which may obviate the need for surgery.
89
How does the treatment of extragenital lichen sclerosus compare to that of morphea?
The treatment modalities for extragenital lichen sclerosus are similar to those used for morphea, including topical corticosteroids, topical calcineurin inhibitors, UVA-1 phototherapy, and methotrexate in combination with prednisone.
90
What is the recommended treatment for generalized extragenital lichen sclerosus that is resistant to topical steroids?
For generalized extragenital lichen sclerosus that is resistant to topical steroids, UVA-1 phototherapy can be beneficial.
91
What is the risk of vulvar carcinoma in noncompliant patients with vulvar lichen sclerosus?
The risk of vulvar carcinoma is 4.7% in partially compliant patients.
92
What surgical treatment is curative for a male patient with genital lichen sclerosus and phimosis?
Circumcision is curative for male genital lichen sclerosus and associated phimosis.
93
What phototherapy modality can be beneficial for a patient with extragenital lichen sclerosus resistant to topical steroids?
UVA-1 phototherapy can be beneficial for resistant extragenital lichen sclerosus.
94
What is the role of topical corticosteroids in the maintenance therapy for vulvar lichen sclerosus (LS)?
Topical corticosteroids are used consistently as maintenance therapy for vulvar lichen sclerosus. They reduce the risk of relapse, with Mometasone furoate applied 2 times a week being effective in decreasing relapse rates. Maintenance therapy with topical corticosteroids 2-3 times a week is recommended even for asymptomatic genital LS.
95
How does circumcision affect male genital lichen sclerosus (LS)?
Circumcision can resolve male genital lichen sclerosus and the associated phimosis. Additionally, potent topical steroids may reduce the need for surgical intervention in these cases.
96
What treatment modalities are effective for extragenital lichen sclerosus (LS)?
Treatment modalities for extragenital lichen sclerosus include:
1. **Topical corticosteroids**
2. **Topical calcineurin inhibitors**
3. **UVA-1 phototherapy**
4. **Methotrexate** in combination with prednisone
5. **Super-potent topical steroids**
For generalized extragenital LS or cases resistant to topical steroids, UVA-1 phototherapy can be beneficial.
