64 - Morphea and Lichen Sclerosus Flashcards
Y/N: Organ involvement in morphea is distinctly different from systemic sclerosis
Yes
Morphea is more common in (males/females)
Females
Most common pediatric subtype of morphea
Linear morphea
Formerly described as Parry-Romberg syndrome
En coup de sabre or
Progressive hemifacial atrophy
Subtypes that predominate in adults
Circumscribed morphea
Generalized morphea
Stages of cutaneous lesions in morphea
Inflammatory
Sclerotic
Atrophic
Stage of morphea:
Erythematous plaques or patches, sometimes with a reticulated appearance
Develop hypopigmented sclerotic plaques at the center, surrounded by an erythematous or violaceous border
Inflammatory stage
Stage of morphea:
Sclerosis develops centrally, has a shiny white color with surrounding hyperpigmentation
Sclerotic stage
Stage of morphea:
Sclerotic plaques softens and becomes atrophic with hypopigmentation or hyperpigmentation
Atrophic stage
The _____ stage is associated with cigarette paper wrinkling (papillary dermis), cliff drop (dermal), or deep indentions altering the contour of the affected body part (subcutis or deeper atrophy)
Atrophic
Morphea subtypes
- Circumscribed
- Linear
- Generalized
a. Coalescent plaque
b. Pansclerotic
c. Mixed
Both linear and generalized morphea may begin with
Circumscribed lesions
Though to be a residua of plaque-type morphea
Borders have a “cliff-drop” appearance resembling “burnt-out” morphea lesions
Atrophoderma of Pasini and Pierini
Generalized morphea is characterized by more than or equal to _____ lesions on at least _____ of 7 different anatomic sites
4
2
3 variants of generalized morphea
- Isomorphic
- Symmetric
- Pansclerotic
In contrast to systemic sclerosis, generalized morphea does not present with
Acrosclerosis or sclerodactyly
Linear morphea usually affects the
Extremities
Face
May present as an atrophic linear plaque on the forehead extending to the scalp (where cicatricial alopecia occurs), brow, nose, and lip
En coup de sabre (“cut of the sword”)
In deep morphea, a “_____ sign” (depression) may be present at the site of tendons and ligaments
Groove
Also known as Shulman syndrome
Presents with rapid onset of symmetric areas of pain and poorly circumscribed indurated, plaques, usually on the extremities
Eosinophilic fasciitis
Most common extracutaneous finding in morphea patients
Musculoskeletal involvement
Complications associated with en coup de sabre
Neurologic
Ocular
_____ morphea is associated with an increased risk of squamous cell carcinoma
Pansclerotic
_____ morphea is associated with an increased rate of autoimmune disease
Generalized
The pathogenesis of morphea appears to involve a transition form a predominantly _____ profile in the early inflammatory stage of morphea to a _____ profile in the later sclerotic stage
Th1
Th2
Histologic examination may aid therapeutic decision making because it is sometimes difficult to determine the degree of activity or depth of involvement by clinical examination alone. Biopsy of the _____ of a lesion may provide insight into both
Advancing edge
ANAs occur in 34% to 80% of patients and are more common in patients with _____ disease
Linear or
Generalized
Y/N: The clinical and prognostic significance of autoantibodies in morphea remains unclear and testing for them is not indicated
Yes
Peripheral eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate or C-reactive protein may occur with active disease of any type, but particularly _____ morphea
Deep
Biopsies should be taken form the _____ when present or _____
Inflammatory or indurated border
Sclerotic center and include subcutaneous fat
For lesions with minimal clinical change, biopsy of _____ is helpful
Site-matched unaffected skin
Becoming increasingly useful for determination of lesion activity and depth
Should be considered when deep morphea is present or suspected
MRI
Ultrasonography
Characterized by acral sclerosis/sclerodactyly, nail-fold capillary changes, Raynaud phenomenon, characteristic internal organ involvement (eg, pulmonary, renal, and GI), and hallmark autoantibodies (these features that are absent in morphea)
Systemic sclerosis
For a morpheaform plaque on the breast, _____ resulting from metastatic breast cancer is an important diagnosis to rule out
Carcinoma en cuirasse
Morpheaform reactions can also develop secondary to
Vitamin K1 injections
Taxanes
IFN-beta1a
Balicatib
A higher risk of recurrence (31% of patients) has been reported for _____ as compared to other subtypes
Linear morphea of the extremities
_____ disease is most responsive to therapy
Early, active
Morphea involving the superficial to mid-dermis would logically be amenable to _____; however, involvement of the deep dermis and beyond should be treated _____
Topical therapy or phototherapy
Systematically
Patients with _____ (particulary those with rapid onset of confluent plaques) are likely at risk for severe, extensive disease
Linear
Generalized
Phototherapy modalities with level 1 evidence
Broadband UVA
Narrowband UVB
UVA-1
Narrowband UVB should be considered for lesions affecting the
Superficial dermis
UVA-based therapies are more appropriate for _____ lesions
Deeper dermal
Disease is expected to improve (progression halted and erythema improved) after _____ treatments and most trials stopped after _____ treatments
10 to 20
20 to 30
Considered a first-line systemic treatment for morphea, especially for deep morphea and rapidly progressive or disabling morphea
Methotrexate
The use of methotrexate (monotherapy) and methotrexate combined with _____ is effective based on level 1 evidence
Systemic corticosteroids
Can be used as a second-line systemic treatment
Effective for morphea refractory to methotrexate or patient with contraindications or intolerance of methotrexate
Mycophenolate mofetil
Level 2 evidence suggests the use of occluded topical _____ might be effective for active, inflammatory superficial plaque-type morphea
Tacrolimus 0.1% ointment
Y/N: The most commonly used treatment for morphea, topical steroids, has not been investigated in a clinical trial. There are also no studies investigating the use of intralesional steroids.
Yes
Vulvar lichen sclerosus has an increased risk of _____, particularly if left untreated
Squamous cell carcinoma
Lichen sclerosus is more frequent in (males/females)
Females
Lichen sclerosus preferentially affects women in the _____ decade of life and children younger than _____
Fifth or sixth decade
10 years of age
Genital lichen sclerosus in males is almost exclusively seen in
Uncircumscribed men
Vulvar lichen sclerosus present with porcelain-white atrophic papules coalescing into plaques on the
Labia minora and majora
Often the classical _____ pattern of the vulva and anus may be observed
Figure-8
Male genital lichen sclerosus is also known as
Balanitis xerotica obliterans
Many male genital lichen sclerosus are simply diagnosed as
Phimosis
Extragenital lichen sclerosus typically affects the
Thighs
Neck
Trunk
Lips
Noncutaneous findings in lichen sclerosus
Association with: Autoimmune thyroid disease Alopecia areata Pernicious anemia Morphea Vitiligo
The lifetime risk of developing squamous cell carcinoma as a complication of longstanding LS has been estimated in the order of _____%
4 to 6
Represent significant risk factors for the development of SCC in LS
Age
Long duration of LS
Human papillomavirus infection
Evidence of hyperplastic changes
_____, a tumor-suppressor gene, is downregulated in vulvar squamous cell carcinoma associated with LS
IRF6
Characterization of the infiltrate of LS has shown a predominance of _____ over _____ cells, and presence of regulatory T cells
CD8 cytotoxic T cells
CD4 T cells
Gene expression profiling of LS has shown significant upregulation of _____ cytokines and chemokines
Th1 and Type 1 IFN-regulated
Classical LS shows a/an _____ epidermis and a _____ infiltrate at the dermal-epidermal junction
Atrophic
Lichenoid
Vulvar lichen planus more commonly involves the _____, whereas LS spares the ______
Vagina
Common complication in children with anogenital LS
Constipation
LS in children has occasionally been associated with _____, a common lesion that can be confused with condyloma and is also associated with constipation
Perineal pyramidal protrusion
First-line treatment for genital LS
Ultrapotent topical corticosteroids, most commonly clobetasol propionate 0.05%
(Ointments/Creams) tend to be less irritating and better tolerated than (ointments/creams)
Ointments
Creams
Will generally resolve male genital LS and the associated phimosis
Circumcision