(LSetA) (Figure 3.37C) - Autoimmune disease occurring in children and women in the 5 th or 6 th decade (less often in men)
- Autoantibody likely to extracellular matrix protein-1 (ECM-1)
- Presents initially as pruritic hypopigmented plaques typically with pink inflammatory border most commonly in genital area; expands and becomes atrophic, ± follicular hyperkeratosis; figure-of-eight pattern if perianal/vulvar area involved; dyspareunia, difficulty urinating, scarring/ contractures (if advanced), ↑ risk of SCC in sclerotic areas
| | | | | | Five percent lifetime risk of SCC | | | | | |
|
- Histology: atrophic epidermis, ± follicular plugging, vacuolar degeneration at basal layer, band-like lymphocytic infiltrate, pale staining homogenized collagen with edema (± subepidermal bulla), dilated lymphatic vessels
- Treatment: superpotent topical corticosteroid
- Associated with other autoimmune diseases (i.e. thyroid disease, pernicious anemia)
| | Figure 3.37
A: Telangiectasias in CREST
(Courtesy of Dr. Paul Getz)
B: Relapsing polychondritis
(Reprint from Morgan MB,
Smoller BR, Somach SC.
Deadly Dermatologic Diseases.
New York, NY: Springer; 2007)
C: Lichen sclerosus
(Courtesy of Dr. Iris K.
Aronson) |
|
