Lichen Sclerosus (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
| Figure 3.38
Acquired perforating dermatosis
(Courtesy of Dr. Paul Getz) |
- Treatment: superpotent topical corticosteroid
- Associated with other autoimmune diseases (i.e. thyroid disease, pernicious anemia)
Perforating Disorders (Figure 3.38) - Group of disorders characterized by transepidermal elimination of altered dermal connective tissue or keratin
- See Table 3 -19
| | | | | | | Table 3-19 Perforating Disorders | | | Disease | | Description | | Histology | | Associations | | Acquired perforating dermatosis
(Kyrle’s disease)
(Acquired reactive perforating collagenosis) | | Intensely pruritic umbilicated papules with keratinous plugs involving extensor limbs, less common on trunk/face, + Köebner phenomenon | | Cup-shaped invagination of epidermis with hyperkeratotic plug containing inflammatory debris and collagen fibers, vertically oriented collagen extruded into plug (in dermis) | | Diabetes or renal failure (many patients on hemodialysis), some cases related to malignancy | | Inherited reactive
perforating collagenosis | | Similar lesions but occur in childhood | | Similar histology to acquired form | | None | | Elastosis perforans
serpiginosa (EPS) | | Skin-colored to red papules in serpiginous, linear or arciform pattern over upper extremities or neck, may measure several centimers in diameter | | ↑↑ Amount of elastic fibers in papillary dermis appearing clumped and ‘clutched’ by epidermis (hyperplastic); elastic fibers and inflammatory cells extruded via granulomatous reaction | | Associated with:
{MADD PORES:Marfan’s, Acrogeria, Down syndrome,
D-Penicillamine, Pseudoxanthoma elasticum, Osteogenesis imperfecta, Rothmund-Thomson, Ehlers Danlos, Scleroderma} | | Perforating folliculitis | | Keratotic follicular papules commonly over extensor surfaces | | Involved hair follicle shows disruption of lateral wall; parakeratotic plug consists of collagen, elastic fibers and inflammatory cells | | Chronic renal failure and diabetes | | Perforating
periumbilical calcific
elastosis | | Keratotic papules involving the abdomen | | Transepidermal elimination of calcified elastic fibers | | African-American women,
multiparity | | | | | | | | | |
| | | | | |
|
| | 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) |
|
