71 - Acquired Perforating Disorders Flashcards
Familial primary perforating disorders that typically present in childhood
Reactive perforating collagenosis
Elastosis perforans serpiginosa
Adult-onset nonfamilial/acquired lesions are most commonly associated with
CKD
DM
4 separate clinicohistopathological entities characterize adult-onset acquired primary perforating disorders
Kyrle disease
Acquired perforating collagenosis
Perforating folliculitis
Acquired elastosis perforans serpiginosa
APD involving the extensor surfaces of the extremities and the trunk
APC
KD
Follicular-based distribution with minimal Koebner response
Perforating folliculitis
Papules in a serpiginous configuration, often with central atrophy, and typically is localized to 1 region of the body
Elastosis perforans serpiginosa
Most commonly presents in early childhood
Strongest Koebnerization response
Reactive perforating collagenosis
Most common cause of CKD among APD patients
Diabetic nephropathy
Lesions associated with CKD and DM
KD
Acquired reactive perforating collagenosis
AEPS is well recognized as a potential adverse effect of
Prolonged D-penicillamine therapy
May be phenotypic variants of a disease spectrum or merely different stages in lesional development
Perforating folliculitis
APC
Most likely represents an extreme phenotype or end-stage manifestation of perforating folliculitis and APC
KD
Traumatized keratinocytes bind to _____ via advanced glycation end product receptor CD36, inducing keratinocyte terminal differentiation and upward movement of keratinocytes along with glycated collagen
Advanced glycation end product-modified collagens I and III
Links keratinocytes with Type IV collagen within the basement membrane
Plays a vital role in epithelial cell signaling, migration, and differentiation
Fibronectin
Imbalances in ______ also have been demonstrated in APD lesions
TGG-beta3
Matrix metalloproteinase-1
Tissue inhibitor of metalloproteinase-1
