11 - 71 - ACQUIRED PERFORATING DISORDERS Flashcards
Acquired perforating disorders represent a group of separately identified cutaneous disorders that occur most often in the setting of what diseases?
chronic renal disease or diabetes mellitus
Describe the clinical manifestation of APD
APD is characterized clinically by round, umbilicated, skin-colored, erythematous or hyperpigmented papules and nodules with a central crust or keratotic plug, predominantly involving the extensor surfaces of the extremities and the trunk (APC, KD).
Mucous membranes are spared
most specific clinical finding of APD
central keratotic core
exhibits papules in a serpiginous configuration, often with central atrophy, and typically is localized to 1 region of the body, such as the neck, trunk, or the extremities
Elastosis perforans serpiginosa
These lesions are frequently asymptomatic and only occasionally manifest a koebnerization response
affects nearly 4 times more males than females
mode of inheritance of the familial form of Elastosis perforans serpiginosa
Autosomal dominant
refers to an extremely rare familial disorder that most commonly presents in early childhood with an equal sex distribution
reactive perforating collagenosis
This form of disease has the** strongest koebnerization response**.
reactive perforating collagenosis
most common systemic conditions associated with adult-onset APD
chronic kidney disease (CKD) and diabetes mellitus (DM)
the most common cause of CKD among APD patients
diabetic nephropathy
AEPS is well recognized as a potential adverse effect of what medication?
prolonged D-penicillamine therapy
Eliminated material in Acquired Perforating Collagenosis
Collagen bundles
Eliminated material in AEPS
elastic fibers
eliminated material in Kyrle Disease
amorphous dermal material with fibrin and/or keratin
Perforating folliculitis is characterized by perforation of the follicular epithelium by what contents?
degenerating collagen and extracellular matrix
Conditions Associated with Acquired Perforating Dermatosis
Treatment of Acquired Perforating Dermatosis
first line topical therapies for APD
age of onset of KD and APC
4th decade (average)
age of onset of perforating folliculitis
3rd decade (average)
age of onset of AEPS
2nd decade (average)
area of predilection of KD and APC
Extensor lower and upper extremities; head, neck, and trunk
area of predilection of AEPS
Nape of neck; upper extremities; face; lower extremities
Which APD is negative for koebner phenomenon?
Perforating folliculitis
Disease associations of AEPS
Down syndrome; Ehlers-Danlos syndrome; osteogenesis imperfecta; pseudoxanthoma elasticum; minor association with renal failure
