Perforating Disorders Flashcards
What is the hallmark of perforating disorders?
Transepidermal elimination of dermal components
What are the main components of the perforating material?
Collagen and/or elastic fibers
What is the most common type of perforating disorder?
Acquired perforating disorder in the setting of scratching in the setting of chronic kidney disease
What is the time of onset for familial reactive perforating collagenosis?
Childhood
What is the most common location for familial reactive perforating collagenosis?
Arms, hands, sites of trauma
What is the perforating substance in familial reactive perforating collagenosis?
Collagen
What is the onset of elastosis perforans serpinginosa?
Childhood, early adulthood.
This is variable in the penicillamine-induced EPS
What are the most common locations for elastosis perforans serpignosa?
Neck, face, arms, other flexural areas
What is perforating folliculitis?
Starts often in early adulthood on the trunk and extremities. The material is necrotic and it is really just normal folliculitis leading to rupture and expression of these materials.
What percent of dialysis patients get perforating dz?
10%
What is the perforating material in the acquired perforating dermatosis?
Necrotic material, collagen, or (rarely) elastic tissue
What group is more susceptible to perforating calcific elastosis?
Very rare, more common in black women
What is the most common location for perforating calcific elastosis?
Abdomen /periumbilical
What is the perforating substance in perforating calcific elastosis?
Calcified elastic tissue
What are the acquired perforating dermatoses?
Acquired reactive perforating collagenosis, Kyrle’s disease, and perforating folliculitis
What is the clinical presentation of acquired perforating dermatosis?
Presents with erythematous skin-colored or hyperpigmented papules and papulonodules with central keratotic core - favors extensor extremities
What are treatments for acquired perforating dermatosis?
Topical antipruritics (pramoxine), ILK, Unna boot, NB or BB-UVB, topical or oral retinoids
What is the histological characteristics of acquired perforating dermatosis?
Composed of transepidermal elimination of collagen and/or elastic fibers + hyperkeratosis a/w epidermal hyperplasia
- Can appear like a ‘reactive’ process b/c collagen and/or elastic fibers seen as ‘foreign’
key is you see the keratotic plug extending through the epidermis, leads to crusted plug
What types of perforating disorders have collagen only in the plug?
Familial reactive perforating collagenosis and reactive perforating collaginosis
What percentage of elastosis perforans serpiginosa is inherited?
40%
What is the material extruded in elastosis perforans serpignosa?
Elastin only
What is the clinical apperence of elastosis perforans serpignosa?
Multiple annular erythematous hyperkeratosis papules (+/- papules)
What is the histology of elastosis perforans serpignosa?
Hyperplastic epidermis with transepidermal elimination of dermal elastic fibers via channels - surface commonly has scale-crust
What is the clinical appearance of perforating calcific elastosis?
p/w verrucous keratotic papules and plaques
- papules localized to the periphery
- Most common in the periumbilical / abdomen >> breast (if superimposed CKD)
Histology of perforating calcific elastosis?
Elastic fibers that are coated with calcium
