Perforating Disorders

Background

Perforating disorders all have the common feature of perforation, also known as transepidermal elimination. Altered connective tissue component within the dermis, such as collagen or elastic fibers, are eliminated through the epidermis. The clinical lesions are often raised, hyperkeratotic to verrcuous nodules. Histologically, dermal elements are eliminated associated with collections of inflammatory cells at the dermal-epidermal junction. Sometimes serial sections of the tissue is needed to demonstrate the perforation. There are several well-defined clinical entities as well as cases of acquired perforation, usually secondary to chronic renal failure.

Disease Manifestations Histopathology

Kyrle's disease 3-5th decades
Females>Males
Extensor surfaces of extremities, scapula, buttocks
Parakeratotic plug perforates into dermis leading to elimination

Perforating folliculitis Average 29 years
Hairy parts of arms, thighs, buttocks
Follicular papules 2-8 mm
Possible association with chemicals in textiles, formaldehyde Suppurative folliculitis with follicular plugging

Reactive perforating collagenosis Childhood onset
Recurrent umbilicated papules disappear after 6-8 weeks
New lesions develop through adulthood
Extremities
Cup-shaped depression of epidermis with basophilic changes in collagen
Perforation of collage

Perforating verruciform collagenoma Usually self-limited following episode of trauma Verruciform hyperplasia with perforation of collagen

Elastosis perforans serpiginosa (Perforating elastosis) Small grouped papules in serpiginous arrangement on neck, upper extremities, upper trunk, and face
Males in second decade
Possible AD mode with variable expression
One third of cases associated with other connective tissue disorders such as Marfan's, acrogeria, scleroderma, Ehlers-Danlos syndrome, osteogenesis imperfecta, or Down's syndrome.
Long term penicillamine therapy
Hyperplastic epidermis associated with channel of basophilic nuclear debris and eosinophilic elastic fibers eliminated through epidermis

Acquired perforating dermatosis Keratotic umbilicated papules with central keratotic plug on extensor surfaces
Causal relationship with chronic renal failure, hemodialysis, and/or diabetes mellitus
May be secondary to deposits of uric acid or hydroxyapatite
Varaible with combinations of the other perforating disorders

Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.

Internet Links

Last Updated 6/22/2001

Disease	Manifestations	Histopathology
Kyrle's disease	3-5th decades Females>Males Extensor surfaces of extremities, scapula, buttocks	Parakeratotic plug perforates into dermis leading to elimination
Perforating folliculitis	Average 29 years Hairy parts of arms, thighs, buttocks Follicular papules 2-8 mm Possible association with chemicals in textiles, formaldehyde	Suppurative folliculitis with follicular plugging
Reactive perforating collagenosis	Childhood onset Recurrent umbilicated papules disappear after 6-8 weeks New lesions develop through adulthood Extremities	Cup-shaped depression of epidermis with basophilic changes in collagen Perforation of collage
Perforating verruciform collagenoma	Usually self-limited following episode of trauma	Verruciform hyperplasia with perforation of collagen
Elastosis perforans serpiginosa (Perforating elastosis)	Small grouped papules in serpiginous arrangement on neck, upper extremities, upper trunk, and face Males in second decade Possible AD mode with variable expression One third of cases associated with other connective tissue disorders such as Marfan's, acrogeria, scleroderma, Ehlers-Danlos syndrome, osteogenesis imperfecta, or Down's syndrome. Long term penicillamine therapy	Hyperplastic epidermis associated with channel of basophilic nuclear debris and eosinophilic elastic fibers eliminated through epidermis
Acquired perforating dermatosis	Keratotic umbilicated papules with central keratotic plug on extensor surfaces Causal relationship with chronic renal failure, hemodialysis, and/or diabetes mellitus May be secondary to deposits of uric acid or hydroxyapatite	Varaible with combinations of the other perforating disorders