Acquired Perforating Disorders at a Glance
- Acquired perforating disorders represent a group of separately identified cutaneous disorders that occur most often in the setting of chronic renal disease or diabetes mellitus.
- The previously recognized Kyrle's disease (KD), acquired elastosis perforans serpiginosa (AEPS), acquired reactive perforating collagenosis (ARPC), and perforating folliculitis (PF) are now classified under the umbrella term of acquired perforating dermatosis.
- Lesions present as umbilicated papules and/or nodules with a central keratotic plug or crust distributed preferentially on extensor surfaces of the extremities.
- Histopathological examination of lesional skin demonstrates invagination of the epidermis with extrusion of dermal material (collagen, elastin, and/or fibrin) through the cup-shaped epidermal depression.
- Treatment is challenging with no universally effective therapy, and patients often exhibit a chronic course.
Acquired perforating disorders represent a group of separately identified cutaneous disorders that occur in adult patients, most often in the setting of chronic kidney disease (CKD) or diabetes mellitus (DM).1 Kyrle's disease (KD), acquired elastosis perforans serpiginosa (AEPS), acquired reactive perforating collagenosis (ARPC), and perforating folliculitis (PF) were previously considered distinct disorders.2–5 Given their shared clinical and histopathological characteristics, these four disorders are now classified under the umbrella term of acquired perforating dermatosis (APD).1 APD is characterized clinically by the presence of umbilicated papules and/or nodules with a central keratotic plug or crust and histologically by the transepidermal extrusion of dermal components (collagen, elastin, and/or fibrin).6 Although some cases may exhibit clinical and histological characteristics that typify one of the four classically recognized disorders, use of the comprehensive term APD is encouraged.
Kyrle, in 1916, first described KD in a young diabetic woman and termed it hyperkeratosis follicularis et parafollicularis in cutem penetrans.5 In 1953, Lutz published the initial description of elastosis perforans serpiginosa (EPS) as keratosis follicularis serpiginosa.7 The first case of AEPS associated with CKD was identified in 1986 by Schamroth, Kellen, and Grieve.2 Mehregan, Schwartz, and Livingood reported the earliest description of reactive perforating collagenosis (RPC) in 1967, and the first case associated with DM was recognized by Poliak et al in 1982.3,8 PF was originally described by Mehregan and Coskey in 1968.4 In 1989, Rapini, Heber, and Drucker recognized the common clinical and histopathologic characteristics of these disorders and introduced the term acquired perforating dermatosis.1 Various terminology has been used in the literature to refer to APD (Table 69-1).
Table 69-1 Synonyms for Acquired Perforating Dermatosis |Favorite Table|Download (.pdf)
Table 69-1 Synonyms for Acquired Perforating Dermatosis
Acquired reactive perforating collagenosis9
Hyperkeratosis follicularis et parafollicularis in cutem penetrans5
Keratosis follicularis serpiginosa7
Perforating folliculitis of hemodialysis14
Reactive perforating collagenosis of diabetes mellitus (DM) and renal failure15
Uremic follicular hyperkeratosis16