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Perforating Disorders


Classically, a group of disorders in which there is transepidermal elimination (“perforation”) of components of the dermis, in particular collagen and/or elastic fibers ( Table 79.1 ). Etiologies are multiple and include inherited as an isolated cutaneous disease or inherited in association with genetic disorders that affect connective tissue (e.g. Ehlers–Danlos syndrome). Most commonly, however, the perforating disorder is acquired and is related to the cutaneous trauma that results from scratching pruritic skin, especially in the setting of chronic kidney disease. A number of other cutaneous diseases occasionally undergo perforation, e.g. granuloma annulare, calcinosis cutis, chondrodermatitis nodularis helicis.

Table 79.1
Major perforating disorders. Courtesy, Ronald Rapini, MD.
Disease Incidence Time of onset Most common location Perforating substance Associations
Acquired perforating dermatosis (APD) Common Adulthood Extensor extremities (legs > arms); occasionally generalized Necrotic material +/or collagen >> elastic fibers Pruritus, usually in the setting of chronic kidney disease +/or diabetes mellitus ∗ affects 10% of patients on chronic hemodialysis
Elastosis perforans serpiginosa (EPS) Rare Childhood, early adulthood; variable if drug-induced Flexures, especially neck, antecubital fossae; face Elastic fibers Genetic disorders (see Fig. 79.5 ); penicillamine
Familial reactive perforating collagenosis (RPC) Rare Childhood Arms, hands, sites of trauma Collagen None
Perforating calcific elastosis Rare, but more common in African-American women Adulthood Periumbilical, abdomen > breast Calcified elastic tissue Primarily multiparity, obesity; occasionally chronic kidney disease
Perforating folliculitis (in the absence of pruritus) Uncommon Early adulthood Extremities, trunk Necrotic tissue May represent rupture of ordinary folliculitis

∗ Occasionally affects patients with pruritus due to other disorders, e.g. scabies, Hodgkin disease, primary biliary cholangitis; can also be induced by several medications (see Fig. 79.5 ).

Acquired Perforating Dermatosis (APD)

  • The most commonly observed perforating disorder (see Table 79.1 ); it is an acquired disease that affects primarily adults; the term APD encompasses several overlapping entities including acquired reactive perforating collagenosis (RPC), Kyrle disease, and perforating folliculitis.

  • In general, affected individuals have pruritic skin that has been scratched; the vast majority of patients have chronic kidney disease and/or diabetes mellitus, with most of the latter individuals having diabetic nephropathy; it affects up to 10% of patients receiving chronic hemodialysis.

  • Occurs less often in patients with pruritus due to other causes, e.g. insect bites, scabies, primary biliary cholangitis, Hodgkin disease.

  • Erythematous, skin-colored or hyperpigmented papules and papulonodules with a central keratotic core that favor the extensor surfaces of the extremities ( Figs 79.1–79.3 ); the central core is a reflection of the transepidermal elimination of collagen and/or elastic fibers as well as hyperkeratosis associated with epidermal hyperplasia.

    Fig. 79.1, Acquired perforating dermatosis.

    Fig. 79.2, Acquired perforating dermatosis.

    Fig. 79.3, Acquired perforating dermatosis.

  • DDx: see Table 79.2 .

    Table 79.2
    Differential diagnosis of perforating diseases.
    This is in addition to other perforating disorders discussed in this chapter.
    Acquired perforating dermatosis and reactive perforating collagenosis

    • Excoriations from a variety of causes (prurigo simplex)

    • Prurigo nodularis

    • Folliculitis

    • Arthropod bites

    • Perforation of exogenous foreign material

    • Perforation of endogenous substances (e.g. calcium)

    • Multiple keratoacanthomas

    • Dermatofibromas

    • If Koebner phenomenon, psoriasis, lichen planus, verrucae (autoinoculation)

    Elastosis perforans serpiginosa (see Ch. 15 for DDx of annular lesions)

    • Granuloma annulare

    • Tinea

    		} Common annular diseases

    • Sarcoidosis

    • Annular elastolytic giant cell granuloma (actinic granuloma)

    • Perforating pseudoxanthoma elasticum

    • Porokeratosis

    • Discoid lupus erythematosus

  • Rx: difficult; topical antipruritics (e.g. pramoxine), sedating antihistamines, CS-impregnated tape, intralesional CS, cryotherapy, tangential excision, Unna boot (impregnated gauze wrapping that serves as a physical barrier against scratching), NB- or BB-UVB, topical or oral retinoids.

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