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62 Part I Disorders Presenting in the Skin and Mucous Membranes Pityriasis Rubra Pilaris (PRP) ICD-9: 696.4 ° ICD-10: L44.4 ■ Rare, chronic, papulosquamous disorder often progressing to erythroderma. ■ Six types exist. ■ Follicular hyperkeratotic papules, reddish-orange progressing to generalized erythroderma. Sharply demarcated islands of unaffected (normal) skin. Classification 3 Type 1: Classic Adult Generalized, beginning on head and neck. Type 2: Atypical Adult Generalized, sparse hair. Type 3: Classic Juvenile Appears within the first 2 years of life, generalized. Type 4: Circumscribed Juvenile In prepubertal children, localized. Type 5: Atypical Juvenile Onset in first few years of life, familial, generalized. Type 6: HIV-Associated Generalized, associated with acne conglobata, hidradenitis suppurativa, and lichen spinulosus. Epidemiology Rare. Affects both sexes and occurs in all races. Etiology and Pathogenesis Unknown. Clinical Manifestation Both insidious and rapid onset occur. Skin Lesions. All types of PRP. An eruption of follicular hyperkeratotic papules of reddish-orange color usually spreading in a cephalocaudal direction (Fig. 3-16). Confluence to a reddishorange psoriasiform, scaling dermatitis with sharply demarcated islands of unaffected skin (Fig. 3-37). In dark skin papules are brown (Fig. 3-18). Distribution. Types 1, 2, 3, 5, and 6: Generalized, classically beginning on the head and neck, then spreading caudally. Progression to erythroderma (except for types 2 and 4). 3 Griffiths WAD. Clin Exp Dermatol. 1980;5:105 and Gonzáles-López A et al. Br J Dermatol. 1999;140:931. ■ Waxy, diffuse, orange keratoderma of palms and soles; nails may be affected. ■ Most effective therapy is MTX, systemic retinoids Scalp and Hair. Scalp affected, as in psoriasis, often leading to asbestos-like accumulation of scale. Hair not affected except in type 2 where sparse scalp hair is observed. Mucous Membranes. Spared. Palms and Soles Pityriasis Pilaris (Type 1, Classic Adult). Palm shows diffuse, waxy, yellowish/orange hyperkeratosis (Fig. 3-19). Nails. Common but not diagnostic. Distal yellow-brown discoloration, nail plate thickening, subungual hyperkeratosis, and splinter hemorrhages. See Section 34. Associated Conditions. Ichthyosiform lesions on legs in type 2. Scleroderma-like appearance of hands and feet in type 5. Acne conglobata, hidradenitis suppurativa, and lichen spinulosus in type 6. Diagnosis and Differential Diagnosis The diagnosis is made on clinical grounds. The differential diagnosis includes psoriasis, follicular ichthyosis, erythrokeratodermia variabilis, and ichthyosiform erythrodermas. Laboratory Examinations Histopathology. Not diagnostic but suggestive: Hyperkeratosis, acanthosis with broad short rete ridges, alternating orthokeratosis, and parakeratosis. Keratinous plugs of follicular infundibula and perifollicular areas of parakeratosis. Prominent granular layer may distinguish PRP from psoriasis. Superficial perivascular lymphocytic infiltrate. Course and Prognosis ■ ◐ ➔ ○ A socially and psychologically disabling condition. Long duration; type 3 often resolves after 2 years; type 4 may clear. Type 5 has a very chronic course. Type 6 may respond to highly active antiretroviral therapy (HAART).
Section 3 Psoriasis and Psoriasiform Dermatoses 63 Management Figure 3-16. Pityriasis rubra pilaris (type 1, classic adult) Orange-red follicular papules beginning on the head and neck have coalesced on the chest of a 57-year-old male. There are sharply demarcated islands of unaffected normal skin. Topical therapies consist of emollients, keratolytic agents, vitamin D 3 (calcipotriol), glucocorticoids, and vitamin A analogues (tazarotene). All are not very effective. Phototherapy (ultraviolet A phototherapy, narrowband ultraviolet B phototherapy, and photochemotherapy) is effective in some cases. Most effective treatment consists of systemic administration of MTX or retinoids (both as in psoriasis). In type 6: HAART. The anti-TNF agents, e.g., infliximab and etanercept are effective. Figure 3-17. Pityriasis rubra pilaris (type 1, classic adult) Orange-reddish papules have coalesced to near erythroderma, sparing isolated islands of normal skin. Also note involvement of the hands in this 55-year-old woman.
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