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Published classification schemata for pityriasis rubra pilaris

Published classification schemata for pityriasis rubra pilaris
Classification Description
Davidson[1]
Familial versus acquired Familial is present in family members and usually onset before 1 year of age.
Childhood versus adult onset Age of onset before or after 18 years of age.
Typical versus atypical Atypical lacks keratotic and follicular lesions and has erythroderma with islands of sparing and palmoplantar keratoderma.
Griffiths[2], Griffiths[3], and Miralles[4]
Type I classical adult Most common subtype in adults. Cephalocaudal spread of follicular hyperkeratosis progressing to erythroderma with palmoplantar hyperkeratosis. May resolve spontaneously after a few years.
Type II atypical adult Rare subtype in adults. Ichthyosiform scale on legs, eczematous areas of skin, sparse scalp hair. Prolonged disease course.
Type III classical juvenile Less common subtype in children. Similar to classical adult pityriasis rubra pilaris, appears in the first few years of life. May resolve spontaneously in less than 1 year.
Type IV circumscribed Most common subtype in children. Sharply demarcated areas of follicular hyperkeratosis on the knees and elbows.
Type V atypical juvenile Rare subtype in children. May be familial. Follicular hyperkeratosis with less erythema. Chronic course.
Type VI HIV-associated Concurrent HIV or AIDS. Classical features may be accompanied by acne conglobata, hidradenitis suppurativa, and lichen spinulosus.
Gelmetti[5]
Acute Resolves in less than 6 months. Approximately 50% of children.
Acute with prolonged course Resolves within 6 to 12 months.
Chronic Present for more than 1 year; half of children had resolution with a mean of 3.2 years, half with no resolution.
Piamphongsant[6]
Type I Children more commonly than adults. Palmoplantar keratoderma only on presentation, approximately half of patients progress to types II to IV.
Type II Children more commonly than adults. Palmoplantar keratoderma and circumscribed patches with follicular plugging on elbows and knees.
Type III Similar incidence in adults and children. Palmoplantar keratoderma and patchy involvement on face and trunk with adjacent, follicular plugging.
Type IV Adults more than children. Palmoplantar keratoderma with erythroderma.
Type V Rare. Palmoplantar keratoderma with contracture and sclerodermoid-like skin.
References:
  1. Davidson CL Jr, Winkelmann RK, Kierland RR. Pityriasis rubra pilaris. A follow-up study of 57 patients. Arch Dermatol 1969; 100:175.
  2. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol 1980; 5:105.
  3. Griffiths WA. Pityriasis rubra pilaris: the problem of its classification. J Am Acad Dermatol 1992; 26:140.
  4. Miralles ES, Nunez M, De Las Heras ME, et al. Pityriasis rubra pilaris and human immunodeficiency virus infection. Br J Dermatol 1995; 133:990.
  5. Gelmetti C, Schiuma AA, Cerri D, Gianotti F. Pityriasis rubra pilaris in childhood: a long-term study of 29 cases. Pediatr Dermatol 1986; 3:446.
  6. Piamphongsant T, Akaraphant R. Pityriasis rubra pilaris: a new proposed classification. Clin Exp Dermatol 1994; 19:134.
Graphic 131805 Version 1.0

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