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. |
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