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Adolescent with papules, waxy hyperkeratosis on palms and soles
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A 14-year-old female presents to your clinic with a new skin eruption of 3 months duration. The parents are highly concerned with the rapid evolution of the rash. They report that the patient was well until about a month ago, when she had a brief upper respiratory infection that resolved without incident. Another dermatologist diagnosed psoriasis and treated her with high and mid-potency steroids and topical calcipotriol cream.
On exam, the patient seems occasionally pruritic, but otherwise appears well. Her palms and soles were uniformly thickened and salmon-colored with plaques ending abruptly at the wrists and ankles and extending over to the tops of the hands and feet (Figures 1 and 2). There were a few red, scaly plaques on her lower legs. After 6 months of treatment with tazarotene 0.05% cream (a topical retinoid) and clobetasol dipropionate cream (a high-potency topical steroid), she had significant improvement (Figures 3 and 4).
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Figure 1: Her palms and soles were uniformly
thickened and salmon-colored with plaques ending abruptly at the wrists and
ankles and extending over to the tops of the hands and feet. |
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Figure 2 |
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Figure 3 |
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Figure 4 |
What is the most likely etiology?
Diagnosis: Pityriasis rubra pilaris, type IV (circumscribed)
Pityriasis rubra pilaris (PRP) is an uncommon skin condition seen in both children and adults and thought to be a result of abnormal keratinization. There are several types of PRP, classified as type I to type V and subdivided into adult and juvenile forms. The etiology of PRP is unknown but is thought to be related to a preceding infection (often streptococcal) or trauma, especially in children with this condition. There are also several reports of autoimmune diseases associated with PRP, suggesting a possible immunologic role. Others have suggested a possible role for vitamin A deficiency or abnormal metabolism, as PRP often responds well to topical and systemic retinoids (vitamin A-derived therapies). However vitamin A levels are usually normal.
Type I and type II PRP are limited to adults and are referred to as “classical” and “atypical,” respectively. Types III, IV and V are limited to children and include “juvenile classical,” “juvenile circumscribed” and “juvenile atypical,” respectively. The classical and atypical forms are similar in adults and children. Most cases of PRP are acquired; however, there are many reports of familial PRP. Familial PRP tends to have a more gradual onset and may persist throughout life.
The classical form (type III) presents with onset usually between 5 and 10 years of age. Erythematous scaly macules progress to orange-pink papules with hyperkeratosis due to follicular plugging that coalesce to form extensive plaques. There is often normal skin between plaques characteristic of this eruption and referred to as “islands of sparing.”
The texture of the skin is often compared to the feel of a nutmeg grater. The eruption usually begins on the scalp, face, neck and upper trunk and progresses in a cephalocaudal direction during a period of weeks. The palms and soles develop red-orange color and waxy hyperkeratosis that end abruptly at the dorsum aspect of the hands and feet, as well as the wrists. The nails may become discolored and thickened with underlying debris and onycholysis. The eruption tends to last 1 to 2 years and then often remits. It is possible to begin with classical PRP (type III) and evolve into other types. In addition, many children do not fit one type.
The circumscribed form (type IV) occurs later in the first decade and has similar morphologic findings, but is often limited to the elbows and knees. Patients also develop waxy hyperkeratosis of the palms and soles in time. There may be occasional involvement elsewhere. The course of type IV is less predictable than type III, tends to wax and wane, and may last several years, usually resolving late in adolescence.
The atypical form (type V) is the least common type, usually occurs at birth or in the first few years of life and may be familial. It is characterized by erythema and hyperkeratosis, as well as waxy hyperkeratosis of the palms and soles. Patients may also develop scleroderma-like changes of the digits. Atypical PRP is usually resistant to therapy but eventually clears on its own.
The differential diagnosis includes plaque-type psoriasis, especially during the early stages, and follicular psoriasis in later stages, follicular ichthyosis, erythrokeratodermias, hereditary palmoplantar keratodermas, erythroderma (diffuse erythema and scale) due to other etiologies such as atopic dermatitis, psoriasis, drug eruptions or cutaneous T-cell lymphoma, and Darier’s disease. Diagnosis is usually made by clinical appearance with skin biopsy for confirmation when needed.
Treatment for PRP can be challenging. Patients may respond to bland emollients, keratolytic agents (urea, salicylic acid, lactic acid preparations), topical steroids, topical calcineurin inhibitors or topical retinoids. Patients with more extensive and recalcitrant disease often respond to oral retinoids such as isotretinoin. However, due to the extensive monitoring and risk for adverse effects, oral retinoids are limited to severe symptomatic cases and should be prescribed by a physician comfortable with close monitoring and managing possible adverse effects.
Other systemic immunosuppressive therapies and biologic agents occasionally work but are generally less successful than oral retinoids. Phototherapy may also be successful; however, some patients with PRP are photosensitive.
For more information:
- Klein A. Am J Clin Dermatol. 2010;11:157-170.
- Sehgal VN. Int J Dermatol. 2006;45:438-446.
- Yang CC. J Am Acad Dermatol. 2008;59:943-948.
Marissa J. Perman, MD, is a pediatric dermatology fellow at The Children’s Hospital of Philadelphia. Disclosure: Dr. Perman reports no relevant financial disclosures.
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