MILITARY MEDICINE, 181, 3:e298, 2016

Pityriasis Rubra Pilaris in a 3-Year-Old Male

CPT Stephen K. Stacey, MC USA*; Steven J. Novek, MD†; LTC Craig L. Maddox, MC USA‡

ABSTRACT A 3-year-old male presented with progressive pruritic red–orange plaques across most of his body with
erythema, desquamation, and fissuring of the hands and feet. He was diagnosed with classic juvenile (type III) pityriasis
rubra pilaris (PRP) and treated with oral isotretinoin for 6 months. His skin findings resolved quickly during the treatment
period, with residual postinflammatory hypopigmentation resolving within a year. PRP is rare in pediatric patients and
standard recommended treatment algorithms for this population are not currently available. Diagnostic and treatment
guidelines for PRP are based almost exclusively on case reports or case series, most of which focus on adult patients.
The presentation, evaluation, and management of PRP are discussed.

INTRODUCTION face. He was also given hydroxyzine and various emollients

CASE
A 3-year-old male was brought to a military treatment facility
by his mother to see his primary care pediatrician. The
patient’s mother was concerned about a new rash he was
forming. He presented with a 4-day history of erythematous
lichenified plaques on the palms and soles in addition to
intensely pruritic well-demarcated red–orange plaques with
islands of sparing and overlying scale spread across his
elbows and knees (Figs. 1 and 2). He was also noted to have
mild desquamation of the upper lip, nose, and ears along with
pinpoint erythematous papules across his trunk. His past med-
ical history was notable only for mild xerosis cutis, while
family history and review of systems were noncontributory.
An active duty dermatologist at the same military treatment
facility was consulted, who arrived to primary care to assist
with management. A punch biopsy was performed of the
right elbow and the patient was prescribed desonide and
ammonium lactate as well as hydroxyzine for pruritus.
Despite initial therapy, over the following several days the
plaques spread throughout his face, scalp, torso, and limbs.
His hands and feet developed a yellow–red waxy appearance
with desquamation, fissuring, and mild edema (Figs. 3 and 4).
The pathology report from the punch biopsy revealed mild
acanthosis and parakeratosis. Direct immunofluorescence
staining for immunoglobulin M, immunoglobulin G, immu-
noglobulin A, complement C3, and Fibrin was performed with
negative results, suggesting against erythema multiforme, or
other autoimmune disorders. He was diagnosed with pityriasis
rubra pilaris (PRP) and started on oral isotretinoin 15 mg daily
(approximately 1 mg/kg). He continued to be treated with
twice daily topical steroids including triamcinolone 0.1%
ointment for the trunk and extremities, clobetasol 0.05%
foam for the scalp, and desonide 0.05% ointment for the
*1-503 IN(ABN), 173D IBCT(A), CMR 427 Box 2962, APO AE 09630.
†U.S. Army Health Center-Vicenza, CMR 427, APO AE 09630.
‡Department of Clinical Specialties and Dermatology Services, U.S. Army
Health Center-Vicenza, CMR 427, APO AE 09630.
doi: 10.7205/MILMED-D-15-00316
for control of symptoms.
The duration of isotretinoin therapy was 6 months. The
papules and plaques resolved within a few weeks. The patient
began to develop easy sloughing of the skin with minor
trauma, but overall his symptoms were well controlled. He
suffered no adverse reactions to isotretinoin other than xerosis
cutis. Complete blood count (CBC), liver function tests, and
lipid profile were within normal limits at onset of treatment
as well as during reassessment 3 months later. At the end of
the treatment period, all signs and symptoms had resolved
and the only remnants of his condition were areas of post-
inflammatory hypopigmentation that slowly resolved over
the next year (Figs. 5 and 6). The patient has had no recur-
rence of PRP after 3 years of follow-up.
DISCUSSION
PRP is a clinically heterogeneous skin disease that is rela-
tively uncommon. Diagnostic and treatment guidelines for
PRP are based almost exclusively off of case reports or case
series, most of which focus on adult patients. Our patient
was diagnosed with classic juvenile PRP (type III). The dis-
ease in our patient followed a fairly typical course with reso-
lution following isotretinoin therapy.
PRP is divided into six types, with type I (classic adult)
being the most well-described. It first became recognized as
a distinct illness in the mid-1800s. Before that, diseases with
characteristics similar to PRP had been described as variants
of psoriasis.
The incidence and prevalence of PRP are not precisely
known, though the disease has been estimated to be present
in approximately 1/5,000 patients presenting with a skin dis-
order.1 It has a bimodal age distribution, being most common
in the first and fifth decades of life. Men and women appear
to be equally affected by PRP and it occurs in all races. It
may occur in the setting of viral illnesses, superficial injury
or burn, malignancy, or seronegative arthritis.2,3 A severe
form of PRP may arise in patients with human immunodefi-
ciency virus (HIV).2
PRP shows significant clinical diversity, with six subtypes
defined by age of onset, distribution and appearance of

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FIGURE 1. The patient at presentation.

FIGURE 3. The patient 10 days after presentation.

(islands of sparing). Common associated findings include fol-

FIGURE 2. The patient at presentation.
lesions, and presence of HIV. The classic presentation of PRP
(types I and III) is diffuse red–orange scaling papules and
plaques that spread in a cephalocaudal progression. The lesions
coalesce extensively with focal areas of normal-appearing skin
licular keratoses, erythroderma, and palmoplantar keratoderma,
which may result in painful fissures on the hands or feet.2,3
Patients infrequently complain of pruritus. Maximum spread
is typically within 2 to 3 months. Mucosal involvement is
rare.4 Other forms of PRP are outlined in Table I.2,3
Diagnosis of PRP is based on clinical recognition con-
firmed by typical histopathological findings. A detailed history
should be obtained to help rule out other causes of the patient’s
symptoms. The differential diagnosis includes psoriasis, kera-
tosis pilaris, atopic dermatitis, ichthyoses, lichen spinulosus,
and other conditions, which can cause erythroderma such as
drug eruptions.2,5
The evaluation of suspected PRP should begin with skin
biopsy. Findings on biopsy may include follicular plugging,
increased granular cell layer, acantholysis, orthokeratosis,
and parakeratosis.4,6 Basic laboratory tests may be obtained to
evaluate possible coexisting conditions such as malignancy as
well as to establish a baseline for monitoring during therapy.
Such laboratory tests include CBC with differential and com-
plete metabolic profile, with fasting lipids added if isotretinoin

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TABLE I. Classification of PRP

Type % of cases Clinical Characteristics Prognosis

Type I: Classic Adult
Type II: Atypical Adult
Type III: Classic Juvenile
Type IV: Circumscribed Juvenile
Type V: Atypical Juvenile
Type VI: HIVAssociated
50 Diffuse red–orange scaling plaques with Typically resolves within 3 years
cephalocaudal spread and islands of sparing,
palmoplantar keratoderma, follicular
keratoses, erythroderma
5 Ichthyosiform lesions, palmoplantar Chronic
hyperkeratosis, alopecia
10 Similar to classic adult, usual onset within first Typically resolves within 1–2 years
2 years of life
25 Focal hyperkeratotic follicular papules on knees May resolve within a year or
and elbows, palmoplantar hyperkeratosis become chronic
5 Ichthyosiform dermatitis, follicular and Chronic
palmoplantar hyperkeratosis
Increasing Similar to type I, also with nodulocystic acne Chronic, poor prognosis
and lichen spinulosus

therapy will be used.7 Considering the association between
type-VI PRP and HIV, it is prudent to obtain an HIV test,
especially if the patient reports risk factors for HIV, the
patient also presents with new-onset nodulocystic acne, or if
the disease is refractory to standard therapy. There are no spe-
cific laboratory abnormalities or serologic markers for PRP.
There are no treatments that are universally effective for
PRP and evidence for the use of accepted therapies stems
primarily from case reports and case series, few of which deal
directly with treatment of children. Systemic retinoids are
considered first-line therapy for PRP and treatment for adults
may begin with 1 mg/kg daily isotretinoin or 0.5 mg/kg
daily acitretin.3,7,8 Treatment of children is less commonly
described with no single agent showing consistent benefit,
though treatment with isotretinoin at 1 mg/kg was highly
effective in our patient. It should be noted that acitretin is
teratogenic for up to 3 years after discontinuation of therapy,
and is therefore not recommended for women of childbear-
ing age. Effects of treatment are usually realized within 90 to
180 days.2
Methotrexate is considered a second-line agent, and has
been reported to be effective when used in combination with
oral retinoids for difficult cases.3,7 Other therapies which have
been employed with varying success include tumor necrosis
factor-alpha inhibitors, cyclosporine, and azathioprine. HIV-
associated PRP is generally refractory to standard therapy.
In these patients, initiation of antiretroviral therapy may
resolve symptoms.9

FIGURE 4. The patient 10 days after presentation. FIGURE 5. The patient after completion of isotretinoin therapy.

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FIGURE 6. The patient after completion of isotretinoin therapy.
Symptomatic relief should be offered with topical emol-
lients.7 If pruritus is present, the patient may be offered oral
antihistamines. Medium to high-potency topical steroids may
be used, though they are unlikely to alter the overall course
of disease unless the patient has limited skin involvement
(such as in type IV).10
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HIGHLIGHTS
— PRP is a rare skin disorder typically characterized by
diffuse orange–red scaling plaques with palmoplantar
keratoderma and follicular keratosis.
— Diagnosis is based off of clinical recognition and con-
firmed by biopsy. Obtaining a CBC, comprehensive
metabolic panel, and HIV test is also recommended.
— Systemic retinoids are the mainstay of therapy. Symp-
tomatic relief including emollients and possibly topical
steroids should also be provided.
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