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Pityriasis rubra pilaris: a clinical review.

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 Disease Management
Pityriasis Rubra Pilaris: A Clinical Review
David Jerard Pincus
Pityriasis rubra pilaris is a
skin condition with many
different clinical presentations.
History, histology, clinical
presentation, its different
classified forms, treatments,
and differential diagnoses are
reviewed.
David Jerard Pincus, MD, is a Surgical
Resident, Jackson Memorial Hospital, Miami,
FL.
448
ityriasis rubra pilaris (PRP) is a
P red scaly eruption character-
ized by the association of pal-
moplantar keratoderma, follic-
ular plugging, and erythematous peri-
follicular papules which may progress
to plaques or erythroderma (White,
2003). Most cases are sporadic and
acquired but a familial form may exist
which is transmitted by an autosomal
dominant or autosomal recessive
mechanism.
This disease was first described
by Devergie in 1856 who character-
ized PRP as a follicular eruption
and noted its association with three
other cutaneous disorders: psoriasis
palmaris, pityriasis capitis, and
pityriasis rubra. In 1877, Richaud
recognized that all of the manifesta-
tions were of the same disorder. In
1889, Besner added the epithet
rubra (White, 2003).
Onset
PRP exhibits a bimodal distri-
bution of age of onset. It first peaks
in early childhood and the second
peak comes after the 5th decade.
Onset in children frequently follows
trauma or infection such as strepto-
coccal. There is spontaneous resolu-
tion of PRP in at least 75% of cases
within 3 months to 7 years of onset.
Relapses are uncommon, but may
occur. Familial PRP does not
resolve spontaneously, although
many cases that have been so classi-
fied may represent other ichthyosi-
form disorders. PRP affects both
male and females equally, but it is
uncommon in the general popula-
tion (Arnold & Buechner, 2004).
PRP is a rare disease, but one study
DERMATOLOGY NURSING/December 2005/Vol. 17/No. 6
reports a frequency of about 1 case
in every 500 new pediatric patients
with a dermatologic disease
(Vijayalakshmi & Mallika, 2003).
The causes of PRP remain
unknown. The epidermis is in a
hyperkinetic state, with a rapid
turnover rate of the follicular ker-
atinocytes, and may approach the
rate found in psoriasis. There is a
resemblance of the histology to that
found in phrynoderma, a distinctive
form of follicular hyperkeratosis
associated with nutritional deficien-
cy with characteristic hyperkeratot-
ic papules that first appear on the
extensor surfaces of the extremities,
shoulders, buttocks. The finding of
clinical follicular occlusion suggests
that a deficiency or malfunction of
vitamin A might be involved
(White, 2003). A decrease in retinol
binding protein is also reported.
Retinol binding protein is the spe-
cific carrier of vitamin A and its
decreased synthesis is a biochemical
marker for PRP. Although decreased
retinol binding protein is a marker
for PRP, it is not proven that
decreased vitamin A is a cause. It is
more likely due to the decreased
serum level of the carrier of vitamin
A (Evangelou, Murdoch, Pala-
maras, & Rhodes, 2005).
Clinical Findings
The initial clinical findings of
PRP may be redness and scaling of
the face and scalp. Skin on the
palms and soles may become quite
thickened, painful, and orange in
color. The patient may experience
debilitating hyperkeratosis. The
 Figure 1.
There is psoriasiform hyperplasia
of the epidermis with alternating
areas of orthokeratosis and
parakeratosis and plugs of the
follicular infundibulum.
skin becomes progressively redder,
and painful fissuring of the palms
and soles may occur. While an exfo-
liative erythroderma may ensue,
characteristic “skip” areas of non-
involvement are usually seen.
Rarely patients may complain of
fever, chills, and malaise (Arnold &
Buechner, 2004). The most com-
mon affected sites are the palmo-
plantar surfaces, elbows, and knees
(75%-80%); dorsa of the hands and
feet (60%); and the face (40%)
(Vijayalakshmi & Mallika, 2003).
The most characteristic clinical
feature is a follicular verrucous
papule about 1 mm in diameter
with a central keratotic plug. A yel-
low-orange ring surrounds the
lesion (Vijayalakshmi & Mallika,
2003). The second most important
diagnostic element is a palmoplan-
tar keratoderma, which is salmon
colored and edematous. The
Achilles tendon area is frequently
involved. As stated previously,
there is a cephalic rash. The hair
and teeth are not involved but occa-
sionally psoriasiform nail changes
may occur. White lacy plaques may
DERMATOLOGY NURSING/December 2005/Vol. 17/No. 6
Figure 2.
A sparse, superficial, perivascular lymphocytic infiltrate is also noted.
be observed on the buccal mucosa
(Vijayalakshmi & Mallika, 2003).
The histological changes seen
in PRP are consistent but not spe-
cific. There is acanthosis with blunt-
ing of rete pegs and suprabasal per-
inuclear vacuolation in isolated
cells. Liquefactive degeneration of
the basal layer is most likely to be
seen overlying the dermal papillae.
The granular cell layer is attenuated
in some areas and thickened in oth-
ers. Mild superficial vasodilatation
of capillaries with a slight to moder-
ate lymphohistiocytic perivascular
infiltrate is found (White, 2003).
Examination of a follicular papule is
most helpful in the diagnosis.
Parakeratosis of the follicular ostea
associated with follicular hyperker-
atosis is seen. Hypertrophy of the
errector pillae is a helpful diagnostic
feature when present. A dermal
perivascular mononuclear cell infil-
trate is present and there are occa-
sional mononuclear cells (see
Figures 1 & 2) (Arnold & Buechner,
2004).
The presence of PRP has occa-
sionally been associated with
immunodeficient states, such as
HIV. In the juvenile forms of this
disease, PRP may be associated
with T-helper cell impairment,
hypogammaglobulinemia, and de-
creased IgA. Underlying diseases
are unusual, but PRP may also be
associated with HIV, hypothy-
roidism, myasthenia gravis, celiac
disease, and acute stem cell
leukemia.
Patients with pityriasis rubra
pilaris can be classified into five types,
according to Griffith’s Classification
(Griffiths, 1975), which differ from
each other on the basis of clinical fea-
tures, age of onset, and prognosis
(see Table 1).
Type I
Classical adult onset PRP
accounts for over half of the
patients. The disease starts out with
a single erythematous patch on the
upper half of the body and within a
few weeks or months, there are large
zones of follicular hyperkeratosis
each with an erythematous perfollic-
ular halo. Islands of unaffected skin,
one to several centimeters in diame-
ter remain scattered over the sheet
of erythema. The scaling is fine and
powdery. On the lower body the
scaling is coarser (see Figures 3, 4, &
5) (Griffiths, 1975).
On the face and scalp, oral vita-
min A (150,000 to 300,000 IU daily)
has minimal improvement among
patients (White, 2003). Vitamin A
storage in the liver has the potential
side effects of teratogenicity and ele-
vated triglycerides (Schachner &
Hansen, 2003). The retinoids,
isotretin, etretinate, and acitretin
appear to be more effective than
vitamin A with over 80% successful
clearance in 3 years (Schachner &
Hansen, 2003). It seems that early
449
 Figure 3. Figure 4. Figure 5.
Fine, powdery scale Large zones of follicular Islands of unaffected skin
hyperkeratosis

Table 1. the legs. Sparseness of the scalp hair

Classifications, Descriptions, and Treatments for is sometimes present. Palmoplantar
hyperkeratosis is coarse and lamel-
Pityriasis Rubra Pilaris
lated (Griffiths, 1975). Patients must
Type Age Clinical Description Treatment be treated on a case by case basis.
Adult Large zones of follicular hyperkeratosis with Retinoids, Type III
onset an erythematous halo. Islands of unaffected isotretinoin,
I skin covering the erythematous sheets. etretinate, Classical juvenile PRP affects
acitretin 10% of patients. It resembles type I
in its mode of onset and appear-
Adult Atypical, long duration, increased scaling. Treated on ance, but it affects children in the
II onset Sparseness of scalp hair. Increased case by case
first year of 2 or life (see Figures 6-
palmoplantar hyperkeratosis. basis
7) (Griffiths, 1975). Sixty percent of
1 - 2 years Resembles Type I PRP. 90% self-limited in 3 Keratolytics, cases self-limit in 1 year, while 90%
years. topical resolve in 3 years. Topical treat-
III steroids, ments with keratolytics, mild topical
emollients
steroids, and emollients are pre-
Pre- Sharply demarcated areas of follicular Systemic ferred over systemic treatments in
pubertal hyperkeratosis and erythema on knees and retinoic acids, pediatric patients with PRP
IV children elbows. topical steroids (Schachner & Hansen, 2003).

Type IV
First years Chronic. Follicular hyperkeratosis. Erythema Retinoids,
of life not prominent. Most cases of familial PRP. methotrexate, Circumscribed PRP affects
cyclosporins, approximately 25% of patients. It
V azathioprine, affects prepubertal children and it is
gamma- characterized by sharply demarcated
interferon areas of follicular hyperkeratosis and
erythema on the knees and elbows.
Hyperkeratosis may be found overly-
diagnosis and early treatment with Type II ing bony prominences. Circum-
retinoids leads to the best chance for Atypical adult onset affects scribed PRP shows no tendency
clearing. If there is a lack of about 5% of patients. This disease is towards progression (Griffiths, 1975).
response or contraindications to the atypical because of its long duration Systemic retinoic acid works quite
use of retinoids, methotrexate could of 20 years or more and atypical well with type IV, especially when
be used (Schachner & Hansen, morphologic features. The scaling is alternated with a topical steroid
2003). more ichthyosiform, especially on (Schachner & Hansen, 2004).

450 DERMATOLOGY NURSING/December 2005/Vol. 17/No. 6

 Figure 6.
Multiple pinkish plaques over the
chest, abdomen, and upper arm
with areas of normal skin in
between lesions.
Figure 7.
Hyperkeratosis of palms
Type V
Atypical juvenile PRP affects
only 5% of patients. It appears in
the first years of life and runs a
chronic course. It is characterized
by follicular hyperkeratosis. Ery-
thema is a constant but not promi-
nent feature. Most cases of familial
PRP belong to this group. Systemic
therapy is largely utilized because
spontaneous remission is unlikely
(Griffiths, 1975). The most com-
monly used systemic agents are
retinoids, methotrexate, cyclo-
sporins, azathioprine, and gamma-
interferon (Schachner & Hansen,
2003).
Studies have shown that
patients who present with one form
DERMATOLOGY NURSING/December 2005/Vol. 17/No. 6
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of PRP have the capability to trans-
form into another form of the dis-
ease. In one study in particular, a
patient presented with type III but
then later went on to develop type
IV. This may suggest a common eti-
ological mechanism for all types of
PRP (Gelmetti, Schiuma, Cerri, &
Gianotti, 1986; Shahidullah &
Aldridge, 1994).
The classic adult type I has the
best prognosis with 80% clearing
within 3 years. Most children with
PRP may be treated with topical
therapy alone. Some forms of topi-
cal therapy include emollients, cal-
cipotriene, retinoic acid, keratolyt-
ics, topical steroids, and hydrating
agents. Systemic therapies include
retinoids (acitretin, etretinate,
isotretinoin), methotrexate, cyclo-
sporin, and psoralen ultraviolet A
(PUVA). Other agents being used,
but none that can be recommend-
ed, include vitamins C and E,
stanozolol, arsenic, and pilocarpine
(Cohen & Prystowsky, 1989; Finzi,
Altomare, Bergamaschini, & Tucci,
1981).
Two other classifications sys-
tems are also used for PRP.
Gelmetti’s Classification (1986) is
based on the duration of the dis-
ease. Piamphogsant’s Classification
(1994) is based on physical findings
and it is divided into four types.
Conclusion
Pityriasis rubra pilaris is an
important disease to be aware of
and understand. The differential
diagnosis includes psoriasis, sebor-
rheic dermatitis, phrynoderma,
atypical keratosis pilaris, follicular
eczema, and erythrokeradermas
(for example, Unna-Thost disease
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and Papillion-Lefevre syndrome).
PRP appears in different clinical
presentations but there are treat-
ments available for all of them.
When treating a patient with PRP, it
is important to identify which type
is presenting and treat accordingly.
Fortunately, the majority of patients
presenting with PRP have a favor-
able prognosis.
References
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Circumscribed juvenile pityriasis rubra
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