Pityriasis rubra pilaris presenting with an abnormal autoimmune profile: two case reports.

Abstract

Pityriasis rubra pilaris is an uncommon inflammatory and hyperproliferative dermatosis of juvenile or adult onset. The etiology of the disease is still unknown.
We present the cases of two Caucasian men aged 53 and 48 who presented with pityriasis rubra pillaris type 1; both patients also exhibited an abnormal immunological profile.
Pityriasis rubra pillaris is currently classified as a keratinization disorder. The abnormal immunological profile reported in our patients along with the comorbidity of pityriasis rubra pilaris with autoimmune disorders reported in the literature poses the question of a possible pathogenetic role for the immune response in this disorder.

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BioMed Central
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Journal of Medical Case Reports
Open Access
Case report
Pityriasis rubra pilaris presenting with an abnormal autoimmune
profile: two case reports
Stamatis Gregoriou*1, Zoe Chiolou2, Christina Stefanaki1, Niki Zakopoulou2,
Dimitrios Rigopoulos1 and George Kontochristopoulos2
Address: 1University of Athens Medical School, Department of Dermatology, Athens, Greece and 22nd Department of Dermatology, Andreas
Sygros Hospital, Athens, Greece
Email: Stamatis Gregoriou* - stamgreg@yahoo.gr; Zoe Chiolou - chiolouz@gmail.com; Christina Stefanaki - cstefana@otenet.gr;
Niki Zakopoulou - zakonico@acn.gr; Dimitrios Rigopoulos - drigop@hol.gr; George Kontochristopoulos - gkontochristo@gmail.com
* Corresponding author
Abstract
Introduction: Pityriasis rubra pilaris is an uncommon inflammatory and hyperproliferative
dermatosis of juvenile or adult onset. The etiology of the disease is still unknown.
Case presentation: We present the cases of two Caucasian men aged 53 and 48 who presented
with pityriasis rubra pillaris type 1; both patients also exhibited an abnormal immunological profile.
Conclusion: Pityriasis rubra pillaris is currently classified as a keratinization disorder. The
abnormal immunological profile reported in our patients along with the comorbidity of pityriasis
rubra pilaris with autoimmune disorders reported in the literature poses the question of a possible
pathogenetic role for the immune response in this disorder.
Introduction
Pityriasis rubra pilaris (PRP) is an uncommon hyperkera-
totic, papulosquamous disease, classified into five groups
subject to clinical appearance, age of onset and prognosis
[1]. Recently, a sixth group has been proposed in acknowl-
edgment of the HIV-associated type of PRP. The etiology
of the disease remains unknown but several studies have
reported an association of PRP with other autoimmune
disorders [2-4]. We present the cases of two patients with
type 1 PRP who presented with abnormal autoimmune
profiles.
Case presentation
Case report 1
A 53-year-old Caucasian man presented with a two-week
history of slightly scaly pruritic erythematous plaques
with an orange hue that covered his face (Figure 1), the
extensor aspects of his arms, forearms and legs, upper
trunk, buttocks and flexures. Patches of normal skin were
evident within those sheets of erythema, together with
prominent erythematous follicular papules at the margins
of the plaques. His palms and soles were slightly hyperk-
eratotic with a yellowish hue. His past medical history was
unremarkable. He had no arthritis, did not report symp-
toms or present with clinical signs that could be attributed
to any autoimmune disorder. The results of his complete
blood count, urine analysis and blood chemistry profile
were unremarkable. Initially, antinuclear antigens (ANA)
were weakly positive (1:80), later rising to high titers
(1:1280) and showing a speckled pattern, whereas anti-
DNA, extractable nuclear antigen (ENA), anticardiolipin
antibodies and cryoglobulins were negative. C3 and C4
Published: 13 November 2009
Journal of Medical Case Reports 2009, 3:123doi:10.1186/1752-1947-3-123
Received: 22 November 2008
Accepted: 13 November 2009
This article is available from: http://www.jmedicalcasereports.com/content/3/1/123
© 2009 Gregoriou et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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were mildly elevated, but CH50 was normal. The patient
did not report any recent infection. Histopathology
showed orthokeratosis alternating with parakeratosis, a
normal granular layer, an absence of Munro microab-
scesses and dilatation of the dermal blood vessels with a
low-grade perivascular inflammatory infiltrate (Figure 2,
Figure 3). Both the clinical and histological pictures were
compatible with PRP and the patient was commenced on
acitretin 50 mg/day. Within 1 month, he had improved
remarkably and his skin had become almost clear. His
ANA titer had decreased to 1:640 after treatment.
Case report 2
A 48-year-old Caucasian man presented to our clinic with
a one-month history of pruritic, slowly expanding scaling
lesions over his face, scalp, upper trunk and the outer
aspects of his arms (Figure 4). His medical history was sig-
nificant for coronary disease and diabetes mellitus II. He
had no arthritis, did not report any symptoms and clinical
examination did not reveal signs that could be attributed
to any autoimmune disorder. Physical examination
revealed slightly scaling erythematous lesions over his
forehead, proximal anterior scalp, the nape of his neck,
face, forearms and upper trunk. The results of his com-
plete blood count, urine analysis and blood chemistry
profile were unremarkable. His ANA displayed a speckled
pattern and had an initial titer of 1:640 (negative >1:80)
which decreased to 1:80 positive during therapy. Ro (Sicca
syndrome A; SSA) antibodies were intensively positive
(145, 1 U, negative <20) and La (Sicca syndrome B; SSB)
antibodies were slightly positive (33 U, negative <20). C3
was mildly increased (223 mg/dL, normal: 84, 1-167 mg/
dL), whereas C4 and CH50 were normal. Anti-dsDNA,
anti-RNP, pANCA, cANCA anti-Sm antibodies, as well as
antibodies against histones and antibodies against cardi-
olipin were not identified. Direct immunofluorescence
from a sun-exposed lesion did not show immunoglobulin
or complement deposition. The patient did not report
arthralgias, myalgias or symptoms of any other system.
According to his immunological profile and his clinical
presentation, the patient was initially diagnosed as suffer-
Pityriasis rubra pilaris on the face of the first patient
Figure 1
Pityriasis rubra pilaris on the face of the first patient.
Hyperkeratosis, parakeratosis and acanthosis in the epider-mis of the first patient
Figure 2
Hyperkeratosis, parakeratosis and acanthosis in the
epidermis of the first patient.

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ing from subacute cutaneous lupus erythematosus
(SCLE). He was started on hydroxychloroquine 200 mg
per day, but the disease eruption extended to his trunk
and lower extremities. Gradually, his soles and palms
became intensively hyperkeratotic, salmon-colored and
he developed ectropion in both eyes. The histological
findings of two biopsy specimens as well as his clinical
picture were compatible with PRP showing alternate areas
of orthokeratosis and parakeratosis. There was no atrophy
of the epidermis. His hydroxychloroquine treatment was
discontinued and he was prescribed acitretin 35 mg/day
and prednisolone 40 mg daily tapered progressively. The
lesions improved only temporarily. Methotrexate 25 mg
per week intramuscularly was added and the patient
improved remarkably within 3 weeks. He is still on fol-
low-up. His SSA antibodies remained positive (130, 2 U,
negative <20) on a subsequent check while SSB antibodies
were within normal limits. C3 was slightly increased after
treatment (247 mg/dL, normal: 84, 1-167 mg/dL).
A diagnosis of PRP was made for both patients based on
clinical and histology data.
Discussion
Case studies of PRP associated with autoimmune disor-
ders including arthritis [2,5] and dermatomyositis [3]
have been reported. Circumscribed juvenile-onset PRP
was recorded in a patient with Down's syndrome and vitil-
igo [6]. PRP has also appeared simultaneously with SCLE,
isolated IgA deficiency,
hypothyroidism, celiac sprue, atopy, diabetes mellitus,
underlying malignancy myasthenia gravis and therapy
with agents known to disturb lymphoid function
[4,5,7,8].
hypogammaglobulinemia,
The first patient we describe had high titers (1:1260) of
ANA, while the second patient's ANA displayed a speckled
pattern and had an initial titer of 1:640 (negative >1:80)
which decreased to 1:80 positive during therapy. Ro (SSA)
antibodies were intensively positive (145, 1 U, negative
<20) and La (SSB) antibodies were slightly positive (33 U,
negative <20). C3 was mildly increased (223 mg/dL, nor-
mal: 84, 1-167 mg/dL). Boyd et al. have described a
patient who presented with simultaneous PRP and SCLE
and an ANA titer of 1:640 in a homogeneous pattern.
Antibodies to dsDNA, histones, Smith antigen, Ro (SSA),
La (SSB) and ribonucleoprotein were not identified.
Moreover, a skin biopsy specimen from a sun-exposed
lesion did not show immunoglobulin or complement
deposition [4]. Another patient described by Polat et al. as
having dermatomyositis displayed elevated serum creat-
ine kinase and aldolase and rheumatoid factor but was
negative for ANA, anti-DNA and anticytoplasmic antibod-
ies [3]. Patients with PRP with joint and muscle involve-
Lymphocytic infiltrate in the dermis (hematoxylin and eosin, ×250)
Figure 3
Lymphocytic infiltrate in the dermis (hematoxylin
and eosin, ×250).
Pityriasis rubra pilaris covering the back of the second patient
Figure 4
Pityriasis rubra pilaris covering the back of the sec-
ond patient.

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ment described in the literature had an unremarkable
immunological profile [2,5].
The widely reported association of PRP with autoimmune
disorders may point to an underlying abnormal immune
response to antigenic triggers or microbial pathogens [7].
Investigators have reported an enhanced spontaneous
activity of T-suppressor cells with an impairment of T-
helper cell functions in a 6-year-old child with PRP [5].
The therapeutic efficacy of azathioprine and calcipotriol
in PRP [7], both inhibitors of T-cell activation, as well as
the association of PRP with T-helper cell dysfunction [7]
and infections such as HIV [9] and hepatitis C [7] may
support the hypothesis of this immunologic abnormality.
Furthermore, patients with PRP have recently been man-
aged with biological treatments including anti-TNF anti-
bodies [10]. Given the response to anti-human TNF
monoclonal antibodies, TNF-α may play a pathophysio-
logical role in PRP.
Conclusion
PRP is currently classified among keratinization disorders.
A large meta-analysis of 26 PRP cases by Magro and Crow-
son that processed data from 250,000 dermapathology
cases, revealed six cases with autoimmune coexistent dis-
orders [7]. The authors conclude that a possible pathoge-
netic role for the immune response and its effect on
epidermal retinoid signaling pathways warrants further
investigation. Given the rarity of the disease, it is difficult
to establish the precise association of PRP with autoim-
mune disorders. Nonetheless, we suggest that clinicians
should look out for a coexisting autoimmune disorder or
abnormal immunological markers in PRP patients.
Patients' perspective
Patient 1
I never had any problems with my skin. I suddenly devel-
oped a rash that kept spreading from my face downwards
until it finally covered a significant part of my body. It was
itchy at times. My hands were hard (hyperkeratotic)
impeding the use of my fingers. I thought I had an allergy
from food or garden work. I initially thought it would
improve by itself using some over-the-counter products
from my pharmacist but when it persisted I was really
worried and went to the hospital. I was hospitalized in the
dermatology clinic for 20 days and received a drug called
Neotigason that greatly improved the problem and con-
tinued it at home. I'm fine now and still consult with my
doctors at A Sygros hospital.
Patient 2
This patient did not wish to contribute any comments.
Abbreviations
ANA: antinuclear antigen; ANCA: antineutrophil cyto-
plasmic antibody; ENA: extractable nuclear antigen; PRP:
pityriasis rubra pilaris; RNP: ribonucleoprotein; SCLE:
subacute cutaneous lupus erythematosus; SSA: Sicca syn-
drome A; SSB: Sicca syndrome B; TNF-α: tumor necrosis
factor alpha.
Consent
Written informed consent was obtained from the patients
for publication of this case report and any accompanying
images. A copy is available for review by the Editor-in-
Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
GS, SC, and RD were the doctors who hospitalized the
first patient and were responsible for diagnosis, laboratory
results and therapy while CZ, ZN, and KG were the doc-
tors responsible for the second patient. GS and KG were
responsible for the design of the article proposing the pos-
sible association between PRP and immunological abnor-
malities. All authors have contributed in writing portions
of the paper. All authors have read and approved the final
manuscript.
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