Acta Dermato-Venereologica 98-10CompleteContent | Page 14
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SHORT COMMUNICATION
A Case of Pityriasis Rubra Pilaris Treated Successfully with the Phosphodiesterase-4 Inhibitor Apremilast
Maria CHO 1 , Tetsuya HONDA 1 *, Chiyuki UESHIMA 2 , Tatsuki KATAOKA 2 , Atsushi OTSUKA 1 and Kenji KABASHIMA 1
1
Department of Dermatology, Kyoto University Graduate School of Medicine, 54 Shogoin-Kawahara, Sakyo, Kyoto 606-8507, and 2 Department
of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan. *E-mail: [email protected]
Accepted Jun 14, 2018; Epub ahead of print Jun 25, 2018
Pityriasis rubra pilaris (PRP) is an inflammatory skin
condition characterized by well-circumscribed hyperke-
ratotic plaques and palmoplantar keratoderma (1). The
aetiology of PRP is unclear and although various treat-
ment options, such as topical steroids and oral retinoic
acids, are available, treatment responses are inconsistent
and management is often difficult (2). We report here a
case of PRP that was treated successfully with apremilast,
a phosphodiesterase-4 (PDE4) inhibitor. Furthermore,
using immunohistochemistry we examined the expres-
sion of nuclear factor of kappa light chain enhancer in B
cells (NF-κB) and caspase recruitment domain family,
member 14 (CARD14), a molecule that induces acti-
vation of NF-κB and is reported to be involved in the
pathogenesis of PRP in the skin.
CASE REPORT
A 60-year-old woman with no significant medical or
family history attended our clinic 4 years ago with a
1-month history of well-demarcated, pruritic erythema
keratodes on the hands, soles of the feet, elbows, and
knees (Fig. 1a). Keratinizing papules on the palms, and
scales on the dorsa of the hands and nail beds, were also
seen. No nail-fold capillary abnormalities or nail changes
were evident, and she had no systemic symptoms. Skin
biopsy at initial presentation showed a “chequerboard
pattern” of alternating parakeratosis and orthokerato-
sis, irregularly elongated rete ridges, acantholysis, and
dermal melanin incontinence (Fig. 2a). Based on the
clinical and histological findings, she was diagnosed
with type I PRP. She developed mild arthralgia 5 months
later and was referred to the rheumatology department.
Fig. 1. Clinical presentation of pityriasis rubra pilaris before and
after treatment with apremilast. (a) Keratinization and erythema
were seen on both feet and knees at initial presentation. (b) Significant
improvements in the skin lesions were seen at 6 months after the start of
treatment with apremilast.
Her autoantibody screening showed an elevated anti-
centromere antibody and limited cutaneous systemic
sclerosis was diagnosed, but no additional treatments
were started by the rheumatologists. Over the following
4 years all strengths of topical steroid ointments, topical
vitamin D 3 analogues and salicylic acid, oral retinoic
acid, and phototherapy were tried. Other treatments,
such as methotrexate, were not considered due to pos-
sible side-effects. Although mild improvements were
seen throughout the treatment period, the skin lesions
always recurred and never completely resolved. As the
symptoms were refractory, our treatment options were
limited and we decided to start apremilast at 30 mg/day.
Although no substantial changes in the hyperkeratosis
of the feet were seen one month after the introduction of
apremilast, significant improvements were seen after 2
months. Currently, the skin lesions have not shown any
Fig. 2. Histological presentation and immunohistochemical staining of pityriasis rubra pilaris for p65 and CARD14. (a) Haematoxylin and
eosin staining of the skin biopsy shows the classic “chequerboard appearance” of alternating parakeratosis and orthokeratosis (original magnification
×5; scale bar=500 mm). (b) Skin biopsy stained for p65 shows nuclear staining in keratinocytes (white circle) and in inflammatory cells surrounding the
vasculature (black square). (original magnification ×10; scale bar=250 mm). (c) Skin biopsy stained for CARD14. Weak expressions of CARD14 were
seen in keratinocytes (white circle) and in dermal inflammatory cells around vessels (black square) (original magnification ×10; scale bar=250 mm).
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2018 Acta Dermato-Venereologica.
doi: 10.2340/00015555-2995
Acta Derm Venereol 2018; 98: 975–976