SHORT COMMUNICATION

Onycho-Pachydermatit with extensive bone marrow edemapredominant in the metacarpals: a ‘‘forme fruste’’ of POPP?

Hatice Tuba Sanal • Sedat Yilmaz •

Muhammet Cinar • Ismail Simsek •

Ayhan Dinc • Cem Tayfun

Received: 28 December 2010 / Accepted: 13 March 2011 / Published online: 30 March 2011

� Springer-Verlag 2011

Abstract Psoriatic onycho-pachydermo-osteo/periostitis

(POPP) syndrome is a rare form of psoriatic arthritis with a

combination of (i) psoriatic onychodystrophy, (ii) con-

nective tissue thickening, and (iii) periostitis of the distal

phalanges. The treatment of the condition has generally

been reported to be unsatisfactory with the traditional

regimes. Here, we describe a case whom we believe is one

presentation of POPP with extensive bone marrow edema

of metacarpal bones without distinctive periostitis.

Keywords Psoriatic arthritis �Psoriatic onycho-pachydermo-periostitis � POPP

Introduction

Psoriatic onycho-pachydermo-osteo/periostitis (POPP) syn-

drome is documented as a rare form of psoriatic arthritis

[1–8]. The striking clinical features of the syndrome include

psoriatic onychodystrophy, connective tissue thickening,

and periostitis of the distal phalanges, producing a drum-

stick-like deformity. Though any of the fingers are reported

to be affected, the great toe is the well known. The treatment

of the condition differs and has generally been reported to

be unsatisfactory with the traditional regimes. Here, we

describe a case whom we believe is one presentation of POPP

and thereby, discuss the knowledge on the disease entity.

Case report

A 20-year-old military recruit presented with painful

swelling, morning stiffness lasting about 2 h, and discol-

oration of his toes and fingers, a history going back seven

years. While there were concomitant nail changes and

sclerodactyly (Fig. 1), he was not having the evidence of

Raynaud’s phenomenon, photosensitivity, or malar rash.

There was no sign of other system findings.

In laboratory investigation, there was no acute phase

response with erythrocyte sedimentation rate of 5 mm/hour

and C-reactive protein of 2.3 mg/dl. Other immunological

parameters including rheumatoid factor, Anti-CCP, ANA,

Anti-SSA, Anti-SSB, Anti-centromer, and Anti-Scl70 were

all negative. Nail fold capillaroscopy revealed non-specific

changes, and there was no hemorrhage, dropout, distortion

or dilatation.

Result of the biopsy obtained from the nail fold of

forefinger was reported to be detected hyperkeratosis, and

minimal perivascular and interstitial inflammation.

Conventional radiographs of both hands were without

any remarkable joint changes or a distinct periosteal

reaction (Fig. 2). Three-phase bone scintigraphy showed

increased uptake in the metacarpophalangeal joints of 2nd,

3rd, and 5th rays on the left side and 1st, 2nd, and 4th on

the right side and both side carpal bones (Fig. 3). Magnetic

resonance (MR) images demonstrated increased signal

intensity representing bone marrow edema within relevant

metacarpal bones on fluid-sensitive sequences (Fig. 4a, b).

Computerized tomography (CT) of both hands was also

unremarkable in terms of the above parameters scrutinized

in his conventional radiographs (Fig. 5). No evident artic-

ular process was seen.

Based on these findings, the patient was diagnosed as

having a form of psoriatic arthritis. Initial treatment with

H. T. Sanal (&) � C. Tayfun

Department of Radiology, Gulhane Military Medical Academy,

Gn.Tevfik Saglam Cad., 06018 Etlik-Ankara, Turkey

e-mail: tubasanal@gmail.com

S. Yilmaz � M. Cinar � I. Simsek � A. Dinc

Department of Rheumatology, Gulhane Military Medical

Academy, Etlik-Ankara, Turkey

123

Rheumatol Int (2012) 32:1449–1452

DOI 10.1007/s00296-011-1914-y

methotrexate (10 mg/week) did not result in sufficient

relief of symptoms, therefore leflunomide (10 mg) has

been added to the therapy. Treatment was well tolerated,

and no side effects were observed.

After 1 year, a remarkable normalization of the nails on

physical examination and resolution of the initial inflam-

matory signal intensities of the metacarpal bones were

observed on MR images but phalanges (Fig. 6). His

morning stiffness was regressed from 2 h to 15–20 min.

Discussion

Psoriatic onycho-pachydermo periostitis (POPP) has been

recently recognized as a separate entity in the late eighties,

and since then less than 20 cases, mostly from Europe,

have been reported. It has been described by several

authors as a combination of findings as psoriatic onycho-

dystrophy, connective tissue thickening above the distal

phalanges accompanied with a periosteal reaction [1–8].

In our case, the primary syntigraphic and MRI finding

was an extensive bone marrow edema without a definite

periosteal reaction observed neither on direct radiographies

nor on CTs. Involvement of the metacarpal bones with

extensive marrow edema without any definite entheseal

continuum has driven us first to think about other diagnoses

such as reflex sympathic dystrophy or some mild form of

frostbite. With the history without a definite trauma or the

geography he used to be, we omitted those differentials.

We thought that this case may be one form of POPP

though this entity was first described as psoriatic onyc-

odystrophy, connective tissue thickening over the distal

phalanges and a periosteal reaction. Connective tissue

thickening may also be one chain in the concept of

‘‘synovio-entheseal complex’’ described by McGonagle

et al. [9]. According to this proposal, immune responses

may initiate in any of these structures either the synovium,

enthesis, periosteal linings or perhaps now connective tis-

sue—as in this case—and may be pivotal players in the

phenotypic expression of psoriasis.

The extensive bone marrow edema in the metacarpal

bones without an observable periostitis may be a blast

physiologic response to the stresses though it is not clear

why these cases can be vulnerable to such stresses [10]. It

is another question as to whether this edema can be a

migratory edema-like pattern getting disappeared in the

metacarpals and now seen at the distal aspect of the rays.

Fig. 2 The AP radiograph of both hands. No remarkable periosteal

reaction or joint changes

Fig. 3 Three-phase bone scintigraphy of both hands showing

increased uptake in the metacarpophalangeal joints of 2nd, 3rd, and

5th digits on the left side and 1st, 2nd, and 4th on the right side and

both side carpal bones at late phase

Fig. 1 Slight swelling around the interphalangeal joints of both

hands of the patient. The nails exhibit some onycholysis

1450 Rheumatol Int (2012) 32:1449–1452

123

The edema in the marrow of the metacarpals has been

dissolved completely after methotrexate treatment. This

implies that marrow edema alone without observable peri-

osteal reaction may have a better prognosis implying the

efficacy of the treatment. Besides no erosional change has

been observed in these bones after the edema as opposed to

the proposals of some authors who believe it is a predictor

or leading factor in the development of erosions [9, 11–13].

In conclusion, we think that as with psoriasis, POPP may

also have different clinical expressions. Though osteoperios-

titis component of the disease was first described predomi-

nantly in distal phalanges owing to its close proximity to nail

appendage, extensive bone marrow edema alone in any of the

hand bones should raise the possibility of this entity. In these

clinical displays, traditional treatment regimes may not work

and an aggressive option may be needed.

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Fig. 6 After treatment, resolution of the initial inflammatory signal

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Fig. 4 MR images of the left

hand. Axial fat saturated

T2-weighted

image (a) demonstrating

increased signal intensity

representing bone marrow

edema of the 2nd, 3rd, and 5th

metacarpal bones. T1-weighted

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(b) with hypointensity of the

marrows of the same bones

Fig. 5 a, b Computerized

tomography of both hands with

no remarkable periostitis or

changes of the interphalangeal

joints

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