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Journal of Rheumatic Diseases Vol. 22, No. 1, February, 2015 http://dx.doi.org/10.4078/jrd.2015.22.1.45 Case Report

Psoriatic Onycho-pachydermo-periostitis of the Fingertips: A Report of Two Cases

Myung Il Park1, Bo Yeon Kim1, In Park1, Ki Tae Kwon1, Dong Joo Shin2, Gun Woo Kim1, Sung Ho Kim1, Seung Woo Han1 Departments of 1Internal Medicine and 2Orthopedic Surgery, Daegu Fatima Hospital, Daegu, Korea

Psoriatic onycho-pachydermo-periostitis (POPP) causes severe nail dystrophy, painful soft tissue swelling, and marked peri- osteal reaction of the involved distal phalanx. There are few reports of POPP involving the great toe. We report on 2 cases of POPP involving the fingertips. A 60-year-old woman presented with fusiform swelling of her right 4th fingertip with severe ten- derness, and her fingernails and toenails had varying degrees of . She had mixed multiple erosions and meta-epi- physeal periostitis at the distal phalanx of the right 4th finger but was treated successfully with and cyclosporine. A 39-year-old woman presented with painful swelling of the left 2nd and 5th fingertip, psoriatic lesions on the knees and soles of the feet, and onycholysis without reactive periostitis of the left 2nd and 5th fingers. She was treated successfully with cyclosporine. Despite its rarity, POPP should be considered when diagnosing arthritic or infectious conditions affecting the dis- tal interphalangeal . (J Rheum Dis 2015;22:45-50)

Key Words. , , Periostitis, Onycholysis

INTRODUCTION CASE REPORT

First described in 1989, psoriatic onycho-pachydermo- Case 1 periostitis (POPP) is a rare variant of A 60-year-old Korean woman with a 4-year history of [1]. The characteristic features of POPP include severe painful swelling of the right 4th fingertip was referred for nail dystrophy, painful soft tissue swelling, and marked evaluation in October 2008. Approximately 10 years pre- periosteal reaction of the involved distal phalanx. To date, viously, onycholysis was noted on her left 1st, 4th, 5th, there have been only 19 case reports worldwide [1-12] and right 4th fingernails, which gradually spread to the and no reports in Korea. Although POPP may involve entire fingernails and toenails. Since March 2004, she both the fingers and toes, the great toes are involved in reported inflamed and painful swelling of her right 4th most reported cases (Table 1) [1-12]. Clinically, POPP can fingertip, and it persisted intermittently. Nine months be misdiagnosed as a fingertip or toe infection compli- later, a pustular lesion with an erythematous base cated by osteomyelitis, because it may precede skin mani- developed on her left palm. In April 2008, the pain in her festations of psoriasis and may affect a single finger or toe. right 4th fingertip was aggravated, and she visited the We present 2 cases of POPP involving the fingertips that clinic. Under the diagnosis of a finger- were successfully treated with either a combination of tip infection complicated by osteomyelitis of the distal methotrexate (MTX) and cyclosporine or cyclosporine phalanx, she underwent an incision and drainage oper- monotherapy. ation of the right 4th fingertip and was treated with anti- biotics despite negative culture results. Postoperatively,

Received：January 25, 2014, Revised：(1st) March 25, 2014, (2nd) April 11, 2014, Accepted：April 20, 2014 Corresponding to：Seung Woo Han, Department of Internal Medicine, Daegu Fatima Hospital, 99 Ayang-ro, Dong-gu, Daegu 701-724, Korea. E-mail：[email protected] pISSN: 2093-940X, eISSN: 2233-4718 Copyright ⓒ 2015 by The Korean College of . All rights reserved. This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

45 Myung Il Park et al.

Table 1. Summary of clinical characteristics of the 21 psoriatic onycho-pachydermo-periostitis patients Disease Distal phalanx Study Age (yr) Sex Skin psoriasis Onycholysis HLA-B27 Treatment duration swelling Fournié et al. [1] 57 NA 2 yr None Both 1st toes None Positive NA (1989)* 45 NA 2 yr None Both 1st toes, left None NA NA 3rd finger 46 NA 20 yr None Both 1st toes None NA NA 39 NA 3 yr None Right 1st toe None NA NA Marguery et al. [2] 55 M 2∼3 yr NA All fingers except Right 3rd, 4th and NA NA (1991)* left 5th finger left 2∼4th fingers De Pontville et al. 40 NA 1 yr NA All fingers and Right 1st finger, Positive NA [3] (1993)* toes right 1st, 3rd, 4th toe, left 1st, 4th toe 62 NA 1 yr NA Right 1st toe None Positive NA Grosshans and 35 NA Several NA Left 1st toe None NA NA Bosser [4] years (1993)* 37 NA 2 yr NA Left 3rd finger, Left 3rd finger Positive NA both 1st toes 49 NA 5 yr NA Right 1st finger None NA NA Boisseau-Garsaud 37 M 2 yr None Left 1st∼5th Left 2nd and 3rd Positive MTX 20 mg/wk et al. [5] (1996) fingers, both 1st fingers, both 1st (response) toes toes 49 M 5 yr Both elbow, Right 1st finger Right 1st finger NA NA scalp Bauzá et al. [6] 50 F 2 yr None Left 3rd∼5th Both 3rd fingers NA Cyclosporin (2000) fingers, right 3rd A 200 mg→MTX finger 15 mg/wk Anders et al. [7] 49 M NA None All fingers and Right 3rd, 4th and NA SSZ→radiotherapy (2002) toes left 2nd and 3rd fingers, left 1st toe Fietta and 33 M 6 yr Both All fingers None Negative Topical Manganelli [8] elbows, (2003) leg Bongartz et al. [9] 42 M 1.5 yr Both feet All toes All toes Positive MTX 15 mg/wk→ (2005) Kapusta and 53 M 0.5 yr Right ear, All fingers and Left 1st, 2nd, and 4th Negative MTX→→ Dumont [10] both feet toes toes; both 2nd, 3rd, (2008) and 5th fingers Watanabe et al. 59 M 3 mo Whole body All fingers and All fingers and toes Negative Etretinate→MTX [11] (2012) toes 8 mg/wk Bethapudi et al. 55 F 1 yr Palm, foot All toes Right 1st, 4th toe, NA NA [12] (2014) left 1st toe This study 60 F 4 yr Both hands, All fingers and Left 4th finger Negative MTX, cyclosporin A (2014) foot toes 39 F 1 yr Both knees, Left 2nd and 5th Left 2nd and 4th Negative Cyclosporin A right foot fingers fingers F: female, HLA: human leukocyte antigen, M: male, MTX: methotrexate, NA: not available, SSZ: . *These first 4 case reports were published in French and the data presented in this table was adapted from the report by Boisseau-Garsaud et al. [5].

46 J Rheum Dis Vol. 22, No. 1, February, 2015 Psoriatic Onycho-pachydermo-periostitis of the Fingertips

Figure 1. The first patient’s skin lesion and three-phase scintigraph. (A∼C) The right 4th fingertip shows fusiform swelling with , and the nails of the fingers and toes demonstrate dystrophic onycholysis. (B, C) Pustular psoriatic lesions are not- ed in the left palm and right great toe. (D) Three-phase bone scin- tigraphy shows a focal radio- nuclide accumulation in the right 4th and left 3rd distal pha- lanx fingers on a delayed static scan. the swelling and inflamed lesion on her 4th fingertip Based on these clinical and radiologic features, we diag- slowly improved, and a follow-up radiograph showed par- nosed her with POPP and pustular psoriasis of the palms tial healing of the osteolytic lesion of the distal phalanx. and soles. The patient was treated with a combination of However, since September 2008, painful swelling of the MTX at 15 mg/wk and 20 mg/d and pre- right 4th fingertip relapsed, which was accompanied by a dnisolone 7.5 mg/d. After 2 months, her psoriatic skin le- milder pain in the left 3rd fingertip. sions slightly improved, but the painful swelling of her At the first outpatient clinic evaluation, her right 4th fin- 4th fingertip did not improve, so we substituted cyclo- gertip showed fusiform swelling with severe tenderness sporine 200 mg per day for the leflunomide. After another and only tenderness of the left 3rd fingertip. All of her fin- 2 months, the pain and swelling of her left 4th fingertip gernails and toenails had varying degrees of dystrophic and her psoriatic skin lesions dramatically improved. The change with deformity, and there were multiple pustular patient has been followed for more than 5 years and re- psoriatic skin lesions on her right 4th finger, the palm of mains well with MTX monotherapy. left hand, and the soles of both feet (Figure 1A∼1C). She did not complain of any chest pain or low . The Case 2 laboratory results included the erythrocyte sedimentation A 39-year-old Korean female was consulted by the rate and the C-reactive protein (CRP) level and were Dermatology department. She had a 12-month history of within normal limits. The anti-nuclear antibody, rheuma- painful swelling of the left 2nd and 5th fingertips, and toid factor, and the human leukocyte antigen (HLA)-B27 these nails showed subungual and ony- were negative. Three-phase bone scintigraphy showed a cholysis (Figure 3A). She also had psoriatic lesions on focal radionuclide accumulation in the right 4th and left both knee and the soles of both feet (Figure 3B and 3C). 3rd distal phalanx of the fingers on a delayed static scan She did not complain of or inflammatory back (Figure 1D). A radiograph of the hand showed mixed pain. She had no medical history suggesting an intestinal multiple erosions and meta-epiphyseal periostitis at the or urogenital infection. Laboratory tests revealed that her distal phalanx with associated soft tissue swelling of the leukocyte count was 7,390 cells/mm3, hemoglobin was right 4th finger. The articular surface and width of the 11.6 g/dL, were 263,000 cells/mm3, and in- joint space of the distal interphalangeal (DIP) joint was flammatory markers were within normal range; the eryth- preserved (Figure 2). rocyte sedimentation rate was 15 mm/h and CRP level

www.jrd.or.kr 47 Myung Il Park et al.

Figure 2. A serial change of the hand is shown on anteroposterior radiographs. (A) The radiograph taken 6 months prior to pre- sentation demonstrates meta-epi- physeal periostitis and multiple erosions of the distal phalanx of the left 4th finger. (B) After 4 months, a reactive bone for- mation is observed at the shaft of the distal phalanx (arrow). (C) The radiograph at presentation reveals progression of erosion and marked soft tissue swelling (arrow).

Figure 3. The second patient’s hand and skin lesions before treatment. (A) The left 2nd and 5th fingers exhibit drumstick-like swelling and , and the fingernails show severe onycholysis. (B, C) Erythematous, hyperkeratotic plaque is seen on both knee and the sole of the right foot. was 0.05 mg/dL. The , anti-nuclear DISCUSSION antibody, anti- cytoplasmic antibody, and HLA-B27 were all negative. Potassium hydroxide (KOH) POPP is a recently described entity in the expanding wet-mount microscopic examination of a nail specimen spectrum of psoriatic arthritis. It is characterized by with onycholysis showed no hyphae or yeast. Conventional psoriatic onychodystrophy and thicken- radiographs of her hand revealed no definite cortical ero- ing of the distal phalanx, including a periosteal reaction. sion or periosteal reaction and no evidence of active Moll and Wright [13] first categorized psoriatic arthritis arthritis. Clinically, she was diagnosed with psoriatic ony- into five subtypes: predominantly DIP involvement; ar- cho-pachydermo-acropathy without periostitis. She was thritis mutilans; symmetric resembling given cyclosporine 200 mg daily, and her finger lesions ; asymmetric ; and sac- improved after 3 months. However, some onycholysis of roiliitis or spondylopathy. According to this classification, her fingernails persisted. POPP does not fit into any category of psoriatic arthritis. Recently, Koó et al. [14] proposed a new classification for

48 J Rheum Dis Vol. 22, No. 1, February, 2015 Psoriatic Onycho-pachydermo-periostitis of the Fingertips psoriatic arthritis through a hierarchical cluster analysis ly 3 reported cases and its traditional role as a first-line of 100 patients, which is characterized by considering not agent for psoriatic arthritis is being challenged by bio- only the arthritic status, but also the type of the skin logics such as anti- (TNF) inhibitors disease. It created new subgroups, including the distal [5,6,8-10]. However, the significant cost differential for group characterized by sausage fingers and the pustular biological agents makes it hard to prescribe an anti-TNF group characterized by psoriatic pustular skin lesions, inhibitor as the first-line choice for POPP. Considering and both groups share the features of nail dystrophy [14]. that our patients showed a good response with the combi- POPP can belong to these distal and pustular groups of nation of MTX and cyclosporine or cyclosporine mono- psoriatic arthritis. therapy, a combination of classic DMARDs may be used The pathophysiology of POPP has been not well together when the efficacy of MTX monotherapy is in- elucidated. The association with HLA-B27 based on the sufficient, prior to anti-TNF inhibitor. data that HLA-B27 was positive in 7 of 19 cases reported so far suggests similarities with other spondyloar- SUMMARY thropathies (Table 1) [1-12]. Actually, the bone forming feature of the periosteal reaction in POPP can be seen in We describe two cases of POPP that were successfully other seronegative [13]. A pro- treated with either a combination of MTX and cyclo- posed mechanism is analogous to that of the ossifying sporine or cyclosporine monotherapy. POPP is a rare form enthesopathy of seronegative spondyloarthropathy, in of psoriatic arthritis, but it needs to be considered in the which inflammation originates at the entheseal sites and differential diagnosis within the context of arthritic or in- is secondarily transmitted to other articular structures. fectious conditions affecting DIP joint. As such, POPP can be explained by the anatomic relation- ship between the nail and the terminal phalanx, whereby CONFLICT OF INTEREST inflammation can spread from the subungual dermis to the terminal phalanx through the fibrous septa, which di- No potential conflict of interest relevant to this article rectly joins them and is deeply inserted into the bone [5]. was reported. Clinically, POPP should be differentiated from other types of arthritic or infectious conditions affecting the REFERENCES DIP joint. The most important differential diagnoses are for ungual mycosis superimposed fingertip infections 1. Fournié B, Viraben R, Durroux R, Lassoued S, Gay R, that can show a very similar clinical manifestation and Fournié A. Psoriatic onycho-pachydermo-periostitis of the should be excluded based on negative nail mycologic big toe. Anatomo-clinical study and physiopathogenic ap- proach apropos of 4 cases. Rev Rhum Mal Osteoartic specimens. In , involvement of the nails 1989;56:579-82. is very similar to that in POPP, but this syndrome pre- 2. Marguery MC, Baran R, Pages M, Bazex J. Psoriatic cedes enteric or genitourinary infection and is usually acropachydermy. Ann Dermatol Venereol 1991;118:373-6. characterized by an abrupt appearing, asymmetric in- 3. De Pontville M, Dompmartin A, De Raucourt S, Macro M, Rémond B, Leroy D. Psoriatic onycho-pachydermo-periostitis. flammation of the synovial tissue [15]. In addition, the Ann Dermatol Venereol 1993;120:229-32. absence of articular involvement of the DIP allows 4. Grosshans E, Bosser V. A case for diagnosis: ungueal exclusion of the acral/digital type of psoriatic arthritis as psoriasis. Ann Dermatol Venereol 1993;120:319-20. 5. Boisseau-Garsaud AM, Beylot-Barry M, Doutre MS, Beylot a cause of the onycholysis lesion. , pustu- C, Baran R. Psoriatic onycho-pachydermo-periostitis. A var- losis, hyperostosis, and osteitis syndrome also can be tak- iant of psoriatic distal interphalangeal arthritis? Arch en into account because of the plantar pustular psoriatic Dermatol 1996;132:176-80. lesions, but synovitis, , and hyperostosis are 6. Bauzá A, Redondo P, Aquerreta D. Psoriatic onycho-pachy- dermo periostitis: treatment with methotrexate. Br J not seen in POPP. Dermatol 2000;143:901-2. Treatment for POPP is generally based on the regimen 7. Anders HJ, Sanden S, Krüger K. Psoriatic onycho-pachy- for psoriatic arthritis. Commonly used disease modifying dermo- periostitis. Z Rheumatol 2002;61:601-4. 8. Fietta P, Manganelli P. and psoriatic anti-rheumatic drugs (DMARDs) are MTX, leflunomide, onychopathy: an unusual association. J Eur Acad Dermatol and cyclosporine. However, according to the few pub- Venereol 2003;17:73-6. lished case reports, MTX provided a limited benefit in on- 9. Bongartz T, Härle P, Friedrich S, Karrer S, Vogt T, Seitz A,

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