Annals of the Rheumatic Diseases 1996; 55: 177-180 177

Osteoarticular manifestations of pustulosispalmaris et plantaris and of psoriasis: two distinctentities

O Mejjad, A Daragon, J P Louvel, L F Da Silva, E Thomine, P Lauret, X Le Loet

AbstractObjective-To test the hypothesis thatpustulosis palmaris et plantaris andpsoriatic arthritis (PsA) are two distinctdiseases, and that the associated derma-toses are therefore also distinct diseases.Methods-We prospectively performedclinical, radiological, biological, and bonescan investigations in 23 outpatients withpustolotic arthritis and 23 outpatients withPsA, matched by gender, age (± one year)and duration of arthritis (+ two years).Results-The anterior chest wall, especi-ally the sternocostoclavicular joints, wasmore frequently involved in pustuloticarthritis than in PsA, both clinically (82%v 43%; p < 0.001) and radiologically (47%v 17%; p < 0-05). Sternocostoclavicularjoints generally presented with erosivelesions in PsA, and with large ossificationsin pustulotic arthritis. Peripheral jointinvolvement was mono- or oligoarticular,affecting proximal joints, in pustuloticarthritis (74% v 21%; p < 0.01), and poly-articular, involving small distal joints, inPsA (60% v 0%; p < 10 ), in whichcondition it was also more often erosive(43% V 8%; p < 0-01). The frequency ofsacroiliitis and of spine involvement wassimilar in pustulotic arthritis and PsA.Biology and bone scan did not help dis-tinguish between the two groups.Conclusions-Pustulotic arthritis andPsA are clinically and radiologically dif-ferent, therefore pustulosis palmaris etplantaris and psoriasis are most probablydistinct dermatological diseases.

(Ann Rheum Dis 1996; 55: 177-180)

Pustulosis palmaris et plantaris (PPP) is achronic pustular dermatosis characterised bysterile pustules on the palms and the soles.'Psoriasis is a well known skin disease with welldefined clinical and pathological signs. Therelationship between psoriasis and PPP is notclearly defined in the literature: some authorsconsider PPP as a variant of psoriasis,2- whileothers believe it to be a separate entity.' 7 8However, there remain grounds for dis-agreement with this latter hypothesis.24We postulated that psoriasis and PPP were

two different entities.' 7 8 Because clinical andpathological cutaneous arguments were notsufficiently convincing for some authors, weundertook a prospective study to compare the

osteoarticular manifestations of psoriasis andPPP. If pustulotic arthritis and psoriaticarthritis proved to be clearly different, we couldaccordingly also consider their associateddermatoses as different.

Patients and methodsBetween 1987 and 1992, in the French ad-ministrative region of Haute-Normandie, 23successive outpatients (15 women, eight men;mean age 45-3 (SD 14-2) years) were identifiedas having histologically proven PPP accordingto Pierard and Kint's criteria.' The clinicalcriteria for selection were: pustules orvesicopustules on the midpalms or thenareminences of the hands, or both (rare pustulesdistant from these sites were accepted); nailsand acral portions of the fingers and toesspared; absence of psoriasis on any other partof the skin; absence of a family history ofpsoriasis (in practice, no patient withhistologically proven PPP had a relative withpsoriasis, therefore we did not have to excludeany patient). Histological criteria comprised afully developed pustule that was large, intra-epidermal in location, unilocular; variousstages of pustule formation could be observed(spongiosis appearing in the epidermis abovethe top of a dermal papilla, giving a vesiclefilled with fluid and mononuclear cells;malpighian roof disrupted, leading to a massiveinvasion of the cavity by polymorphonuclearleucocytes that penetrate into the intracellularspaces of the vesicle wall, where spongiformpustules were seen). All the subjects had or hadhad osteoarticular manifestations characterisedby peripheral arthralgia or arthritis, spine orsacroiliac joint pain, and anterior chest wallpain or swelling. Five patients with pustules onthe palms and soles associated with psoriasis onother parts of their skin were not includedbecause histological examination of a pustulespecimen revealed psoriasis and not PPP; thusno patient included in the PPP group wassuffering from psoriasis.Twenty three PsA outpatients in an active

phase of their rheumatic disease matched bygender, age (± one year), age at onset ofarthritis, and duration of arthritis at firstexamination (± two years), were also recruitedto the study (15 women, eight men; mean age45-6 (SD 13-1) years). The clinical criteria forselection were: scalp, sacral region, extensorsurfaces of the limbs, nails, intertriginous areasmainly affected; entire skin may be affected;lesions sharply demarcated and covered with

Department ofRheumatology,Hopital deBois-Guillaume,CHU de Rouen,76031 Rouen Cedex,FranceO MejjadA DaragonL F Da SilvaDepartment ofRadiologyJ P LouvelDepartment ofAnatomo-pathologyE ThomineDepartment ofDermatologyP LauretGroupe de Rechercheen Immuno-PathologieX le LoetCorrespondence to:0 Mejjad,Service de Rhumatologie,H6pital de Bois-Guillaume,CHU de Rouen,76031 Rouen Cedex,France.

Accepted for publication30 October 1995

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Table 1 Demographic characteristics ofpatients withpustulosis palmaris et plantaris (PPP) or psoriasis

PPP Psoriasis

Age (years) 45-3 (14-2) 45-6 (13-1)Age at onset of arthritis (years) 36-9 (14-4) 38-0 (13 8)Duration of arthritis at first

examination (years) 7-1 (1-7) 8-0 (1-1)

Values are mean (SD).No significant differences between groups.

layers of fine scales, removal ofwhich by gentlescraping usually revealing fine bleeding points.Histological criteria comprised: elongation ofthe rete ridges with thickening in their lowerportion; elongation and oedema ofthe papillae;thinning of the suprapapillary portions of thestratum malpighii, with the occasionalpresence of a very small spongiform pustule;parakeratosis and the presence of Munromicroabscesses. Psoriasis was recognisedclinically by an experienced dermatologist(P L), and histologically in case of doubt.9The 46 patients underwent the following

investigations:(1) Physical examination by the same rheu-

matologist. All osteoarticular manifestations,past or present, with spontaneous pain or painon pressure, were considered. Spine involve-ment was clinically assessed by pain (past or

present) and by the Shober's test.(2) Technetium-99m pyrophosphate bone

scan, radiography of hands, feet, pelvis,dorsolumbar spine, and manubriosternal joint,and computed tomography of sternoclavicularjoints. Additional radiographs were taken incases of bone scan abnormality and osteo-articular manifestations in other sites. Radio-graphs and computed tomography data of thepatients were read blindly and separately bytwo rheumatologists and two radiologists welltrained in osteoarticular radiology.'0 In the

Table 2 Dermatologicalfindings

Patient PPP Psoriasis

Clinical distribution Histological Clinical distribution of Histologicalofpustules examination* psoriasis examination*

1 Palms and soles PPP Face, scalp, trunk, elbows,knees, sacral region

2 Palms and soles PPP 75% of the entire skin Psoriasis3 Palms and soles PPP Elbows, scalp, legs, trunk4 Palms and soles PPP Knees elbows, scalp, legs5 Palms and soles PPP Elbows, knees, nails6 Soles PPP Knees, scalp, nails7 Palms and soles PPP Elbows, knees, thighs8 Palms and soles PPP Thighs Psoriasis9 Palms and soles PPP Elbows, scalp, palms and

soles, nails10 Palms and soles PPP Elbows, knees, sacral region1 1 Soles PPP Arms, forearms, scalp, legs12 Palms and soles PPP Scalp, legs13 Palms and soles PPP Elbows, scalp, palms and Psoriasis

soles14 Palms and soles PPP Elbows, knees, scalp, sacral

region, thighs15 Palms PPP Elbows, knees, scalp16 Palms PPP Elbows, knees, sacral region17 Palms PPP Scalp, sacral region18 Palms and soles PPP Scalp, knees Psoriasis19 Palms and soles PPP Scalp, toes, nails, elbows,

knees20 Palms PPP Scalp, elbows, knees, sacral

region, trunk21 Soles PPP Elbows, knees, sacral region,

scalp22 Palms and soles PPP Legs, scalp Psoriasis23 Palms and soles PPP Elbows, forearms, fingers,

nails, scalp

*All PPP = histologically proven pustulosis palmaris et plantaris; all psoriasis = histologicallyproven psornasis.

event of disagreement between readers, aconsensus was obtained by 'collegial' reading,according to Sonosaki's classification forsternoclavicular joints whenever possible." 12Radiological interpretation was descriptiveonly when Sonosaki's classification was notadequate. Dilsen's classification was used formanubriostemal joints'3 and that of Dale forsacroiliac joints.'4

(3) Laboratory investigations: full bloodcount, erythrocyte sedimentation rate (ESR),serum alkaline phosphatases, latex agglutina-tion test, serum IgA by laser nephelometry,HLA-A, -B, -C and -DR typing, and bacterio-logical examination of pustules, when pos-sible.

Statistical analysis was by the x2 test tocompare the two groups.

ResultsTable 1 shows the demographic characteristicsof PPP and psoriasis patients; table 2 lists thedermatological findings.

Skeletal and skin symptoms appearedsimultaneously (±6 months) in nine of the 23PPP patients (39%), and in four of the 23psoriasis patients (17%) (not statisticallysignificant). Skin and skeletal flares occurred ineight PPP patients (35%) and two patientswith psoriasis (8X7%) (p < 005).Two PPP patients had relatives with an

unclassified rheumatism without skin disease;no familial case of PPP, psoriasis, or pustuloticarthritis was found. Nine ofthe 23 PsA patients(39%) had relatives with psoriasis and twopatients had relatives with rheumatism (un-classified rheumatism in the first case, andpsoriatic arthritis in the second).

Table 3 shows the clinical findings. Thetwo groups were not different for spineand sacroiliac joint manifestations, but theydiffered in anterior chest wall involvement(p < 0 00 1), especially that of the sterno-clavicular joint, which was more frequentlyimplicated in the PPP group (p < 005). Thefrequency of peripheral joint manifestationsdid not differ between the groups, but thenature of this involvement was different:mono- or oligoarticular (17/23), but neverpolyarticular, in the case of PPP, but predomi-nantly polyarthritis (14/23) in the case ofpsoriasis. The main sites were the shoulders(64%) for PPP and small distal joints (58%) forpsoriasis. Enthesitis at a distance from jointswas noted in four PPP patients, involvingeither heel, iliac crest, ischium, or trochanter,and in 11 psoriatic patients (p < 005).Table 3 also shows the radiological findings.

The anterior chest wall was more frequentlyinvolved during pustulotic arthritis (p < 005).In spite of more frequent lesions of thesternoclavicular joints in the PPP group, thedifference was not statistically significant.However, the three psoriatic patients withsternoclavicular arthritis presented with erosivelesions (fig 1), whereas the eight PPP caseshad associated erosive lesions and ossifications(fig 2). Spine and sacroiliac manifestationswere not different between the two groups:

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Pustulotic arthritis and psoriatic arthritis

Table 3 Clinical and radiologicalfindings and comparison between patients withpustulosis palmaris et plantaris (PPP) orpsoriasis

Clinicalfindings Radiologicalfindings

PPP Psoriasis p PPP Psoriasis p(n = 23) (n = 23) (n = 23) (n = 23)

Anterior chest wall 19 (82 6) 10 (43 5) <0 001 11 (47 8) 4 (17-4) <0 05Sternoclavicular 14 (60 8) 7 (30 4) <0 05 8 (34 8) 3 (13-0) NSManubriostemal 5 (21-7) 2 (8 7) NS 3 (13-0) 1 (4-3) NSSternocostal 7 (30 4) 4 (17-4) NS

Spine 19 (82 6) 15 (65 2) NS 7 (30 4) 8 (34-8) NSSacroiliac joints 8 (34 8) 7 (30-4) NS 8 (34 8) 4 (17-4) NSPeripheral joints 17 (73 9) 19 (82-6) NS 2 (8 7) 10 (43-5) <0 01

Arthralgia 9 4 <0 02Arthritis 8 15 <0 001Mono/oligoarticular 17 5Polyarticular 0 14 <10-

Values are number (%).NS = not significant.

sacroiliitis, syndesmophytes, and multipleosteophytes were found with the samefrequency. Erosive arthritis of the peripheraljoints was more frequent in the psoriasis group(p < 0.01). We noted only two cases of erosivearthritis in the PPP group, involving the hip inthe first case, and radioulnar and second and

Figure 1 Computed tomography scan ofsternocostoclavicular joints in a patient withpsoriatic arthritis, demonstrating only erosive lesions, without ossification.

Figure 2 Computed tomography scan ofsternocostoclavicular joints in a patient withpustulotic arthritis, demonstrating large ossifications.

Table 4 Bone scanfindings and comparison betweenpatients with pustulosis palmaris et plantaris (PPP) orpsorasis

PPP Psoriasis p(n = 23) (n = 23)

Anterior chest wall 12 (52-2) 7 (30 4) NSStemoclavicular joints 11 (47 8) 4 (17-4) <0 05Manubriosternal joint 1 (4-3) 3 (13-0) NS

Spine 4 (17-4) 7 (30 4) NSSacroiliac joints 7 (30 4) 7 (30 4) NSPeripheral joints 7 (30 4) 12 (52 2) NS

Values are number (%).NS = not significant.

third metacarpophalangeal (MCP) joints in thesecond case.

Chronic recurrent multifocal osteitis wasobserved in three patients with PPP: oneinvolved the femur, another the clavicle, andone patient had tibial, femoral and pelviclocalisations. In the psoriasis group, weobserved only one patient with vertebral andclavicular localisations.Table 4 shows the bone scan findings. No

statistical difference was observed between thetwo groups, except that sternoclavicular jointinvolvement was more frequent in pustuloticarthritis (p < 0 05).There was no statistical difference between

the groups in the frequency of increased ESR(>20 mm/lst h), increased leucocyte count(>109/l), or serum alkaline phosphataseconcentrations. Latex agglutination test wasnegative in all cases. Serum IgA titre was morefrequently increased in the psoriasis group(78-9% v 40%; p < 0001). HLA-A, -B, -C,-DR typing was unremarkable except for theHLA-B27 antigen, which was found in 9-5%of PPP patients-a rate not different fromthat observed in a control population inNormandy"5-and in 27-3% of patients withpsoriasis.

Spondylarthropathy testing revealed nine(39%) PPP patients and 23 (100%) psoriasispatients to be positive according to the criteriaof the European Spondylarthropathy StudyGroup (ESSG)."6

DiscussionMany authors consider psoriasis and PPP to bethe same disease,21 while others view them asdifferent dermatological disorders;' 7 8 debateon the clinical and anatomopathologicalfeatures ofPPP and its relationship to psoriasiscontinues. 7 We postulated that PPP andpsoriasis were separate entities, 7 8 and weundertook the present prospective study tocompare the frequency and the type ofosteoarticular manifestations of these twodermatological diseases. If the findings forpustulotic arthritis and psoriatic arthritis wereclearly different, we could consider them asseparate entities.We investigated two different groups of

patients with well-defined dermatological cri-teria at inclusion (table 2) and demonstratedthat, statistically, the two types of rheumatismwere different in several manifestations, andoften in the kind of joint involvement. Thus we

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conclude that PPP and psoriasis are probablytwo distinct dermatological diseases.The anterior chest wall and, especially, the

stemoclavicular joints were more frequentlyimplicated during PPP. The frequency ofclinical anterior chest wall involvement in PPPas reported in the literature varies from 60 to100%;'1121819 our findings confirmed thishigh incidence.While the frequency of peripheral joint signs

and symptoms was about the same in the twogroups, the nature of this involvement wasdifferent: mono- or oligoarticular for PPP, butpolyarticular for psoriasis. This difference wasconfirmed by radiological findings. In spite ofa non-significant difference for stemoclavicularinvolvement (probably attributable to the smallnumber of the patients included in this study),the type of lesions in general differed betweenthe conditions: erosive arthritis was found inpsoriasis (fig 1), while there was an associationof erosive lesions and ossifications in PPP(fig 2); this was described previously by Sono-saki et al." 12 Erosive arthritis of the peripheraljoints is rare in PPP; we found two such cases,involving the hip in the first, and the wrist andMCP joints in the second. Only 11 other caseshave previously been reported in theliterature.2023 Erosive arthritis of the peripheraljoints is a more common feature in psoriaticarthritis,24 as was confirmed in our study.

Biological findings did not differ between thetwo groups and were thus not helpful indistinguishing between the two entities. Therewas no link with the HIA-B27 antigen in thePPP group, which was in agreement with otherresults.'2 18 The frequency of B27 antigen inthe psoriasis group (27-3%) was similar to thatreported from other studies.25 26We tested all patients for the ESSG criteria,

and 39% of the PPP patients were positive. Itseems valid to consider anterior chest wallinvolvement as an enthesitis, because of thelarge number of ligaments and tendons in thesternocostoclavicular and manubriosternalregions. When we also considered PPP as acriterion, in common with psoriasis, 20 PPPpatients (87%) became positive according tothe ESSG criteria. In agreement with Sonozakiet al, we believe that pustulotic arthritis couldbe included in the spondylarthropathygroup. 12

In conclusion, even if the osteoarticularmanifestations associated with PPP and withpsoriasis are considered to belong to thespondylarthropathy group of diseases, theyhave been shown to be different, as we initiallypostulated. In light of these data, we believethat PPP and psoriasis are separate dermato-logical entities. However, an epidemiologicalstudy is required, to determine how many

patients have 'pure' PPP, how many have'pure' psoriatic arthropathy, and how manyhave an 'overlap'.The authors thank Mrs Foster for reviewing this paper.

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14 Dale K, Vinje 0. Radiography of the spine and sacro-iliacjoints in ankylosing spondylitis and psoriasis. Acta RadiolDiagnosis 1985; 26: 145-59.

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20 Huaux J P, Malghem J, Rombouts J J, et al. Osteomyeliterecidivante multifocale chronique del'enfant et pustulosepalmo-plantaire. A propos d'un nouveau cas. Rev Rhum1987; 54: 403-5.

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