Histopathology and correlates of systemic disease inadult Henoch-Sch€onlein purpura: A retrospectivestudy of microscopic and clinical findings in 68

patients at Mayo Clinic

Timothy J. Poterucha, BS,a David A. Wetter, MD,b Lawrence E. Gibson, MD,b,c Michael J. Camilleri, MD,b,c

and Christine M. Lohse, MSd

Rochester, Minnesota

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Background: The histopathology of Henoch-Sch€onlein purpura (HSP) is well defined, but specificmarkers have not been correlated with systemic involvement.

Objective: We sought to evaluate whether histopathologic markers were associated with renal or othersystemic involvement in adult HSP.

Methods: We retrospectively reviewed clinical information and pathology slides of 68 adult patients withHSP seen at Mayo Clinic between 1992 and 2011.

Results: Of the 68 patients, mean age was 45.8 years and 41 (60%) of the patients were male. Renalinvolvement was observed in 30 patients (44%), gastrointestinal tract in 27 (40%), joint in 32 (47%), and anysystemic signs in 52 (76%). Patients who were older than 40 years and had leukocytoclastic vasculitis withan absence of eosinophils on skin biopsy specimen had higher rates of renal involvement than those whodid not have both of these features (75% vs 27%; P\ .001). Patients with skin biopsy specimens showingleukocytoclastic vasculitis and an absence of histiocytes had higher rates of gastrointestinal tractinvolvement (P = .03). Age of 40 years or younger was associated with increased risk for gastrointestinaltract involvement and a nonsignificant trend for joint involvement (P = .004 and P = .06, respectively).

Limitations: This study is retrospective, and the causative factors of HSP were unable to be determined inmany patients.

Conclusion: Patients older than 40 years with HSP who had an absence of eosinophils on skin biopsyspecimen had a nearly 3-times increased risk of renal involvement compared with patients who did nothave both features. ( J Am Acad Dermatol 2013;68:420-4.)

Key words: Henoch-Sch€onlein purpura; Histopathology; Kidney; Leukocytoclastic vasculitis; Lightmicroscopy; Petechiae; Purpura; Vasculitis.

Abbreviations used:

GI: gastrointestinalHSP: Henoch-Sch€onlein purpuraLCV: leukocytoclastic vasculitis

Henoch-Sch€onlein purpura (HSP) is anIgA-associated small-vessel leukocytoclas-tic vasculitis (LCV) that typically presents

with lower extremityepredominant nonthrombocy-topenic palpable purpura. In addition to cutaneousmanifestations, HSP can affect the kidneys,

the Mayo Medical School, College of Medicine,a Department

Dermatology,b Division of Dermatopathology,c and Division

Biomedical Statistics and Informatics,d Mayo Clinic.

ing sources: None.

licts of interest: None declared.

pted for publication August 9, 2012.

Reprint requests: David A. Wetter, MD, Department of

Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN

55905. E-mail: wetter.david@mayo.edu.

Published online September 10, 2012.

0190-9622/$36.00

� 2012 by the American Academy of Dermatology, Inc.

http://dx.doi.org/10.1016/j.jaad.2012.08.011

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VOLUME 68, NUMBER 3Poterucha et al 421

gastrointestinal (GI) tract, and joints. Much researchin HSP over the past 20 years has focused onidentifying prognostic factors of renal disease, suchas clinical factors and direct immunofluorescencefindings. Clinical factors associated with renal dis-ease in adults include older age, pyrexia, recentinfectious history, inflammatory markers, purpura

CAPSULE SUMMARY

d Henoch-Sch€onlein purpura is welldefined histopathologically but, to ourknowledge, no correlations have beenmade between histopathology andsystemic involvement.

d The presence of renal involvement wassignificantly associated with: (1) skinbiopsy specimens showingleukocytoclastic vasculitis withouteosinophils; and (2) age older than 40years. Patients with both these featureshad the highest risk of renalinvolvement.

d Further studies are needed to developclinical predictive tools for systemicinvolvement in adult Henoch-Sch€onleinpurpura.

above the waist, anemia andhematuria at presentation,and onset of disease duringsummer months.1-3 One re-cent study found that some ofthese variables, includingolder age, hematuria, and se-verity of leukocytoclasis,were associated with a re-lapsing course of HSP.4

Other studies have noteddecreased rates of renal in-volvement with a drug-induced cause.5,6

To our knowledge, apaucity of medical literatureexists regarding potentialassociations between histo-pathologic findings and sys-temic involvement in adultHSP. To explore potentialassociations, we conducteda retrospective review ofadult patients with HSP

treated at Mayo Clinic from 1992 to 2011.

METHODSPatients and inclusion criteria

With the inclusion criteria for adult HSP delin-eated by Takeuchi et al7 and Poterucha et al,8 thisstudy identified 68 adult patients (age [18 years)who had: (1) a diagnosis of HSP with palpablepurpura consistent with the disease, (2) LCV onhistopathologic evaluation, and (3) presence of IgAon direct immunofluorescence. These patients wereidentified and studied in the recent report byPoterucha et al.8 Per the inclusion criteria ofPoterucha et al,8 patients must have had HSP thatwas not believed to be caused by another clinicaldisorder and ‘‘patients were excluded if they hadviral hepatitis, connective tissue disease, hemato-logic disorders, coexisting malignancy, cryoglobu-linemia, systemic lupus erythematosus, or otherautoimmune disease.’’ To be included in our study,patients must have had skin biopsy slides available atour institution. Of the 87 patients in the original studyby Poterucha et al,8 68 patients had histopathologyslides stored and formed our final patient cohort.

Clinical data were managed using research elec-tronic capture tools hosted at Mayo Clinic.9

Clinical data abstracted from patient charts in-cluded lesion distribution; presence of ulcerations ofnecrosis on physical examination; presence or ab-sence of GI tract, joint, renal, pulmonary, and eyeinvolvement; direct immunofluorescence results;

and laboratory information.The criteria used to assesssystemic involvement werethe same as those used inthe recent study byPoterucha et al8: ‘‘Joint in-volvement was determinedthrough the presence of ar-thritis or arthralgias on clini-cal history and examination.Patients were considered tohave gastrointestinal tract in-volvement when medicalhistory showed abdominalpain or hematochezia tem-porally related to the devel-opment of HSP cutaneousmanifestations. Renal in-volvement was determinedthrough microscopic pro-teinuria or hematuria, orboth, or through IgA deposi-tion on renal biopsy speci-

men within 1 month of the occurrence of HSP skinlesions.’’

Review of all available patient biopsy specimenswas done by 2 dermatopathologists in the study(L. E. G. and M. J. C.). Each patient had between1 and 3 histopathology slides stored, each of whichcontained 2 to 8 sections of tissue. All availablesections for each patient were reviewed. All clinicaldata, including systemic and cutaneous features,were masked to the pathologists. Histopathologicinformation recorded included presence or absenceof the following: red blood cell extravasation,apoptosis of keratinocytes, papillary dermal edema,eccrine gland involvement, intraepidermal or sub-epidermal vesiculation, intraepidermal or subepi-dermal microabscess formation, papillary dermalinflammation, reticular dermal inflammation, sub-cutaneous fat involvement, endothelial cell swell-ing, neutrophil infiltration into vessel walls,perivascular eosinophils, perivascular lymphocytes,perivascular histiocytes, perivascular plasma cells,nuclear debris, intravascular thrombosis, fibrinoidnecrosis, arteriole involvement, and deep venuleinvolvement. These histopathologic markers weresimilar to those used by Linskey et al10 in their 2011

Table I. Systemic characteristics andhistopathologicresults in 68 adults with Henoch-Sch€onlein purpura

Characteristic Patients, No. (%)

Cutaneous ulceration 12 (18)Systemic involvementRenal 30 (44)GI tract 27 (40)Joint 32 (47)Eye 1 (1)Any of the above 52 (76)

Histopathologic resultsLeukocytoclastic vasculitis 68 (100)Intraepidermal or subepidermalmicroabscess formation

21 (31)

Papillary dermal inflammation only 59 (87)Eccrine gland involvement 23 (34)Subcutaneous fat involvement 5 (7)Apoptosis of keratinocytes 13 (19)Papillary dermal edema 28 (41)Intraepidermal or subepidermalvesiculation

13 (19)

Red blood cell extravasation 66 (97)Intravascular thrombosis 5 (7)Lymphocytes 67 (99)Eosinophils 35 (51)Histiocytes 14 (21)Plasma cells 1 (1)Deep arteriole involvement 1 (1)

GI, Gastrointestinal.

Table II. Association between absence of tissueeosinophils and older age ([40 years) with renalinvolvement

Renal involvement

Tissue eosinophils*

P valueAbsent (n = 33) Present (n = 35)

.008No 13 (39) 25 (71)Yes 20 (61) 10 (29)

Age, y

# 40 (n = 26) [40 (n = 42)

.03No 19 (73) 19 (45)Yes 7 (27) 23 (55)

*Percentages are calculated using number of patients with or

without tissue eosinophils or younger or older than 40 years as

denominator.

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MARCH 2013422 Poterucha et al

investigation of IgA vasculitis at MassachusettsGeneral Hospital.

Statistical analysisStatistics were conducted in a similar manner

to that used in the study by Poterucha et al.8

Continuous features were summarized with mean,SD, median, and range; categorical features weresummarized with frequency count and percentage.Associations between features were evaluatedwith 2-sample t test, x2 test, and Fisher exact test.Statistical analyses were performed using the SASsoftware package (Version 9.2, SAS InstituteInc, Cary, NC). All tests were 2-sided, andP values less than .05 were considered statisticallysignificant.

RESULTSIn all, 68 adult patients with HSP were identified

who had histopathologic slides available for micro-scopic review. Of these patients, 41 (60%) were male(mean [SD] age, 45.8 [17.7] years; median [range] age,46.5 [19-78] years). Renal involvement was identifiedin 30 (44%); GI tract, 27 (40%); joint, 32 (47%); anysystemic sign, 52 (76%); and cutaneous ulceration ornecrosis, 12 (18%). Review of the histopathologicfeatures is detailed in Table I. The cause of the HSPcould be determined in 18 of the 68 patients:infection-induced (n = 6), drug-induced (n = 1),and infection with concomitant antibiotic use(n = 11). In the other 50 patients, the cause couldnot be definitely ascertained.

In this patient population, patients with LCV andabsence of eosinophils on histopathologic reviewwere significantly more likely to have renal involve-ment than patients with skin biopsy specimensshowing LCV with presence of eosinophils (P =.008) (Table II). In addition, patients who were olderthan 40 years were significantly more likely to haverenal disease than patients aged 40 years old oryounger (P = .03). When both age and presence ofeosinophils were taken into account, the only pa-tients at increased risk of renal involvement werethose who were both older than 40 years and hadLCV without eosinophils on skin biopsy specimen(Table III). This subgroup had a 75% rate of renaldisease (18 of 24 patients) vs 27% (12 of 44 patients)for all other patients (P\.001). Age younger than 40years was associated with increased levels of GI tractinvolvement and showed a nonsignificant trend forincreased level of joint involvement (Table IV).Finally, patients with LCV and absence of histiocyteson review of histopathologic tests were significantlymore likely to have GI tract involvement thanpatients with biopsy specimens showing the

presence of histiocytes (P = .03) (Table V). Skinulceration was not found to be a marker for systemicdisease, and other statistical tests that were per-formed on other histopathologic and clinicalmarkers were not found to be significantly associated

Table III. Rate of renal involvement as function ofboth tissue eosinophils and age

Age, y

Histopathologic

findings Patients, No.

Patients with renal

involvement, No. (%)*

# 40 LCV witheosinophils

17 5 (29)

# 40 LCV withouteosinophils

9 2 (22)

[40 LCV witheosinophils

18 5 (28)

[40 LCV withouteosinophils

24 18 (75)*

LCV, Leukocytoclastic vasculitis.

*Rate of renal involvement in subgroup with age older than 40

years and with absence of tissue eosinophils is statistically

different from all other patients combined (P\ .001).

Table IV. Association between younger age (# 40years) and gastrointestinal tract and jointinvolvement

Systemic involvement

Age, y*

P value# 40 (n = 26) [40 (n = 42)

Gastrointestinal tract .004No 10 (38) 31 (74)Yes 16 (62) 11 (26)

Joint .06No 10 (38) 26 (62)Yes 16 (62) 16 (38)

*Percentages are calculated with number of patients younger or

older than 40 years as denominator.

Table V. Association between gastrointestinal tractinvolvement and absence of histiocytes

GI tract

involvement

Histiocyte presence*

P valueAbsent (n = 54) Present (n = 14)

.03No 29 (54) 12 (86)Yes 25 (46) 2 (14)

GI, Gastrointestinal.

*Percentages are calculated with number of patients with or

without tissue histiocytes as denominator.

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with systemic involvement (Tables VI-VIII; availableat http://www.jaad.org).

DISCUSSIONOur study analyzed histopathologic features, age,

and systemic factors in 68 adult patients with HSP.Wefound lack of eosinophils on histopathologic evalu-ation and increased patient age ([40 years) to beassociated with renal disease. Conversely, younger

patients were more likely to have both GI tract andjoint involvement. In addition, absence of histiocyteson histopathologic evaluation was associated withincreased GI tract involvement. To our knowledge,this work is the first on the correlation of specifichistopathologic featureswith systemic illness in adultHSP.

Study of prognostic markers for renal involvementis an important area of investigation because itpermits stratification of patients with HSP into riskcategories to allow greater follow-up on those mostat risk for renal disease. In the current study, themostintriguing finding was the inverse associationof eosinophils on skin biopsy specimen withrenal involvement. In this patient group, roughlyhalf (51%) of the specimens had eosinophils, butthose patients who had eosinophils were only half aslikely (29%) to manifest renal disease than thosepatients whose specimens did not contain eosino-phils (61%). In previous studies, tissue eosinophilshave been observed in drug-induced cutaneoussmall-vessel vasculitis.11 In addition, it has beendocumented that drug-induced vasculitis has a lowrate of progression to renal disease.5,6 It is possiblethat in this current study population, the presence ofeosinophils was a marker of drug-induced HSP.Because the origin of the HSP could not be defini-tively determined in the large majority of our pa-tients, we are unable to test this hypothesiseffectively. However, it must be considered thateosinophils could be a marker for a less severepathologic disease course regardless of the origin. Inour clinical experience, determining the specificcause of adult HSP can be difficult because patientsoften have medication changes, infectious diseases,and other medical conditions that could precipitatean episode of vasculitis. Our findings may suggestthat the presence or absence of eosinophils on skinbiopsy specimens may be helpful in determining therisk of renal involvement in adult patients with HSPof unclear origin.

As noted in past studies,3 older age was associatedwith increased risk of renal involvement in adultpatients with HSP in the current study. We could notdetermine whether this finding is a function ofdifferent pathophysiology of adult disease or justgradually decreasing glomerular filtration rate, but itis likely that both explanations contribute. Of note,when patients were stratified by both age and tissueeosinophil presence, the only group with increasedrisk of renal disease was the patients older than 40years whose skin biopsy specimens contained notissue eosinophils. Indeed, patients with both thesefeatures had nearly a 3-times increased risk of renalinvolvement compared with all other patients who

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MARCH 2013424 Poterucha et al

did not have both features (75% vs 27%). The other 3subgroups each had a risk of renal disease of lessthan 30% (Table III). The combination of these 2features (age [40 years and skin biopsy specimenshowing LCV with a lack of eosinophils) may affordclinicians a useful renal risk stratification tool whenevaluating adult patients with HSP.

Our study has several limitations. First, it is aretrospective study. Second, the origin of the HSP inour patients, such as drug use or infection, could notbe accurately and consistently determined becauseof multiple possible causes. This limited our ability todetermine whether the lower rate of renal involve-ment observed in patients with eosinophils presenton skin biopsy specimen was solely because of adrug-induced cause or was an independent markerof better renal outcomes. Third, we were unable todetermine the age of each lesion biopsied in ourcohort of patients. Fourth, we performed a qualita-tive assessment (ie, presence or absence) of histo-pathologic features (eg, eosinophils and histiocytes)rather than a quantitative assessment of dermalinflammatory cells. Fifth, the patients in this studywere from a single academic center and often hadrecurrent disease. Cases of adult HSP seen in thegeneral community setting at smaller practice centersmay have lower rates of severe disease because ofreferral bias. Finally, given limited long-term follow-up data available in the medical records, we wereunable to document long-term outcomes of ourpatient cohort and examine for clinicopathologiccorrelations related to long-term patient outcomes.

In conclusion, we present clinical and histopath-ologic information from a series of 68 adult patientswith HSP seen at Mayo Clinic. We found the highestrisk of renal involvement in adult patients older than40 years who also had skin biopsy specimens show-ing LCV with an absence of eosinophils. Futureresearch on this topic should continue to refine

prognostic markers of renal involvement in adultHSP through prospective study design.

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3. Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D.

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Table VI. Associations with renal and gastrointestinal tract involvement for 68 patients

Feature

Renal involvement, No. (%)

P value

GI tract involvement, No. (%)

P valueNo (n = 38) Yes (n = 30) No (n = 41) Yes (n = 27)

Sex .12 .89Male 26 (68) 15 (50) 25 (61) 16 (59)Female 12 (32) 15 (50) 16 (39) 11 (41)

Age, y .19 \.001# 30 13 (34) 6 (20) 4 (10) 15 (56)[30 25 (66) 24 (80) 37 (90) 12 (44)

Age, y .03 .004# 40 19 (50) 7 (23) 10 (24) 16 (59)[40 19 (50) 23 (77) 31 (76) 11 (41)

Age, y .08 .09# 50 27 (71) 15 (50) 22 (54) 20 (74)[50 11 (29) 15 (50) 19 (46) 7 (26)

Age, y .59 .05# 60 30 (79) 22 (73) 28 (68) 24 (89)[60 8 (21) 8 (27) 13 (32) 3 (11)

Apoptosis of keratinocytes .43 .25No 32 (84) 23 (77) 35 (85) 20 (74)Yes 6 (16) 7 (23) 6 (15) 7 (26)

Papillary dermal edema .41 .12No 24 (63) 16 (53) 21 (51) 19 (70)Yes 14 (37) 14 (47) 20 (49) 8 (30)

Eccrine gland involvement .27 .94No 23 (61) 22 (73) 27 (66) 18 (67)Yes 15 (39) 8 (27) 14 (34) 9 (33)

Vesiculation .65 .25No 30 (79) 25 (83) 35 (85) 20 (74)Yes 8 (21) 5 (17) 6 (15) 7 (26)

Microabscess .70 .37No 27 (71) 20 (67) 30 (73) 17 (63)Yes 11 (29) 10 (33) 11 (27) 10 (37)

Reticular dermal inflammation 1.0 .30No 33 (87) 26 (87) 34 (83) 25 (93)Yes 5 (13) 4 (13) 7 (17) 2 (7)

Subcutaneous fat involvement .65 .64No 36 (95) 27 (90) 37 (90) 26 (96)Yes 2 (5) 3 (10) 4 (10) 1 (4)

Eosinophils .008 .58No 13 (34) 20 (67) 21 (51) 12 (44)Yes 25 (66) 10 (33) 20 (49) 15 (56)

Histiocytes .62 .03No 31 (82) 23 (77) 29 (71) 25 (93)Yes 7 (18) 7 (23) 12 (29) 2 (7)

Intravascular thrombosis .65 1.0No 36 (95) 27 (90) 38 (93) 25 (93)Yes 2 (5) 3 (10) 3 (7) 2 (7)

Cutaneous ulceration .27 .75No 33 (87) 23 (77) 33 (80) 23 (85)Yes 5 (13) 7 (23) 8 (20) 4 (15)

GI, Gastrointestinal.

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Table VII. Associations with joint involvement and any systemic signs for 68 patients

Feature

Joint involvement, No. (%)

P value

Systemic signs, No. (%)

P valueNo (n = 36) Yes (n = 32) No (n = 16) Yes (n = 52)

Sex .88 .71Male 22 (61) 19 (59) 9 (56) 32 (62)Female 14 (39) 13 (41) 7 (44) 20 (38)

Age, y .10 .03# 30 7 (19) 12 (38) 1 (6) 18 (35)[30 29 (81) 20 (63) 15 (94) 34 (65)

Age, y .06 .07# 40 10 (28) 16 (50) 3 (19) 23 (44)[40 26 (72) 16 (50) 13 (81) 29 (56)

Age, y .11 .27# 50 19 (53) 23 (72) 8 (50) 34 (65)[50 17 (47) 9 (28) 8 (50) 18 (35)

Age, y .15 .03# 60 25 (69) 27 (84) 9 (56) 43 (83)[60 11 (31) 5 (16) 7 (44) 9 (17)

Apoptosis of keratinocytes .49 .72No 28 (78) 27 (84) 14 (88) 41 (79)Yes 8 (22) 5 (16) 2 (13) 11 (21)

Papillary dermal edema .16 .73No 24 (67) 16 (50) 10 (63) 30 (58)Yes 12 (33) 16 (50) 6 (38) 22 (42)

Eccrine gland involvement .10 .39No 27 (75) 18 (56) 12 (75) 33 (63)Yes 9 (25) 14 (44) 4 (25) 19 (37)

Vesiculation .24 .27No 31 (86) 24 (75) 15 (94) 40 (77)Yes 5 (14) 8 (25) 1 (6) 12 (23)

Microabscess .64 .55No 24 (67) 23 (72) 10 (63) 37 (71)Yes 12 (33) 9 (28) 6 (38) 15 (29)

Reticular dermal inflammation .29 .67No 33 (92) 26 (81) 15 (94) 44 (85)Yes 3 (8) 6 (19) 1 (6) 8 (15)

Subcutaneous fat involvement .66 .33No 34 (94) 29 (91) 16 (100) 47 (90)Yes 2 (6) 3 (9) 0 5 (10)

Eosinophils .82 .31No 17 (47) 16 (50) 6 (38) 27 (52)Yes 19 (53) 16 (50) 10 (63) 25 (48)

Histiocytes .72 .29No 28 (78) 26 (81) 11 (69) 43 (83)Yes 8 (22) 6 (19) 5 (31) 9 (17)

Intravascular thrombosis .18 .33No 35 (97) 28 (88) 16 (100) 47 (90)Yes 1 (3) 4 (13) 0 5 (10)

Cutaneous ulceration .68 1.0No 29 (81) 27 (84) 13 (81) 43 (83)Yes 7 (19) 5 (16) 3 (19) 9 (17)

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Table VIII. Composite organ score and presence of eosinophils

Composite organ score

Eosinophil presence

Present (n = 35) Absent* (n = 33)

1 (Skin only) 10 62 (Skin 1 1 organ [renal, GI tract, or joint]) 12 113 (Skin 1 2 organs) 10 114 (Skin 1 3 organs) 3 5

GI, Gastrointestinal.

*x2 Test showed no significant association between composite organ score and absence of eosinophils (P = .67).

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