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Pyodermatitis-pyostomatitis vegetans: case report andreview of medical literature*

Piodermatite-pioestomatite vegetante: relato de caso e revisão de literatura

Fernanda de Abreu Toledo Matias1 Dário Júnior de Freitas Rosa1

Maria Teresa Feital de Carvalho2 Maria Christina M. Nogueira Castañon3

Abstract: Pyodermatitis-pyos to ma ti tis vege tans is a rare inflam ma tory der ma to sis of unk nown etio logy,with a typi cal muco cu ta neous invol ve ment. We report the case of a woman with pus tu lar and vesi cu larlesions in the axil lae, evol ving with vege ta ting pla ques and pus tu les with annu lar grou ping. The disea sepro gres sed with vul var and ingui nal invol ve ment as well as invol ve ment of the oral, nasal and ocu larmucous mem bra nes. She star ted the treat ment with pred ni so ne (40 mg/day), with remis sion of the lesionsafter one month of use of such medi ca tion. Association with inflam ma tory bowel disea se occurs in 70% ofthe cases. Immunofluorescence, which is typi cally nega ti ve, helps to cha rac te ri ze the disea se. A rapid res -pon se to syste mic ste roids is expec ted. Keywords: Corticosteroids; Vesiculobullous skin disea ses, eosi no phi lia; Adrenal cor tex hor mo nes

Resumo: A pio der ma ti te-pioes to ma ti te vege tan te é uma rara der ma to se infla ma tó ria de etio lo gia des co nhe -ci da, com típi co com pro me ti men to muco cu tâ neo. Relatamos caso de pacien te femi ni na com lesões pus tu -lo sas e vesi cu lo sas em axi las, evo luin do com pla cas vege tan tes e pús tu las com agru pa men to anu lar. Houvepro gres são com com pro me ti men to vul var, ingui nal e muco sas oral, nasal e ocu lar. Proposto o diag nós ti co,optou-se por ini ciar pred ni so na 40mg ao dia, com remis são das lesões após um mês de uso da medi ca ção.A asso cia ção com doen ça infla ma tó ria intes ti nal ocor re em 70% dos casos. A imu no fluo res cên cia é um fatorque ajuda a carac te ri zar a doen ça, sendo tipi ca men te nega ti va. A rápi da res pos ta à tera pêu ti ca com cor ti cos -te roi des sis tê mi cos é espe ra da. Palavras-chave: Corticosteroides; Dermatopatias vesi cu lo bo lho sas; Eosinofilia

Received on 17.09.2010.Approved by the Advisory Board and accepted for publication on 05.10.2010.* Work carried out at the University Hospital from the Federal University of Juiz de Fora (HU – UFJF) – Juiz de Fora (MG), Brazil.

Conflict of interest: None / Conflito de interesse: NenhumFinancial funding: None / Suporte financeiro: Nenhum

1 Resident doctor of the Dermatology Service at the University Hospital from the Federal University of Juiz de Fora (HU – UFJF) – Juiz de Fora (MG), Brazil.2 Ph.D. in Dermatology. Assistant professor at the Department of Internal Medicine – Dermatology Service from the Federal University of Juiz de Fora (UFJF) –

Juiz de Fora (MG), Brazil.3 Ph.D. in Dermatology. Associate professor at the Department of Morphology from the Federal University of Juiz de Fora (UFJF) – Juiz de Fora (MG), Brazil.

©2011 by Anais Brasileiros de Dermatologia

CASE REPORT

INTRODUCTIONPyodermatitis-pyostomatitis vegetans (PD-PSV)

is a rare inflammatory dermatosis characterized bypustular and vesicular lesions and vegetating plaques,with typical mucocutaneous involvement. Cutaneouslesions can affect the genital and axillary areas as wellas the scalp. Less frequently it affects the face, chestand extremities.1,2 Mucosal involvement is char-acteized by multiple pustules and vesicles on erythe-matous base that, in general, disrupt themselvesresulting in exulcerations. Oral mucosa is the mostaffected and there might occur lesions in the nasaland conjunctival mucosae.1,2,3,4 PD-PSV has strong asso-

ciation with gastrointestinal diseases, being consid-ered a marker of inflammatory bowel disease (IBD).1,2,5

Hallopeu6 described in 1889 a new clinical enti-ty characteized by vegetating pustular lesions withbenign outcome, not associated with constitutionalsymptoms. Nine years later, five similar cases were,once more, described by him and the entity wasnamed pyodermatitis-vegetans. Two of these five casespresented the vegetating cutaneous lesions associatedwith pustular lesions in the oral mucosa. McCarthy,7 in1949, introduced the term pyostomatitis vegetants,that he supposed to be part of Hallopeu’s

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Pyodermatitis Vegetans, after reporting the case ofthree patients who presented isolated lesions int theoral mucosa with two of these cases evolving with skinlesions.

There are disagreements about the real originof this entity. Some believe that it is a form of pemphi-gus, others, an idiosyncratic reaction to skin infec-tions, a variant of dermatitis herpetiformis or a dis-tinct entity.6,7, 8-10

CASE REPORT Forty-seven year old female patient, previously

healthy, presented in november 2006,insidious onsetof pustular and vesicular lesions, pruritic,and painfulin axillae, evolving with vegetating plaques coatedwith pustules, papules and vesicles with annulargrouping in intensively erythematous (Figure 1A).There was progression with involvementof the vulva,skin of the inguinal and hypogastric areas with annu-lar vegetating plaques coated by pustules besidesexulcerated lesions in lower left periorbital regionand lower perioral region (Figure 1B). Absence oflesions in the oral mucosa at the early stage of the dis-ease. The patient denied gastrointestinal or constitu-tional symptoms.with the condition.

The patient had to be hospitalized due to theexuberance of the clinical condition and systemicantibiotic therapy (cephalothin 2g every six hours) wascarried out during seven days without success. Duringhospitalization it was performed biopsy of the axillaryvegetative lesion and histopathological study whichshowed: ulceration and adjacent epithelial hyperpla-sia, focal subepidermal cleft, mixed inflammatory infil-trate predominantely eosinophilic with perivascular

distribution, adnexa and interstitial, associated withpapillary eosinophilic abscesses and eosinophilicspongiosis (Figure 2). Presence of neutrophilic papilli-tis in rare areas. Direct and indirect immunofluorese-cence were carried out and were negative.

Laboratory investigation revealed peripheraleosinophilia (11% - 979/mm3) and increase in thespeed of ESR (erythrocyte sedimentation rate) (55 mmin the 1st hour). Antineutrophil cytoplasmic antibod-ies and antinuclear antibody were negative. Enzymesand liver function tests were normal. Colonoscopyand barium enema unchanged.

Proposed the diagnosis of PD-PSV, it was decideto start prednisone 40mg day, with remission of thelesions after a month of use of the medication, pre-senting only residual hyperchromic macules (Figure3). The medication was gradually withdrawn over 6months. Before the use of prednisone 5mg in alter-nate days the patient presented lesion recurrencebeing necessary to return to the use of the dose of 40mg/day.

In an attempt to adequately control the condi-tion and reduce the dose of prednisone, dapsone wasstarted with partial remission of the clinical condition(dapsone 100mg/day associated with prednisone5mg/day). Intermittent recurrences with the appear-ance of lesions in the oral and nasal mucosae and inthe left upper eyelid characterized by vesicles and pus-tules on the erythematous base besides the impair-ment of the left conjunctival mucosa by intense enan-thema (Figure 4).

Associated with clinical improvement there wasnormalization of eosinophil count in peripheralblood.

FIGURE 1: A. Erythema -tous papules, pustules,vesicles and lesions coat-ed by crusts that form veg-etating plaques in axillaryregion; B. Involvement ofthe vulvar, inguinal andhypogastric regions

FIGURE 2: A. Epithelialhyperplasia, focal subepi-dermal cleft, mixedinflammatory infiltratepredominantely eosino -philic with perivasculardistribution, adnexa andinterstitial, associated withpapillary eosinophilicabscesses and eosino -philic spongiosis. B. De -tail of the interstitial in -flam ma tory infiltrate pre-dominantely eosinophilic

A

B

A

B

Pyodermatitis-pyostomatitis vegetans: case report and review of medical literarture 139

DISCUSSIONPyodermatitis-pyostomatitis vegetans (PD-PSV)

is a rare eosinophilic inflammatory dermatosis ofunknown cause and difficult diagnosis characterizedby vesiculous and pustulous lesions and vegetatingplaques with mucocutaneous involvement.2 It general-ly occurs in individuals at the end of their third decadeof life being the proportion of incidence between thefemale and male sex of 3:1.1

In most cases, the oral mucosa is affected, pre-senting pustulous exophytic lesions with erythema-tous halo, friable membrane that easily disintegratesleading to exulcerated lesions. Lips, gums, soft andhard palate, vestibule and tonsils are the most fre-quently affected areas.1,5,11 Vaginal, nasal and ocularmucosae can also be affected. 2,3,4 Mucosal lesions mayprecede, may appear simultaneously or succeed skinlesions.5,11,12 The patient presented lesions in the oral,ocular and nasal mucosae during the evolution of thecondition with clinical characteristics simillar to theones described in the medical literature.

Cutaneous involvement is frequent, occuring in58% of the cases.2 It is characterized by erythematouspustulous papules, vesicles and lesions coated bycrusts which coalesce into vegetating plaques.Lessions are asymmetric and affect mainly axillae,sculp, vulva and groin. Less frequently, other regionscan also be affected like face, chest and extremities.1,2,13

In the reported case, the patient presented the charac-terisitic cutaneous manifestations in typical areas asfirst manifestations of the disease. The association ofPD-PSV with IBD is well stablished, occurring in 70%of the cases. 1 More commomly associated with ulcer-ative colitis (53%), being Crohn’s disease seen in 11%of the patients.2 Although PD-PSV is considered a

marker of IBD this connexion is not absolute and it isnot found in all described cases ,suggesting the exis-tence of a variable exclusively mucocutaneous.11

However, skin lesions may precede gastroin-testinal symptoms in approximately 15% of the casesindicating the need of regular and careful gastroen-terological monitoring of these patients.2,5,12

Approximately 26% of the cases of PD-PSV presentsome kind of liver dysfunction such as sclerosingcholangitis, chronic hepatitis and pericolangitis.2

In the present case, gastroenterological investi-gation including laboratory tests, barium enema andcolonoscopy were performed and showed nochanges. However, regular gastroenterological moni-toring is kept aiming at detecting IBD or liver dysfunc-tions at an early stage in case they occur.

There are reports of association with zinc defi-ciency, possibly caused by malabsorption resultingfrom the inflammatory bowel disease and improve-ment of the skin condition with the supplementationof this mineral.13

Etiology of PD-PSV remains unknown. Someauthors suggest an spectrum of neutrophilic der-matoses and others propose that it represents a clini-cal manifestation of pyoderma gangrenosum. Othersclassify it as a hypersensitivity reaction such as the ery-thema nodosum or pyoderma gangrenosum, associat-ed with IBD, and there are still some that associate itwith other mucosal diseases that presentintraephitelial abscesses and acantholysis such aspemphigus vegetans and vulgaris.2,5,12 Unlike PD-PSV,pemphigus vulgaris is potentially fatal and both, thevegetans and vulgaris forms, are well characterized asautoimmune bullous diseases revealing intercellular

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FIGURE 3: Residual hyper-chromic macules in axil-lary and vulvar regionsafter introduction of corti-costeroid treatment

FIGURE 4: A. Exulceratedlesions on palate; B.Vesico-pustular lesion onthe left upper eyelid onerythematous base besidesinvolvement of the leftconjunctival mucosa byintense enanthema

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deposits of IgG and C3 by direct immunofluorescenceand positivity of circulating antibodies by indirectimmunofluorescence.14 Besides that, many times theyare refractory to therapy in opposition to the excel-lent response to steroids presented by PD-PSV.

In PD-PSV, histopathology reveals pseu-doephiteliomatous hyperplasia with intraephitelialmicroabscesses and eosinophilic spongiosis.Acantholysis and suprabasal clefts can be seen. Adense perivascular inflammatory infiltrate formed byneutrophils, eosinophils, lymphocytes and plasmacells can be seen on the dermis. As for the presentcase, the histopathological findings were similar tothose found in the medical literature. Although verysimilar to the findings observed for pemphigus vege-tans there are also some characteristics of dermatitisherpetiformis such as the presence of neutrophilicpapillitis in scarce areas. These morphological aspectspossibly contribute for understanding the existing dis-agreements reported by some authors concerning thereal nature of this disease.6,7,10 Concerning the pre-dominance of eosinophils in the inflammatory infil-trate further studies, retrospective and prospective,could be valuable in trying to enframe the entity, froma morphological point of view, within the pattern ofeosinophilic dermatoses and not in the pattern ofneutrophilic dermatoses.

Immunofluorescence constitutes another fac-

tor that helps to characterize the disease. It facilitatesthe differentiation between pemphigus vegetans andPD-PSV, as this has both direct and indirect immuno-fluorescences negative. However, a direct immunoflu-orescence slightly positive does not exclude PD-PSV.As for the case presented here, both direct and undi-rect immunofluorescence were negative confirmingthe findings of medical literature.

In the reported case, the hematological and bio-chemical assessments are within the normal limits inmost cases except for an increase in the erythrocytesedimentation rate and for the peripheral eosinophil-ia that can be found in 90% of the cases, having diag-nostic value.2 Eosinophilia can be 20% bigger than thedifferential leukocyte count. Cultures for fungi, bacte-ria and viruses are generally negative.

The quick response to therapy with systemiccorticosteroids is common in PD-PSV as observed inthe reported case. Topical corticosteroids, dapsone,sulfasalazine, azathioprine and cyclosporins havealready been used with success.1,2,4,5,11,15 There is reportof the effectiveness of infliximab associated withmethotrexate to control PD-PSV.15 Total remission hasalready been observed after total colectomy for treat-ment of subjacent IBD.16

Prognosis for PD-PSV is excellent. When associ-ated with IBD its development and severity tend tofollow the progression of the basic disease. ❑

REFERENCES 1. Ko HC, Jung DS, Jwa SW, Cho HH, Kim BS, Kwon KS, et al. Two cases of

pyodermatitis-pyostomatitis vegetans. J Dermatol. 2009;36:293-7.2. Nigen S, Poulin Y, Rochette L, Lévesque MH, Gagné E. Pyodermatitis-pyostomatitis

vegetans: two cases and a review of the literature. J Cutan Med Surg. 2003;7:250-5.3. Leibovitch I, Ooi C, Huilgol SC, Reid C, James CL, Selva D. Pyodermatitis-

pyostomatitis vegetans of the eyelids case report and review of the literature.Ophthalmology. 2005;112:1809-13.

4. Peuvrel L, Barbarot S, Gagey-Caron V, Tessier MH, Cassagnau E, Stalder JF. Pyodermatitis-pyostomatitis vegetans with nasal involvement. Ann Dermatol Venereol. 2008;135:753-6.

5. Storwick GS, Prihoda MB, Fulton RJ, Wood WS. Pyodermatitis-pyostomatitis vegetans: a specif marker for inflammatory bowel disease. J AM Dermatol. 1994:31:336-41.

6. Hallopeau H. "Pyodermite vegetante". ihre Beziehungen zur dermatitis herpetiformisund dem pemphigus vegetans. Arch Dermatol Syphilol. 1898;43:289-306.

7. McCarthy P. Pyostomatitis vegetans: report of three cases. Arch Dermatol Syphilol.1949;60:750-64.

8. Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X. Lever's Histopathology of the skin. 10th ed. Philadelphia: Lippincott Williams & Wilkins, 2009. p. 546.

9. Degos R, Carteaud A. Pyodermite végétante de Hallopeau. Ann Dermatol Syph. 1953;80:254-62.

10. McCarthy P, Shklar G. A syndrome of pyostomatitis vegetans and ulcerative colitis. Arch Dermatolol. 1963; 88:913-9.

11. O'Hagan AH, Irvine AD, Allen GE, Walsh M. Pyodermatitis-pyostomatitis vegetans: evidence for an entirely mucocutaneous variant. Br J Dermatol. 1998;139: 552-3.

12. Soriano ML, Martinez N, Grilli R, Farina MC, Martin L, Requena L. Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999;87:322-6.

13. Ficarra G, Cicchi P, Amorosi A, Piluso S. Oral Crohn's disease and pyostomatitisvegetans. Oral Surg Oral Med Oral Pathol. 1993;75:220-4.

14. Cunha PR, Barraviera SRCS. Dermatoses bolhosas autoimunes. An Bras Dermatol. 2009:84:111-24

15. Bens G, Laharie D, Beylot-Barry M, Vergier B, Noblesse I, Beylot C, et al. Succesfultreatment with infliximab and methotrexate of pyostomatitis vegetans associated with Crohn's disease. Br J Dermatol. 2003;149:181-4.

16. Yasuda M, Amano H, Nagai Y, Tamura A, Ishikawa O, Yamaguchi S. Pyodermatitis-pyostomatitis vegetans associated with ulcerative colitis: successful treatment with -total colectomy and topical tacrolimus. Dermatology. 2008;217:146-8.

MAILING ADDRESS / ENDEREÇO PARA CORRESPONDÊNCIA:Fernanda de Abreu Toledo MatiasAv. Eugênio do Nascimento s/nº Bairro: Dom36038 330 Bosco Juiz de Fora - MG, BrazilE-mail: feabreumed@yahoo.com.br

How to cite this article/Como citar este artigo: Matias FAT, Rosa DJF, Carvalho MTF, Castañon MCMN.Pyodermatitis-pyostomatitis vegetans: Case report and review of medical literarture. An Bras Dermatol.2011;86(4 Supl 1):S137-40.