|Case for diagnosis.|
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|PMID: 23197220 Owner: NLM Status: In-Data-Review|
|Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis characterized by pustular and vegetating lesions of the skin and oral mucosa. It is considered a highly specific marker for inflammatory bowel diseases. The authors describe a case of pyodermatitis-pyostomatitis vegetans in a pediatric patient who presented marked clinical improvement after beginning treatment with oral corticosteroids, azathioprine, and dapsone. Bowel surveillance is mandatory, since the dermatosis is associated with inflammatory bowel diseases in more than 70% of patients, especially ulcerative colitis.|
|Kleyton de Carvalho Mesquita; Izelda Maria Carvalho Costa|
|Type: Journal Article|
|Title: Anais brasileiros de dermatologia Volume: 87 ISSN: 1806-4841 ISO Abbreviation: An Bras Dermatol Publication Date: 2012 Dec|
|Created Date: 2012-11-30 Completed Date: - Revised Date: -|
Medline Journal Info:
|Nlm Unique ID: 0067662 Medline TA: An Bras Dermatol Country: Brazil|
|Languages: eng Pagination: 929-31 Citation Subset: IM|
|University of Brasilia.|
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Journal ID (nlm-ta): An Bras Dermatol
Journal ID (iso-abbrev): An Bras Dermatol
Journal ID (publisher-id): An. bras. dermatol.
Publisher: Sociedade Brasileira de Dermatologia
http://www.anaisdedermatologia.org.brhttp://www.scielo.br/abd©2012 by Anais Brasileiros de Dermatologia
Received Day: 16 Month: 12 Year: 2011
Accepted Day: 13 Month: 11 Year: 2012
Print publication date: Season: Nov-Dec Year: 2012
Volume: 87 Issue: 6
First Page: 929 Last Page: 931
PubMed Id: 23197220
|Case for diagnosis*|
|Kleyton de Carvalho Mesquita1|
|Izelda Maria Carvalho Costa2|
1MSc student of Health Sciences - University of Brasilia (Universidade de
Brasilia - UnB) - Dermatologist, Federal District Department of Health (Secretaria do
Estado de Saúde do Distrito Federal - SES-DF) - Brasilia (DF), Brazil
2PhD in Dermatology, Federal University of Sao Paulo (Universidade
Federal de Sao Paulo - UNIFESP) - Professor of Dermatology, University of Brasilia
(Universidade de Brasília - UnB) - Brasilia (DF), Brazil
|Correspondence: Mailing address: Kleyton de Carvalho Mesquita. QE 34, conjunto P, casa
25. Guará II, 71065-162 Brasília, DF, E-mail address:
We describe the case of a twelve-year old patient presented with a four-month history of painful coalescent ulcerations in the oral cavity and lips, including the tongue and palate, with edema, erythema, and crusts. Three months later, he presented erythematous vegetating ulcers on the basis of the penis and in the perianal region (Figure 1). He had no other complaints.
Laboratory tests resulted in normal complete blood count, complement, and immunoglobulins. Serology for viral hepatitis, syphilis, and HIV were negative. Tests showed high inflammatory activity. The ASCA test (ASCA IgG: 46,13U; IgA: 50,89U) was positive; the p-ANCA and c-ANCA tests were negative. Colonoscopy was normal.
Histopathology of the lower lip and of the lesions on the basis of the penis showed suprabasal acantholytic cleft and a mixed inflammatory process, with eosinophils. Skin fragments from the perianal region revealed epidermal hyperplasia, neutrophil abscesses, intraepithelial eosinophils, and a moderate mixed inflammatory process, with eosinophils on the dermis (Figure 2). Direct Immunofluorescence (DIF) was negative for immunoglobulin and complement deposits in the oral mucosa.
Based on the clinical presentation and histopathological findings, the main diagnoses considered were pemphigus vegetans (a variant of pemphigus vulgaris) and pyodermatitis-pyostomatitis vegetans (PD-PSV). The differentiation between them could only be made by immunofluorescence, since clinical presentation and histopathological findings are very similar in both diseases. Direct and indirect immunofluorescence (DIF and IIF) are negative or weakly positive in PD-PSV, whereas they are positive and reveal strong intercellular deposits of IgG and C3 in pemphigus vegetans.1-3 Considering that our patient's DIF test was negative, we made the definite diagnosis of PD-PSV.
PD-PSV is a rare inflammatory disease characterized by pustular and vegetating plaques that affect the skin and mucous membranes. The etiology of PD-PSV is unknown, and its pathogenesis is poorly understood.1,4,5 It is associated with gastrointestinal disease and has been described as a highly specific marker for inflammatory bowel diseases (IBD).6,7 Diagnostic differentiation between PD-PSV and pemphigus vegetans is essential, even though immunosuppressant regimen would be similar. The association of PD-PSV with IBD is well known, and IBD precedes the onset of oral lesions by months or years in most cases. Ulcerative colitis occurs in 70-78%, and Crohn's disease is seen in 11% of patients. In about 15% of cases, skin lesions precede gastrointestinal symptoms. Therefore, patients with PD-PSV must be monitored to detect the onset of IBD. There is not a single treatment protocol, and none of the treatment regimens presented solid scientific evidence of having superior efficacy.2,3,8,9
The patient was treated with prednisone 1mg/Kg/day and azathioprine 1mg/Kg/day. Corticosteroid doses were gradually tapered and stopped at the end of six months. After normal dosing of glucose-6-phosphatedehydrogenase, dapsone 100mg/day was introduced as a corticoid-sparing agent, and azathioprine was discontinued one month later. The patient showed improvement of the lesions, which was slower for the perianal plaques (Figure 3). The patient was followed up as an outpatient for nine months. After that, dapsone was discontinued. He took part in periodic screening protocols (clinical and laboratorial) for early detection of IBD.
Conflict of interest: None
Financial funding: None
fn01* Work conducted at the Hospital University of Brasilia (Hospital Universitário de Brasília) - University of Brasilia (Universidade de Brasília - HUB-UnB) - Brasilia (DF), Brazil.
|1.||Cunha PR,Barraviera SR. Dermatoses bolhosas autoimunesAn Bras DermatolYear: 20098411112419503978|
|2.||Nigen S,Poulin Y,Rochette L,Lévesque MH,Gagné E. Pyodermatitis-Pyostomatitis Vegetans: Two Cases and a Review of the LiteratureJ Cutan Med SurgYear: 2003725025512574902|
|3.||Hegarty AM,Barrett AW,Scully C. Pyostomatitis vegetansClin Exp DermatolYear: 2004291714723710|
|4.||Matias FAT,Rosa DJF,Carvalho MTF,Castañon MCMN. Piodermatitepioestomatite vegetante: relato de caso e revisão de literaturaAn Bras DermatolYear: 2011861S137S14022068794|
|5.||Femiano F,Lanza A,Buonaiuto C,Perillo L,Dell'Ermo A,Cirillo N. Pyostomatitis vegetans: a review of the literatureMed Oral Patol Oral Cir BucalYear: 200914|
|6.||Al-Rimawi H,Hammad M,Raweily E,Hammad H. Pyostomatitis vegetans in childhoodJ PediatrYear: 1998157402405|
|7.||Leibovitch I,Ooi C,Huilgol S,Reid,James CL,Selva D. Pyodermatitis-pyostomatitis vegetans of the eyelids case report and review of the literatureOphthalmologyYear: 20051121809181316095701|
|8.||Yasuda M,Amano H,Nagai Y,Tamura A,Ishikawa O,Yamaguchi S. Pyodermatitispyostomatitis vegetans associated with ulcerative colitis: successful treatment with total colectomy and topical tacrolimusDermatologyYear: 200821714614818523389|
|9.||Ruiz-Roca J,Berini-Aytés L,Gay-Escoda C. Pyostomatitis vegetans. Report of two cases and review of the literatureOral Surg Oral Med Oral Pathol Oral Radiol EndodYear: 20059944745415772593|
Keywords: Colitis, ulcerative, Crohn disease, Eosinophilia, Inflammatory bowel diseases, Skin diseases.
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