Print version ISSN 0365-0596
An. Bras. Dermatol. vol.87 no.6 Rio de Janeiro Nov./Dec. 2012
WHAT IS YOUR DIAGNOSIS?
Caso para diagnóstico
Kleyton de Carvalho MesquitaI; Izelda Maria Carvalho CostaII
IMSc 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
IIPhD 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
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.
Keywords: Colitis, ulcerative; Crohn disease; Eosinophilia; Inflammatory bowel diseases; Skin diseases
A piodermatite-pioestomatite vegetante é uma dermatose mucocutânea rara, caracterizada por lesões pustulosas e vegetantes na pele e mucosa oral. É considerada um marcador altamente específico para doenças intestinais inflamatórias. Os autores descrevem um caso de piodermatite-pioestomatite vegetante em paciente pediátrico, o qual apresentou boa resposta a corticoterapia oral associada à azatioprina e à dapsona. A vigilância intestinal é mandatória, uma vez que a dermatose está associada a doenças intestinais inflamatórias em mais de 70% dos casos, especialmente a colite ulcerativa.
Palavras-chave: Colite ulcerativa; Dermatopatias; Doença de Crohn; Doenças inflamatórias intestinais; Eosinofilia
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.
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Kleyton de Carvalho Mesquita.
QE 34, conjunto P, casa 25. Guará II
71065-162 Brasília, DF
E-mail address: firstname.lastname@example.org
Received on 16.12.2011.
Approved by the Advisory Board and accepted for publication on 13.11.2012.
Conflict of interest: None
Financial funding: None
* Work conducted at the Hospital University of Brasilia (Hospital Universitário de Brasília) - University of Brasilia (Universidade de Brasília - HUB-UnB) - Brasilia (DF), Brazil.