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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.87 no.2 Rio de Janeiro Mar./Apr. 2012
Multiple cavitary pulmonary nodules in association with pyoderma gangrenosum - case report*
Múltiplos nódulos pulmonares cavitados em associação com pioderma gangrenoso - relato de caso
Maraya de Jesus Semblano BittencourtI; Luciana Fontenele Brito SoaresII; Lilia Sampaio LobatoIII Alexandre Dias MançanoIV; Helandro Stuart Castro LeandroV; Diana Mendes da FonsecaVI
IMaster's degree in tropical diseases - Physician at the Fundação Santa Casa de Misericórdia do Pará (FSCMPA) - Belém (PA), Brazil
IISpecialist in Radiology and Diagnostic Imaging by the Brazilian College of Radiology and Diagnostic Imaging - Physician Radiologist at Hospital Anchieta - Brasília (DF), Brazil
IIISpecialist in Radiology and Diagnostic Imaging by the Brazilian College of Radiology and Diagnostic Imaging - Physician Radiologist at the Fundação Santa Casa de Misericórdia do Pará (FSCMPA) - Belém (PA), Brazil
IVSpecialist in Radiology and Diagnostic Imaging by the Brazilian College of Radiology and Diagnostic Imaging - Physician Radiologist at Hospital Anchieta - Brasília (DF), Brazil
VSpecialist in Internal Medicine - Resident physician of the Nephrology Service of the Teaching Hospital de Clínicas Gaspar Vianna (FHCGV) - Belém (PA), Brazil
VIResident physician of the Dermatology Service of the Universidade Federal do Pará (UFPA) - Belém (PA), Brazil
Pyoderma gangrenosum is a rare neutrophilic disease of unknown origin that is associated with systemic diseases in 50% of cases. It is characterized by erythematous-violaceous nodular lesions that quickly progress to painful ulcers, with undermined edges, necrotic-hemorrhagic, varying in size and depth, located mainly in the lower limbs. Extracutaneous locations of pyoderma gangrenosum are rare, usually involving the lungs; the main differential diagnosis in these cases is Wegener granulomatosis. We report a case of pyoderma gangrenosum, which showed multiple cavitary lung nodules, with good response to high doses of steroids. Once excluded the possibility of Wegener granulomatosis, the authors concluded that it was the manifestation of systemic pyoderma gangrenosum with pulmonary involvement.
Keywords: Cavitation; Multiple pulmonary nodules; Pyoderma gangrenosum; Wegener granulomatosis
Pioderma gangrenoso é doença neutrofílica rara de etiologia desconhecida, que se associa a doenças sistêmicas em 50% dos casos. Caracteriza-se clinicamente por lesão nodular eritematoviolácea ou pústula que progride rapidamente para úlcera dolorosa, de bordas irregulares, fundo necrohemorrágico e localização preferencial nos membros inferiores. Manifestações sistêmicas do Pioderma gangrenoso são raras, envolvem geralmente os pulmões e o principal diagnóstico diferencial nestes casos é a granulomatose de Wegener. Relatamos um caso de paciente portador de pioderma gangrenoso que apresentava múltiplos nódulos pulmonares cavitados, com boa resposta a altas doses de corticoterapia. Uma vez excluída a possibilidade de granulomatose de Wegener, os autores concluíram tratar-se da manifestação sistêmica do pioderma gangrenoso.
Palavras-chave: Cavitação; Granulomatose de Wegener; Nódulos pulmonares múltiplos; Pioderma gangrenoso
Pyoderma gangrenosum (PG) is a chronic, ulcerative, destructive, neutrophilic disease of unknown etiology that in about 50% of cases is associated with systemic illnesses such as inflammatory bowel disease, rheumatoid arthritis, monoclonal gammopathy, immunological dysfunction, hematological disorders and malignancy.1-4 Systemic involvement is rare in PG and the lungs are the most affected organs. 5-9 The main differential diagnosis in these cases is Wegener granulomatosis (WG).
A 17-year-old male patient had presented ulceronodular lesions in the inguinal region for 6 years and received several previous treatments, without improvement. One month before cutaneous lesions worsened, he had dyspnea, cough with hemoptysis, chest pain and febrile episodes. The dermatological exam revealed ulcers with discretely elevated erythematous-violaceous borders, necrotic and hemorrhagic base bilaterally in the inguinal region. There were no palpable lymph nodes (Figure 1). The histopathological exam of the cutaneous lesion showed spongiotic foci, lymphocyte and eosinophil exocytosis in the epidermis and, in the dermis, vascular proliferation and dense mixed inflammatory infiltrate composed of frequent neutrophils and eosinophils around vessels, annexes and dermal interstices (Figure 2). Atypias and parasites absent in exam with H&E. The correlation of cutaneous and histopathological findings was compatible with PG. The computerized tomography of the thorax revealed the presence of multiple pulmonary nodules, some of them with central cavitation, without mediastinal lymph node enlargement or pleural effusion (Figures 3, 4 and 5). Myelogram: reactive hypercellularity. Colonoscopy: non specific, mild chronic colitis. The pulmonary biopsy was not carried out due to lack of operational conditions. Tomography of facial sinuses, bronchoalveolar lavage, bronchoscopy, AARB search in sputum examination, CA 19-9, CEA, alpha-fetoprotein, VDRL, Antinuclear antibodies (ANA) or antinuclear factor (ANF) profile, antiDNA, complements C3, C4 and CH50, anti SM, anti RNP, antiRo, cANCA and pANCA, hemocultures, urea, creatinine and EAS without alterations. After two weeks of corticotherapy at full dose the patient obtained expressive improvement of the symptom complex and thorax control tomography showed evidence that the cavitary lung nodules has disappeared (Figure 6). Since there was no involvement of the upper respiratory tract or kidney alteration, cANCA and pANCA were negative and fast response to corticotherapy, WG hypothesis was excluded and the diagnosis of PG pulmonary manifestation confirmed.
Pyoderma gangrenosum (PG), first described by Brunsting and O'Larry in 1930, is a rare neutrophílic, ulcerative, painful and non infectious dermatosis, described as having a strong association with ulcerative colitis of unknown pathogenesis. 1,2,3 It is clinically characterized by erythematous-violaceous nodular lesion or pustule that progresses quickly to painful single or multiple ulcer, with irregular and loose borders, necrotic and hemorrhagic base, preferentially located on lower limbs.2,4 It may be classified into four types, based on clinical and histopathological characteristics: classical (ulcerative), pustular, bullous and vegetative.1,3 Skin biopsy is essential for diagnosis confirmation.4 The differential diagnosis includes systemic vasculitis, infection, ischemic ulceration and primary T-cell lymphoma. The aggressive nature of ulcers, negative cultures for bacteria and fungi as well as compatible histopathology assist in the diagnosis.8
The clinical progression usually has a chronic course, with frequent recurrences. Until now, PG treatment is considered controversial and there are no established protocols. Treatment options are: broad spectrum antibiotics, corticosteroids, immunosuppressors and immunomodulators. Prednisone in high doses (1mg/kg/day) is the most effective agent for control of the systemic manifestations of PG, especially the pulmonary form. 6,9,10 The pulmonary manifestation of PG may present as solitary unilateral opacity, interstitial pneumonitis, pleural effusion and multiple pulmonary nodules, the most frequent form. 6,11-15 The main differential diagnosis in such cases is WG, which makes the computed tomography-guided lung biopsy necessary. 6,8,9 PG and WG, in a limited form, present similarities in the clinical and histopathological presentations.3-5 In lung PG, however, the inflammatory lesions are aseptic, without necrotizing granulomatous inflammation or vasculitis. 8,10 In addition, the c-Anca is highly specific for WG, present in 80-90% of the patients with classical WG and in 55-66% if those with limited WG. We report a rare case of PG with cutaneous and systemic manifestation of cavitary pulmonary nodules. Only eleven similar cases have been published in the international literature. 9 It is of fundamental importance to know this clinical association, so little known by dermatologists and radiologists, for more success in the diagnosis, treatment and followup of these patients.
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Maraya de Jesus Semblano Bittencourt
Travessa Timbó, 1890, Aptº 905 - Marco
CEP: 66085-654 - Belém, PA - Brazil
Received on 06.03.2011
Approved by the Advisory Board and accepted for publication on 15.05.2011
Conflict of interest: None
Financial funding: None
* Work carried out at the Fundação Santa Casa de Misericórdia do Pará (FSCMPA) - Belém (PA), Brazil.