Sindrome de addison

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Sindrome de addison

  1. 1. Braz J Oral Sci. April/June 2004 - Vol. 3 - Number 9 Addison’s disease associated to paracocidioidomycosisClaudio Maranhão Pereira3Silmara Regina Silva1 AbstractPriscila Spaziane Camargo1 Paracoccidioidomycosis is a deep mycosis caused by theAndrea Mantesso2 Paracoccidioides brasiliensis and it has been considered the most fre-Rodrigo Calado Nunes Souza 1 quent systemic mycosis in Latin America. Mulberry-like ulcers on the1 Dentistry Section, Municipal Healthy Service, oral mucous membranes are one of the earliest manifestations of theCampinas-SP, Brazil disease. Infection may remain subclinical, localized or disseminate oc-2 Oral Pathology, São Leopoldo Mandic Dental casionally.Research Institute, Campinas-SP, Brazil Adrenal involvement by granulomatous diseases is the main cause of3 Oral Pathology, School of Dentistry of this gland insufficiency in Brazil. The aim of this study is to report onePiracicaba, UNICAMP, São Paulo, Brazil case of oral paracoccidoidomycosis that provided the diagnosis of the systemic mycosis disease with secondary Addison’s disease. In spite of not being rare the involvement of the adrenal glands for the P. brasiliensis, it was not possible to find in the literature another case with oral clinical and progression features similar to this.Received for publication: September 30, 2003 Key Words:Accepted: March 2, 2004 Addison’s disease, paracoccidioidomycosis, adrenal glands.Correspondence to:Claudio Maranhão PereiraFaculdade de Odontologia de Piracicaba-UNICAMPSemiologia e Patologia Oral -Diagnóstico OralAv. Limeira, 901 - Areião - Caixa Postal 52 -CEP: 13414-900 - Piracicaba/SP - Brazil+ 55 19 3412 5213;Fax: + 55 19 3412 5218E-mail: claudiomaranhao@hotmail.com 475
  2. 2. Braz J Oral Sci.3(9):475-478 Addison’s disease associated to paracocidioidomycosisIntroduction were observed by special stain SPA (Schiff’s periodic acid).Paracoccidioidomycosis (South America blastomycosis) The clinicopathological features allowed the diagnosis ofis a chronic mycosis disease caused by the dimorphic paracocidioidomycosis. After diagnosis, chestfungus Paracoccidioides brasiliensis 1-2 . Geographic radiographs were made and confirmed pulmonaryfungal distribution is restricted to Latin America where it involvement.is considered the most prevalent systemic mycosis 3-5. It The patient was referred to the Municipal Center of Infectis still relatively unknown in the United States and Disease for further management of his systemic disease.Europe 4,6 . At the moment, it was suspected involvement of theP. brasiliensis may grow in the soil, in water, on plants in adrenal glands due to the history of progressive skinrural areas, and reaches the human host by the inhalation darkening, dysphagia and weight loss (Figure 3). Afterof airbone propagules 5. Ninety per cent of cases have hormonal level exams of adrenal glands, that showed lowoccurred in males patients with rural activities7, and peak basal steroid levels and lack of response to ACTHincidence in the 30 to 50 year age group1-2,6. The disease (adrenocorticotropic hormone) therapy, the diagnosis ofhas many clinical forms, particularly lymph node and Addison’s disease caused by P. brasiliensis was made.pulmonary involvements as well as mixed forms1, 2, 8, 9. One The patient was treated with steroids, sulfa and, after 5of the early and more frequent manifestations is the months, the oral lesion resolved, but the clinical featurespresence of mulberry-like ulcers on the oral mucous of adrenal complications yet persist. The patient was notmembranes 2,7,10-11. returned to the Medical Service for follow-up.Infection may remain subclinical, localized or mayoccasionally disseminate 6,12 . Hematogenousdissemination of paracoccidioidomycosis to abdominallymph nodes, spleen, liver, adrenal glands, skin, or braincan result in life-threatening complications 1,4,9. Adrenalglands involvement has been documented and post-mortem studies report adrenal abnormalities in 44 to 80%of cases 13-15 . Paracoccidioidomycosis is regularlyassociated with adrenal insufficiency in 10-15% of !symptomatic cases 14-16. ! !Although the involvement of the adrenal glands for P. !brasiliensis is not uncommon, there is not many casesreported Addison’s disease associated to oral !paracoccidioidomycosis. A case of oralparacoccidioidomycosis with involvement of adrenalglands is reported herein. Fig. 1. Ulcerative lesion in right lateral-posterior/venter tongue.Clinical caseA 61-year-old male was referred to the Municipal DentistryService of Campinas-SP, Brazil, in November 2001,complaining about a painful ulcerative lesion on the rightposterior-lateral/venter tongue lasting 2 months. Thepatient had a 3-week history of mild progressive darkeningof the skin, dysphagia and weight loss. He smoked formore than 30 years.On clinical examination, the patient had a 2.0 X 2.0 cmulcer on the right lateral/venter tongue. The ulcer had agranular surface with little inflammatory halo (Figure 1).With clinical diagnosis was squamous carcinoma, thepatient was submitted to an incisional biopsy. Themicroscopic examination showed non-necrotizinggranulomas with abundant multinucleated giant cells inthe conjunctive tissue, and zones of Fig. 2. Histological aspect of oral lesion. Observe area of focalpseudoepitheliomatous hyperplasia (Figure 2). Yeast-like granulomatous reactions showing multinucleated giants cells withorganisms with thick capsules in multiple budding forms P. brasiliensis within the cytoplasm (H.E, X 100).476
  3. 3. Braz J Oral Sci.3(9):475-478 Addison’s disease associated to paracocidioidomycosis P. brasiliensis could be detected in 22% to 48% of patients, with a 9% to 14% prevalence of symptomatic Addison’s disease14,19,22. Colombo et al4, 1994, reported hypofunction of adrenal glands in only 14% of patients with disseminated paracoccidioidomycosis. The higher prevalence of adrenocortical hypofunction is associated with the disseminated forms of paracoccidioidomycosis; however, the possibility of exclusive adrenal involvement as the sole manifestation of the disease has been demonstrated 14,16-17. The relationship between the pathogenetic factors of paracocidioidomycosis and adrenocortical hormones has not been explored. In recent years, many studies have documented that numerous cytokine family members influence the secretory activity of the hypothalamic- pituitary-adrenal (HPA) axis, which generally inhibit or modulate inflammation through the immunossuppressive effects of the glucocorticoids23. More recently, the adrenal androgen dehydroepiandrosterone sulfate (DHEA-S) was implicated as an immunomodulator hormone24. Leal et al.25Fig. 3. Face of the patient. He had a history of mild progressive (2003) examined the functional status of adrenocorticaldarkening of the skin and weight loss. hormones and their relationship with inflammatory cytokine patterns in patients with activeDiscussion paracocidioidomycosis. Their findings demonstrate aIt is generally accepted that in typical Addison’s disease, significant inverse correlation between DHEA-S andthe destructive lesion of the adrenal glands must be of a interleucine-6 plasma levels in paracocidioidomycosis,chronic progressive nature such as might be associated which may be of pathogenetic significance in this andwith amyloidosis, tumor formation, tuberculosis, and other others inflammatory diseases 25 .infectious diseases 16-18 . Adrenal involvement by Although the typical oral paracoccidioidomycosis lesionsgranulomatous diseases is the main cause of the gland consist of multiples mulberry-like ulcers in palate, gums,insufficiency in Brazil 1,14 , but infectious forms of the buccal mucosa, lips, and tongue, the patient reportedAddison’s disease are relatively rare in the United States18. herein showed only one ulcerative lesion withAmong these, paracoccidioidomycosis is considered the granulomatous surface and inflammatory halo. Thesemost prevalent systemic mycosis in Latin America1,3,14. clinical features, associated with chronic smoking and Paracoccidioides brasiliensis exhibits a high tropism for drinking habits, lead to a wrong clinical diagnosis ofthe adrenal glands which results in a low hormone reserve squamous carcinoma. On the other hand, with definitiveand, in more severe cases, in symptoms of primary adrenal diagnosis of paracoccidioidomycosis, it was possible toinsufficiency 19 . Adrenal involvement has been suspect of adrenal glands involvement due to the patientdemonstrated in 21% to 80% of autopsy cases 20 . had history of mild progressive darkening of the skin,Azevedo 21, in 1934, was the first to report one case with dysphagia and weight loss.extensive necrotic changes in adrenal gland caused by P. Paracoccidioidomycosis is most relevant to dentistrybrasiliensis. Del Negro 14, 1961, reported involvement of because lesions may involve especially the head and neck,the adrenal glands in 3 paracocidioidomycosis patients typically the oral and nasal mucosa, and facial skin2. Inautopsied in study with 27 individuals. spite of not being rare the involvement of the adrenalSeveral reports have described adrenal dysfunction in glands for the P. brasiliensis, it was not possible to findparacocidioidomycosis. These studies usually focused on in the literature a similar case that the oralcortisol responses to synthetic ACTH paracoccidoidomycosis lesion provided the diagnosis of(adrenocorticotropic hormone) administration and mycosis disease disseminated with secondary Addison’sestablished graded impairment of adrenal dysfunction 13- disease caused by P. brasiliensis. The patient reported14,22 . Because differing methodologies were used to herein received diagnosis of paracoccidioidomycosis andinterpret the data, comparisons are difficult. Addison’s disease due to one oral lesion. It was confirmedAdrenocortical hypofuction associated with disseminated the value of dentistry on systemic diseases diagnosis. 477
  4. 4. Braz J Oral Sci.3(9):475-478 Addison’s disease associated to paracocidioidomycosisReferences 22. Adab A, Gomez I, Velez P, Restrepo A. Adrenal function in1. Franco M, Mendes RP, Moscardi-Bacchi M, Rezkallah-Iwaso paracoccidioidomycosis a prospective study in patients before M, Montenegro MR. Paracoccidioidomycosis. Bailliere’s Clin and after ketoconazole therapy. Infection 1986; 14: 22-32. Trop Med Cornmun Dis 1989; 4: 185-220. 23. Turnbull AV, Rivier CL. Regulation of the hypothalamic-2. Almeida OP, Jorge J, Scully C, Bozo L. Oral manifestations of pituitary-adrenal axis by cytokines: actions and mechanisms of paracoccidioidomycosis (South American blastomycosis). Oral action. Physiol Rev 1999; 79: 61-71. Surg Oral Med Oral Pathol 1991; 72: 430-35. 24. Suzuki T, Suzuki N, Daynes RA, Engleman EG.3. Marques SA, Franco MF, Mendes RP, Silva NC, Baccili C, Curcelli Dehydroepiandrosterone enhances IL-2 production and ED et al. Aspectos epidemiológicos da paracoccidioidomicose cytotoxic effector function of human T cells. Clin Immunol na área endêmica de Botucatu (São Paulo, Brazil). Rev Inst Med Immunopathol 1991; 61:202–11. Trop São Paulo 1983; 25: 87-92. 25. Leal AMO, Magalhães PKR, Martinez R, Moreira AC.4. Brummer E, Castaneda E, Restrepo A. Paracoccidioidomycosis: Adrenocortical hormones and interleukin patterns in an updape. Clin Microbiol Rev 1993; 6: 89-117. paracocidioidomycosis. J Inf Dis 2003;187:124-27.5. San-Blas G. Paracoccidioidomycosis and its etiologic agent Paracoccidioides brasiliensis. J Med Vet Mycol 1993; 31: 99-113.6. Murray HW, Littman ML, Roberts RB. Disseminated paracoccidioidomycosis (South American blastomycosis) in the United States. Am J Med 1974; 56: 209-20.7. Scully C, Almeida OP. Orofacial manifestations of the systemic mycosis. J Oral Pathol Med 1992; 21: 289-94.8. Restrepo A, Trujillo M, Gomez I. Inapparent lung involvement in patients with the subacute juvenile type of paracoccidioidomycosis. Rev Inst Med Trop São Paulo 1989; 31: 18-22.9. Londero AT, Ramos CD, Lopes JOS. Progressive pulmonary paracoccidioidomycosis: a study of 34 cases observed in Rio Grande do Sul (Brazil). Mycopathologia 1978; 63: 53-6.10. Bicalho RN, Espírito Santo MF, Ferreira de Aguiar MC, Santos VR. Oral paracoccidiodomycosis: a retrospective study of 62 brazilian patients. Oral Dis 2001; 7: 56-60.11. Sposto MR, Scully C, Almeida OP, Jorge J, Graner E, Bozzo L. Oral paracoccidioidomycosis. A study of 36 South American patients. Oral Surg Oral Med Oral Pathol 1993; 75: 461-5.12. Restrepo A, Robedo M, Guitierrez F, Sanclemente M, Castaneda E, Calle G. Paracoccidioidomycosis (south American blastomycosis): a study of 39 cases observed in Medellin, Colombia. Am J Trop Med Hyg 1970; 19: 68-76.13. Colombo AL, Faiçal S, Kater CE. Systematic evaluation of the adrenocortical function in patients with paracoccidioidomycosis. Mycopathologia 1994; 127: 89-93.14. Del Negro G, Wajchenberg BL, Pereira VJ, Shnaider J, Cintra ABU, Assis LM et al. Addison’s disease associated with south American blastomycosis. Ann Int Med 1961; 54: 189-97.15. Faiçal S, Borri ML, Hauache OM, Ajzen S. Addison’s disease caused by Paracoccidioides brasiliensis: diagnosis by needle aspiration biopsy of the adrenal gland. AJR Am J Roentgenol 1996; 166: 461-2.16. Torres CM, Duarte E, Guimãres JP, Moreira LF. Destructive lesion of the adrenal gland in South American blastomycosis (Lutz’ disease). Am J Pathol 1952; 28: 145-55.17. Onate JM, Tobon AM, Restrepo A. Adrenal gland insufficiency secondary to paracoccidioidomycosis. Biomedica 2002; 22: 537-41.18. Ten S, New M, MacLaren N. Clinical review: Addison’s disease. J Clin Endocrinol Metab 2001; 86: 2909-22.19. Do Valle AC, Guimarães MR, Cuba J, Wanke B, Tendrich M. Recovery of adrenal function after treatment of paracoccidioidomycosis. Am J Trop Med Hyg 1993; 48: 626-9.20. Halvorsen RA, Heaston DK, Johnston WW, Ashton PR, Burton GM. CT guided thin needle aspiration of adrenal blastomycosis. J Comput Assist Toogr 1982; 6: 389-91.21. Azevedo AP. Blastomycose da glândula suprarenal, por Coccidioides immitis, sem lesão lymphaticas e com foco de fibrose nos pulmões. Mem Inst Oswaldo Cruz 1934; 29: 189- 93.478

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