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Paracoccidioidomycosis

Paracoccidioidomycoses

Paracoccidioidomycosis constitutes a fungal infection, caused by Paracoccidioides brasiliensis and Paracoccidioides lutzii. This condition is prevalent in Central and South America and can affect multiple systems.


Presentation

Paracoccidioidomycosis is observed both in juvenile and adult patients, with the two types of the disease having considerably distinct characteristics in terms of clinical presentation. The juvenile form can assume an acute or subacute course; a plethora of disseminated skin lesions appear within some weeks or months following the inoculation of P. brasiliensis [1]. Lesions can develop in any region of the body, but are primarily found on the face, neck, torso and perioral areas; these are typically ulcerated or vegetative [2] [3] [4]. The cases also present with hepatomegaly and lymphadenopathy, while sinus tracts are occasionally formed in the location of the enlarged lymph node. Constitutional symptoms such as malaise, fever, and loss of weight can also accompany the main infection signs. Involvement of the respiratory tract with corresponding complaints is rare but has been observed.

Adult paracoccidioidomycosis is typically chronic and may lead to the involvement of multiple organ systems. Lymphadenopathy is a significant finding and every lymph node may be affected; cervical lymph nodes are involved in the majority of the cases. Polymorphic skin lesions are usually restricted to the face and have a variable morphology. Other features that are frequently observed include those arising from the respiratory tract, such as a cough and dyspnea, as well as malaise and fever. Emphysema, pulmonary fibrosis, and bullae are direct complications and may play a significant role in the development of cor pulmonale and pulmonary hypertension in a minority of the cases. Laryngeal or pharyngeal lesions may complicate the clinical picture, with patients presenting acutely due to stridor, dysphagia, and dysphonia. Aguiar-Pupo stomatitis is another potential sign and involves concurrent nasal and pharyngeal ulceration, alongside lymphadenopathy that affects the cervical or mandibular lymph nodes. Additional symptoms encompass adrenal gland insufficiency that can be exacerbated by an Addisonian crisis (rare), bowel obstruction due to mesenteric lymph node enlargement, abdominal cramps, and hepatosplenomegaly. Central nervous system (CNS) involvement remains a characteristic that includes only the adult disease type and may cause meningitis and encephalitis [5] [6] [7].

Fever
  • As in the majority of paracoccidioidomycosis cases, pulmonary involvement results in shortness of breath, a productive cough and hemoptysis, as well as general symptoms of weight loss, fever and fatigue.[en.wikipedia.org]
  • The present report describes a patient with extensive cutaneous and pulmonary paracoccidioidomycosis, with disseminated papules and pustules, fever and pulmonary symptoms, probably related to immunosuppression caused by a renal carcinoma.[ncbi.nlm.nih.gov]
  • Other features that are frequently observed include those arising from the respiratory tract, such as a cough and dyspnea, as well as malaise and fever.[symptoma.com]
Weight Loss
  • A male patient, 45 years old, who underwent kidney transplantation 3 years ago presenting with diarrhea, severe weight loss, and anemia; no breathing complaints.[ncbi.nlm.nih.gov]
Malaise
  • Other features that are frequently observed include those arising from the respiratory tract, such as a cough and dyspnea, as well as malaise and fever.[symptoma.com]
  • A 28-year-old male patient presented with a three-month history of fever, respiratory symptoms and malaise. Chest X-rays revealed bilateral apical infiltrates, right pleuritis and hilar lymphadenomegaly.[ncbi.nlm.nih.gov]
  • Pulmonary paracoccidioidomycosis: Most cases have an indolent onset and patients present with chronic symptoms such as cough, fever, night sweats, malaise and weight loss. Chest x-rays are characteristic but not diagnostic.[web.archive.org]
  • Signs and symptoms Juvenile form Lymphadenopathy and hepatosplenomegaly Systemic symptoms - Include fever, weight loss, and malaise; present in most patients.[emedicine.com]
Plethora
  • The juvenile form can assume an acute or subacute course; a plethora of disseminated skin lesions appear within some weeks or months following the inoculation of P. brasiliensis.[symptoma.com]
Constitutional Symptom
  • Constitutional symptoms such as malaise, fever, and loss of weight can also accompany the main infection signs. Involvement of the respiratory tract with corresponding complaints is rare but has been observed.[symptoma.com]
Splenomegaly
  • Computed tomography (neck/chest/abdomen) showed generalized lymph node enlargement, splenomegaly, pleural effusion and ascites.[ncbi.nlm.nih.gov]
  • He also presented cervical, axillar and inguinal lymphadenomegaly plus hepato-splenomegaly. Biopsy samples from skin lesions and cervical lymph nodes showed diagnostic structures of P. brasiliensis ( Fig. 3 ).[elsevier.es]
  • Seventy-seven percent of the patients had disseminated disease characterized as lymphadenopathy (73%), hepatomegaly (43%), splenomegaly (29%), and osteoarticular lesions (18%).[hivinsite.ucsf.edu]
  • Predominant clinical manifestations were cervical lymph node enlargement (100%), hepatomegaly (25%), and splenomegaly (25%).[wwwnc.cdc.gov]
Cervical Lymphadenopathy
  • In addition, physical examination revealed small ulcerated cutaneous lesions ( 0.5 cm in diameter) over the face, left arm and back and cervical lymphadenopathy.[academic.oup.com]
  • He was admitted to our service on August 2005, with a 6-month history of fever, cough, emaciation (weight loss 34 kg), bloody sputum, dyspnea on exertion, and fistulous cervical lymphadenopathy.[ncbi.nlm.nih.gov]
Generalized Lymphadenopathy
  • Physical examination disclosed generalized lymphadenopathy, skin lesions, abdominal distension, mild tachypnea and a left breast mass.[ncbi.nlm.nih.gov]
  • In young people, a progressive form of the disease (akin of tuberculous sepsis in tuberculous priminfection) occurs with high prostrating fever, generalized lymphadenopathy, and pulmonary involvement with milliary lesions.[en.wikipedia.org]
Cough
  • As in the majority of paracoccidioidomycosis cases, pulmonary involvement results in shortness of breath, a productive cough and hemoptysis, as well as general symptoms of weight loss, fever and fatigue.[en.wikipedia.org]
  • A 47-year-old man from Brazil received infliximab to treat psoriatic spondyloarthropathy and presented with cough, dyspnea, weight loss, and fever. Chest computed tomography revealed a pulmonary nodule and biopsy confirmed paracoccidioidomycosis.[ncbi.nlm.nih.gov]
Hemoptysis
  • As in the majority of paracoccidioidomycosis cases, pulmonary involvement results in shortness of breath, a productive cough and hemoptysis, as well as general symptoms of weight loss, fever and fatigue.[en.wikipedia.org]
  • […] frequently present chronic or adult type represents the majority of cases ( 90%) and it usually results from the reactivation of a quiescent lung infection in adulthood the presentation is usually nonspecific and includes a cough, dyspnea, expectoration hemoptysis[radiopaedia.org]
Aspiration
  • Examination of a smear prepared from aspirated tracheobronchial secretion and stained by Grocott-methenamine-silver revealed structures consistent with Paracoccidioides brasiliensis and Strongyloides stercoralis.[ncbi.nlm.nih.gov]
Productive Cough
  • As in the majority of paracoccidioidomycosis cases, pulmonary involvement results in shortness of breath, a productive cough and hemoptysis, as well as general symptoms of weight loss, fever and fatigue.[en.wikipedia.org]
Stridor
  • Laryngeal or pharyngeal lesions may complicate the clinical picture, with patients presenting acutely due to stridor, dysphagia, and dysphonia.[symptoma.com]
Abdominal Pain
  • The current case describes a 36-year-old man who presented abdominal pain and intestinal constipation, being suspected and then confirmed as paracoccidioidomycosis after already be diagnosed with this disease by a dentist through oral manifestations.[ncbi.nlm.nih.gov]
  • Visceral: Typically, focal lesions cause enlargement mainly of the liver, spleen, and abdominal lymph nodes, sometimes causing abdominal pain. Infections may be mixed, involving combinations of all 3 patterns.[merckmanuals.com]
Abdominal Cramps
  • Additional symptoms encompass adrenal gland insufficiency that can be exacerbated by an Addisonian crisis (rare), bowel obstruction due to mesenteric lymph node enlargement, abdominal cramps, and hepatosplenomegaly.[symptoma.com]
Hepatomegaly
  • Hepatomegaly was also noted on physical examination. Radiographic examination revealed numerous rounded lytic lesions on the bones of the hands (Panel B), arms, legs, feet, and skull. A reticulonodular infiltrate was present in both lungs.[nejm.org]
  • The cases also present with hepatomegaly and lymphadenopathy, while sinus tracts are occasionally formed in the location of the enlarged lymph node.[symptoma.com]
  • […] years, when it has been more frequently described. 3,8,9,11 Recently, Morejón et al. showed that paracoccidioidomycosis and HIV-infected patients ( n 53) presented CD4 count cells/μL in 83.7%, lymphadenomegaly in 80%, cutaneous lesions in 66.7% and hepatomegaly[elsevier.es]
  • Seventy-seven percent of the patients had disseminated disease characterized as lymphadenopathy (73%), hepatomegaly (43%), splenomegaly (29%), and osteoarticular lesions (18%).[hivinsite.ucsf.edu]
  • Predominant clinical manifestations were cervical lymph node enlargement (100%), hepatomegaly (25%), and splenomegaly (25%).[wwwnc.cdc.gov]
Low Back Pain
  • However, in patients with chronic low-back pain who live or used to live in endemic regions, this infection should be considered as a possible differential diagnosis.[ncbi.nlm.nih.gov]
Back Pain
  • However, in patients with chronic low-back pain who live or used to live in endemic regions, this infection should be considered as a possible differential diagnosis.[ncbi.nlm.nih.gov]
Skin Ulcer
  • Histopathological examination of a skin ulcer and culture of purulent discharge from the lesions revealed Paracoccidioides brasiliensis .[nejm.org]
  • Symptoms are skin ulcers, adenitis, and pain due to abdominal organ involvement. Diagnosis is clinical and microscopic, confirmed by culture. Treatment is with azoles (eg, itraconazole ), amphotericin B, or sulfonamides.[merckmanuals.com]
Cutaneous Manifestation
  • Cutaneous manifestations are secondary to lymphatic and hematogenous dissemination. These deep mycoses are exceptional in Europe.[doi.org]
  • The characteristics of our case highlight the importance of investigating the epidemiological antecedents in cases of polymorphous cutaneous manifestations to avoid delay in diagnosis and diminish possible sequelae that will impact directly on the patient's[colombiamedica.univalle.edu.co]
Facial Scar
  • Even with adequate treatment of paracoccidioidomycosis, the severe fibrosis that accompanies healing often results in long-term sequelae, such as disfiguring facial scarring, chronic dyspnea and cor pulmonale (from severe pulmonary fibrosis), and buccal[emedicine.com]

Workup

Patients with a suspected paracoccidioidomycosis require an extensive workup, with an aim to confirm the infection and investigate the systems affected. Due to the fact that culture sensitivity is low and requires up to 30 days in order to render results, the most efficient and common way to diagnose the specific infection is via direct visualization of yeast in preparations collected from various regions, including cutaneous lesion scrapings, sputum or tissue per se [8]. Immunodiffusion and counterimmunoelectrophoresis can also be employed, as they both yield a satisfactory sensitivity of 80% and a specificity that exceeds 90%. The aforementioned techniques can also contribute greatly in terms of screening and follow-up. Solid-phase immunoassays can usually detect anti-gp43 antibodies in the serum and have a significant specificity and sensitivity.

The workup of pediatric patients also comprises a complete blood count, as hemoglobin is usually low and eosinophil counts are elevated. Hypergammaglobulinemia is a frequent pathological finding. Radiographs are indispensable, as the potential lymph node involvement requires accurate depiction and pulmonary function tests are also carried out to diagnose obstruction.

Adults may be eligible candidates for thoracic radiographs or computerized tomography scans (CT scans), as chronic paracoccidioidomycosis can lead to the formation of pulmonary infiltrates in the basal and/or central pulmonary regions [9]. A CT or magnetic resonance imaging (MRI) scan can also help to eliminate other conditions that can lead to similar symptomatology. Findings that suggest a possible paracoccidioidomycosis-induced CNS pathology should also be investigated via a brain CT scan or MRI scan [10].

Skin Test Positive
  • Clinical manifestations: Clinical manifestions vary from subclinical infections that are detected only by skin-test positivity; to chronic unifocal infection where only a single organ is involved; to chronic multifocal infection where more than one organ[web.archive.org]
Paracoccidioides Brasiliensis
  • Lacaz -- Paracoccidioides brasiliensis ultrastructural findings / Flavio Queiroz-Telles -- Biochemistry of Paracoccidioides brasiliensis dimorphism / Gioconda San-Blas and Felipe San-Blas -- Immunochemistry of Paracoccidioides brasiliensis antigens [worldcat.org]
  • The case herein presented is the first autochthonous report of PCM due to Paracoccidioides brasiliensis PS2 species in the state of Rio de Janeiro, Brazil, an important endemic area.[ncbi.nlm.nih.gov]

Treatment

  • Antifungal treatment is required for patients with PCM.[ncbi.nlm.nih.gov]
  • Itraconazole is now the preferred first-line treatment. Other effective antifungal treatments include voriconazole and posaconazole. Ketoconazole, while effective, has more significant toxicity and side effects.[en.wikipedia.org]

Prognosis

  • We present two cases of juvenile form of paracoccidioidomycosis (PCM), a systemic mycosis frequently found in rural areas, whose prognosis is poor in children and young adults.[ncbi.nlm.nih.gov]
  • This juvenile form has a more severe prognosis even with treatment. The most common form, the so-called adult form of paracoccidioidomycosis, is almost certainly a reactivation of the disease.[en.wikipedia.org]
  • Treatment and prognosis P. brasiliensis is a very sensitive fungus and responds to a great range of antifungal drugs.[radiopaedia.org]
  • Outlook and prognosis Overall estimated 5-30% mortality, sometimes as a result of co-morbidity such as tuberculosis or AIDS. Late diagnosis contributes to death.[life-worldwide.org]

Etiology

  • Lesions located in the distal regions of the lower limbs are not uncommon and particularly in the feet may go unnoticed or even be confused with infectious or neoplastic lesions of different etiologies.[ncbi.nlm.nih.gov]

Epidemiology

  • Disseminate the epidemiological and clinical data of unreported cases of PCM in Mexico from 1972 until 2012 is the aim of this work.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • A literature review was conducted with discussion of clinical, pathophysiological and therapeutic aspects of PCM.[ncbi.nlm.nih.gov]
  • 2012 Patient details Age Adult Location Brazil Localisation Head / face / labia Description Paracoccidioidomycosis, endemic to South America, with nodules and plaques on face, neck and oral mucosa Primary Lesions Nodule Nodule / warty Papule Plaque Pathophysiology[dermquest.com]
  • (See Pathophysiology and Etiology.)[emedicine.com]

Prevention

  • Consequently, rapid diagnosis and treatment are crucial to prevent progression of the disease, which can result in death. Currently, there are several established methods for PCM diagnosis.[ncbi.nlm.nih.gov]
  • Sulfonamides were the first treatment found to be effective for paracoccidioidomycosis, and are favored for their low cost; however, they are not as effective in preventing relapse and take longer to take effect.[en.wikipedia.org]
  • Oestrogen blocks the mould to yeast transition in the fungus, preventing infection. AIDS increases the risk of more severe infection. Smoking probably increases the risk of chronic pulmonary disease.[life-worldwide.org]

References

Article

  1. Restrepo A, McEwen JG, Castañeda E. The habitat of Paracoccidioides brasiliensis: how far from solving the riddle? Med Mycol. 2001;39:233–241.
  2. Blotta MH, Mamoni RL, Oliveira SJ, et al. Endemic regions of paracoccidioidomycosis in Brazil: a clinical and epidemiologic study of 584 cases in the southeast region. Am J Trop Med Hyg. 1999;61:390–394.
  3. Londero AT, Ramos CD. Paracoccidioidomicose: estudo clinico-micologico de 260 casos observados no interior do Estado do Rio Grande do Sul. J Pneumol. 1990;16:129–132
  4. Pereira RM, Bucaretchi F, de Melo Barison E, Hessel G, Tresoldi AT. Paracoccidioidomycosis in children: clinical presentation, follow-up and outcome. Rev Inst Med Trop S Paulo. 2004;46:127–131.
  5. de Almeida SM, Queiroz-Telles F, Teive HA, Ribeiro CE, Werneck LC. Central nervous system paracoccidioidomycosis: clinical features and laboratorial findings. J Infect. 2004 Feb;48(2):193-8.
  6. Pedroso VS, Vilela Mde C, Pedroso ER, Teixeira AL. Paracoccidioidomicose com comprometimento do sistema nervoso central: revisão sistemática da literatura. Paracoccidioidomycosis compromising the central nervous system: a systematic review of the literature. Rev Soc Bras Med Trop. 2009;42:691–697.
  7. Marques SA. Paracoccidioidomycosis: epidemiological, clinical, diagnostic and treatment up-dating. An Bras Dermatol. 2013 Sep-Oct; 88(5): 700–711.
  8. Moreto TC, Marques ME, de Oliveira ML, Moris DV, de Carvalho LR, Mendes RP. Accuracy of routine diagnostic tests used in paracoccidioidomycosis patients at a university hospital. Trans R Soc Trop Med Hyg. 2011 Aug; 105(8):473-8.
  9. Freitas RM, Prado R, Prado FL, et al. Pulmonary paracoccidioidomycosis: radiology and clinical-epidemiological evaluation. Rev Soc Bras Med Trop. 2010 Nov-Dec;43(6):651-6.
  10. Reis F, Collier PP, Souza TF, et al. Neuroparacoccidioidomycosis (NPCM): magnetic resonance imaging (MRI) findings. Mycopathologia. 2013 Feb;175(1-2):181-6.

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Last updated: 2019-07-11 20:48