Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.1 Rio de Janeiro Jan./Feb. 2011
Priscilla Maria Rodrigues PereiraI; Patrícia Bandeira de Melo AkelII; Livia Lima de LimaIII; Eduardo Nobuo KimuraIV; Alex Panizza JalkhV
IMedical doctor, Dermatology resident from the Federal University of Amazonas (UFAM) - Manaus (AM), Brazil
IIMaster's degree in Tropical Pathology from the Federal University of Amazonas (UFAM). Accredited Dermatology professor from UFAM. Dermatology preceptress in the residency program from the Getúlio Vargas University Hospital (HUGV) - Manaus (AM), Brazil
IIIMedical doctor, Dermatology resident from the Alfredo da Matta Foundation (FUAM) - Manaus (AM), Brazil
IVDermatologist from the Federal University of Amazonas (UFAM) - Manaus (AM), Brazil
VDermatologist from the Federal University of Amazonas (UFAM). Volunteer professor from the Federal University of Amazonas (UFAM) - Manaus (AM), Brazil
Paracoccidioidomycosis is an endemic systemic mycosis in Brazil, frequent in the rural areas and often in adult men. It is reported the case of a farmer, who is an illicit drugs' user, with insidious manifestations affecting kidneys, lungs, lymphonodes, bones and lately, the skin, with a delay of more than one year in the diagnosis and effective therapy It is important to include paracoccidioidomycosis as differential diagnosis, even in the absence of cutaneous lesions, for early recognition and treatment, given the high mortality of this entity.
Keywords: Diagnosis; Paracoccidioidomycosis; Skin manifestations
Paracoccidioidomycosis (or South American blastomucosis, or Lutz-Splendore- de Almeida disease) is a systemic mycosis of high prevalence in Brazil, native of the Americas, caused by Paracoccidioides brasiliensis, a dimorphic fungus.1,2,3 It was originally described in Brazil, by Adolfo Lutz, in 1908.2,3 It is endemic in the country predominantly in the south, south-east and midwest regions of Brazil.3,4 The main source of infection is inhalational and the pulmonary complex can be eliminated becoming a quiescent focus or progressing to internal organs.1,5 In most cases (70-80%), paracoccidioidomycosis (PCM) is multifocal.2
Twenty year-old male patient, born as a farmer and raised in Santarém-Pará. Two years before he had shown high fever, axillary and cervical lymphadenopathy, weight loss, dry cough, backache and asthenia. Has a background history of alcoholism, smoking and chronic a user of cocaine. The patient presented nephritic proteinuria and right pleural effusion. After comprehensive clinical investigation it was introduced as a treatment for linphadenopathyc tuberculosis. After 6 months, while still undergoing regular treatment for tuberculosis, returns maintaining of the previous condition and with a generalized micro and polyadenopathy besides erythematous nodules on dorsum of the thorax, abdomen and the neck, with a pleuritic pain, hepatomegaly and ascites. During hospitalization it was carried out tomography of the chest that revealed a right pleural efusion, hypodense lesions and osteolytic lesions in the sternum and right rib (Figures 1 and 2). Doctors suspected of sarcoidosis and the medication for tuberculosis was suspended. Evolved with worsening of the dematological condition and growth of new nodular-tumor lesions with signs of suppuration and flogose (Figure 3) requiring a dermatological evaluation. The first skin biopsy showed chronic granulomatous dermatitis, subepidermal, nonspecific, with absence of alcohol-acid resistant bacilli and a negative Grocott staining for fungi. It was carried out a new skin biopsy and a swab of a ganglion exudate. On direct microscopic examination it was observed the presence of roundish cells, birefringent, with multiple buddings, compatible with Paracoccidioides brasiliensis (Figure 4), that were confirmed by culture. Histopathology revealed granulomas with fungal yeast cells with a double membrane wall in multiple budding that is best seen when stained by silver.. Amphotericin B was used for the treatment, with clinical improvement after a cumulative dose of 1g (Figure 5). The patient was discharged after 2 months with sulfamethoxazole and trimethoprim for maintenance and he had regular dermatological and pulmonary monitoring, without presenting new lesions for 1 year.
PCM occurs mainly in men (9-13:1), from 30-60 years of age, in the rural area, being rare in children and young adults.2,5 It presents acute, subacute or chronic evolution that can affect one (unifocal) or more organs (multifocal).5,6 In this case the profes-sion, origin and the chronicity of the condition were relevant aspects in clinical suspicion of this mycosis. However, the systemic onset presented itself gradually and slowly, hampering the definitive diagnosis.
Mechanisms related either to the resistance or to the susceptibility of males to Paracoccidioides brasiliensis are still unknown.5,7 Factors linked mainly to the host are nutritional and socioeconomical factors, co-infections, immunosuppressive therapeutic, alcoholism and smoking.8,9 Airways and lungs are the most common sites of inoculation and initial location of the disease, ranging from 50 to more than 90% of the cases.7,9,10 Usually, respiratory symptoms are nonspecific such as fever, chest pain, cough, expectoration, hemoptysis and dyspnea in extensive forms and chest X-ray shows in 80-90% of the cases, bilateral images, macro and micronodular, infiltrative or interstitial, associated to fibrosis or opacification, usually in the medial and inferior third of the lobes.4,8,7,10 Miliaria, pneumonic and cavitary9 lesions are also found. It is rare the occurence of ascites and pleural infusion8 , that was seen on the very beginning of the clinical condition of this patient. This association with lymphadenopathy led to the initial hypothesis of tuberculosis which was only discharged after therapeutic trial, showing the importance of concomitant tuberculosis investigation, that occurs in 12% of the cases.7
PCM can take various clinical forms, depending on the affected organ.7 Frequency, number and morphology of skin lesions are consequence of the agent/host interaction.1 Purely cutaneous disease incidence ranges between 12-15% and originates from hematogenous dissemination of the fungus;10 of contiguous pre-existing lesion; or rarely, from direct inoculation.1,8 The hematogenous pathway is predominant and, in general, with multiple lesions, as reported here.1 Skin lesions following contiguous bone injury is rare1. The skin alterations are polymorphic ranging from acneiform lesions to exudative and/or ulcerativevegetative nodules.1,5,7 Except for anthrax, there is no pathognomonic skin condition.1
Lymph nodes are affected secondarily to skin and/or visceral involvement, being predominant at the cervical or submandibular, supracavicular and abdominal lesions, simulating linphadenopathic tuberculosis.2,9 Adenopathies can be regional or generalized, with fluctuation and fistulization.7
The cutaneous-ganglionic and hepatosplenic involvement is classically demonstrated in the acutesubacute form, typical of young people and heavily immunossupressed patients.8,9 Bone alterations can be seeing as osteolytic lesions mainly of the clavicles, the ribs and the humerus with a tendency towards symmetry9. In this case, the sternum was affected and reports or citations of this fact were not found in other patients. Ocular involvement is rare, affecting mainly eyelids and conjunctiva.2 Gastrointestinal and genitourinary tracts, nervous system and suprarenals can be occasionally affected specially in severe cases7,9, as our patient, who presented nephritic proteinuria, that was the reason for his hospitalization
The association of PCM and AIDS is varied, being predominant the acute-subacute form, expressing itself by cervical or generalized ganglia infarction, hepatosplenomegaly, frequent atypical skin lesions, bone lesions, tendency towards systemic dissemination and, occasionally, association with TB.11 So, it is recommended to exclude retrovirus in disseminated multifocal cases besides immunosuppressive factors such as the use of illicit drugs which were investigated in this case. The effective drugs against PCM comprise three groups: amphotericin B, sulfadiazine, and other sulfonamides compounds and azoles with systemic action.2,7,9 Classic amphotericin B a is drug of choice in the serious or multifocal cases.8 Its choice was made possible by hospital apparatus, which allowed quick clinical remission of the serious and lagged condition of this case.
PCM in Brazil should be considered mainly in men exposed to occupational or non-occupational rural activities or from endemic areas. The approach should be individualized and multidisciplinary since both diagnosis and the treatment of disseminated cases are a challenge similarly to this case that was diagnosed with a delay of more than one year.
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7. Ramos-e-Silva M. Micoses Profundas. Dermatologia Atual. 2000;6:6-13. [ Links ]
8. Marques SA, Camargo RMP, Marques MEA. Caso para diagnóstico. Paracoccidioidomicose. An Bras Dermatol. 2007;82:579-81. [ Links ]
9. Sampaio SAP, Rivitti EA. Micoses Profundas. In: Sampaio SAP, Rivitti EA, editores. Dermatologia. 3 ed. São Paulo: Artes Médicas; 2007. p.723-33. [ Links ]
10. Achenbach R, Negroni R, Khaski S, Lococo L, Beresñak A, Gai L. Paracoccidioidomycosis: unusual clinical presentation and utility of computerized tomography scanning for diagnosis. Int J Dermatol. 2002:41:881-2. [ Links ]
11. Valle ACF, Wanke B, Wanke NCF, Lima NS, Perez M. Tratamento da Paracoccidioidomicose: Estudo retrospectivo de 500 Casos - II. Avaliação dos resultados terapêuticos com sulfamídicos, anfotericina B, associação sulfametoxazol/trimetoprim, cetoconazol e miconazol. An Bras Dermatol. 1993;68:65-70. [ Links ]
Mailing address: Received on 16.09.2009. * Work carried out at the Federal University of Amazonas (UFAM) - Manaus (AM) - Brazil.
Livia Lima de Lima
Rua A29 Conjunto Ajuricaba - 293, Planalto
69046310 Manaus - AM, Brazil
Mobile number: 92 9985-5252
Approved by the Advisory Board and accepted for publication on 07.12.2009.
Conflict of interest: None
Financial funding: None
Received on 16.09.2009.
* Work carried out at the Federal University of Amazonas (UFAM) - Manaus (AM) - Brazil.