Disseminated Lobomycosis in an 86 Year-Old Brazilian Department of Pathology, Institute of Tropical Woman with a 55-Year History of Disseminated Cutaneous Lesions

TropicalBlackwellOxford,IJDInternational0011-9059var_onlineissn©XXX 2008 TheUK Publishing International Journal Ltdof Dermatology Society of Dermatology medicine rounds DisseminatedATalhariCASE 55-year REPORT et al.history of disseminated skin lesions lobomycosis Carolina Talhari, Camila Bandeira Oliveira, Mônica Nunes de Souza Santos, Luis Carlos Ferreira, and Sinésio Talhari

From the Department of Dermatology, Abstract Institute of Tropical Medicine of Amazonas, Lobomycosis, also known as Jorge Lobo’s disease, is a granulomatous, chronic fungal disease Brazil, Department of Dermatology, Faculty of that involves the skin and subcutaneous tissue. The disease has been reported in South Medicine, State University of Amazonas, American, Central and North American countries, being particularly frequent in the Brazilian Brazil, Department of Dermatology, Faculty of Medicine, Nilton Lins University, Brazil, and Amazon region. We report a case of disseminated lobomycosis in an 86 year-old Brazilian Department of Pathology, Institute of Tropical woman with a 55-year history of disseminated cutaneous lesions. Medicine of the Amazonas, Brazil

Correspondence Carolina Talhari, MD Department of Dermatology Institute of Tropical Medicine of the Amazonas State Brazil E-mail: [email protected]

Case Report

An 86-year-old woman presented a 55-year history of cutaneous lesions distributed on the face and right lower and left upper limbs. She came from the state of Acre, located in the Brazilian Amazon region, where she worked as a rubber collector for many decades. Physical examination revealed multiple keloid-like nodules and papules with smooth surfaces located on the nose and extremities (Fig. 1). There were no palpable lymph nodes. A lesional skin biopsy specimen showed an atrophic epidermis. In the dermis, multiple granulomas were seen. The granulomas consisted of lymphocytes, histiocytes, and giant cells containing numerous oval fungal structures (Fig. 2a). The Grocott Methanamine Silver (GMS) stain revealed numerous round, isolated, and chained yeast cells consistent with Lacazia loboi (Fig. 2b). Based on clinical and histopathological findings, a diagnosis of lobomycosis was made. The patient is currently undergoing treatment with itraconazole, 200 mg daily.

Discussion

Lobomycosis is a chronic, granulomatous fungal disease that affects the skin and subcutaneous tissues.1 The etiological agent, L. loboi,2 is an extremely slow growing fungus with a prolonged incubation period, which explains the chronic Figure 1 Keloid-like nodules and papules on the: (a) nose 582 evolution of the disease. It has not yet been cultivated in vitro (b) right lower limb

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after a prolonged period of evolution.1,3–5 Lesions can appear as isolated or aggregated, multiple forms, particularly in the exposed, cooler areas of the body such as upper and lower limbs and ears. The disease may be restricted to one anatomic area, or disseminated, when several different sites are involved.1,3 Mucous membranes are not affected, and there is only one reported case of testicle involvement.3 Lymph node involvement has been observed.4 Transformation of chronic lobomycosis lesions into squamous cell carcinoma has been also reported.6 Despite the prolonged evolution of the disease and disseminated lesions presented by our patient, there were no lymph node involvement or malignancy. Leprosy, anergic cutaneous leishmaniasis, chromoblasto- mycosis, paracoccidioidomycosis, Kaposi’s sarcoma, keloids, fibroma, neurofibromas, dermatofibrosarcoma protuberans, and metastatic lesions should be included in the differential diagnosis of lobomycosis.1,3,5 Diagnosis is established by direct visualization of the lobo- myces which are seen as yeast-like rounded thick-walled cells occurring in chains of 2–10 cells.3 Histopathological examination is pathognomonic. The epidermis is usually atrophic and the dermis is occupied by a fibrous, diffuse, inflammatory granuloma composed of histiocytes, and giant cells containing the typical thick-walled cells. Periodic acid-Schiff, GMS or Gridley’s silver stain clearly distinguish the yeast-like cells.1,3,4 There is no treatment for lobomycosis that is completely effective. Localized lesions are treated with cryosurgery or wide surgical excision ensuring that margins are free of infection to avoid recurrence.1,3 Disseminated lesions are better treated with chemotherapy, such as clofazimine, itraconazole or a Figure 2 (a) Lesional skin biopsy specimen showing multiple combination of both drugs.1,3,5,7 giant cells containing numerous oval fungal structures (H&E, original magnification, ×100). (b) Grocott Methanamine Silver stain demonstrating numerous round, isolated, and chained yeast cells consistent with Lacazia loboi (original magnification, ×100) References 1 Paniz-Mondolfi AE, Reyes Jaimes O, Dávila Jones L. Lobomycosis in Venezuela. Int J Dermatol 2007; 46: 180–185. and is known to cause disease in humans, marine, and 2 Taborda PR, Taborda VA, McGinnis MR. Lacazia loboi 1,3,4 marine–freshwater dolphins. general nov., comb. nov., the etiologic agent of lobomycosis. The disease is endemic in the Brazilian Amazon and other J Clin Microbiol 1999; 37: 2031–2033. tropical zone countries in South and Central America.1,5 3 Talhari S, Pradinaud R. Topley & Wilson’s microbiology and Human cases have been reported in the United States and microbial infections. Medical Mycology. Washington, DC: Europe, in patients with an epidemiologic history of Edward Arnold Ltd, 2005: 430–435. travel to endemic countries, or in those that had contact with 4 Opromolla DV, Belone AF, Taborda PR, et al. Lymph node dolphins.1,4 The disease was first identified in 1931 by Jorge involvement in Jorge Lobo’s disease. Report of two cases: Lobo. Due to the presence of microorganisms resembling tropical medicine. Int J Dermatol 2003; 42: 938–941. 5 Talhari S, Souza MDG, Mendes AP, et al. Deep mycoses in Paracoccidioides brasiliensis, Lobo called the disease keloidal Amazon region. Int J Dermatol 1988; 27: 481–484. blastomycosis. After the second human case was identified 1 6 Baruzzi RG, Rodrigues DA, Michalany NS, et al. Squamous- seven years later, the disease was named Jorge Lobo’s disease. cell carcinoma and lobomycosis (Jorge Lobo’s disease). The lesions generally appear after a traumatic event, such Int J Dermatol 1989; 28: 183–185. as cuts obtained while working in agriculture or after insect 7 Fisher M, Chrusciak Talhari A, Reinel D, et al. Successful and animal bites. They are characterized as cutaneous treatment with clofazimine and itraconazole in a 46 years old nodules, papules or plaques of various sizes that can have smooth, patient after 32 years duration of disease. Der Hautarzt 2002; verrucous or ulcerated surfaces, and can become infiltrated 53: 677–681.