BACKGROUND: Paracoccidioidomycosis (PCM) is an endemic systemic mycosis in Latin America, classically characterized by pulmonary, mucosal and lymphatic involvement. Extrapulmonary manifestations affecting uncommon organs remain poorly characterized and may contribute to diagnostic delay and adverse outcomes. METHODS: We conducted a retrospective case series of proven or probable atypical extrapulmonary PCM diagnosed at a Brazilian tertiary referral center between 2006 and 2025. A systematic review registered in PROSPERO and conducted according to PRISMA recommendations identified osteoarticular, bone marrow and urogenital PCM cases between 1970 to November 2025 in PubMed/MEDLINE and Embase without language restrictions. RESULTS: Among 205 PCM patients evaluated at our institution, 14 (6.8%) presented atypical extrapulmonary involvement. Combined with 153 cases identified in 75 studies, 167 cases were analysed. Osteoarticular involvement was the most frequent manifestation (53.9%), predominantly affecting young patients with acute/subacute disease and presenting mainly as osteomyelitis with lytic lesions. Bone marrow involvement occurred exclusively in acute/subacute PCM and was associated with post-mortem diagnosis (9.7%) and markedly elevated mortality (77.8%). Urogenital PCM mainly affected middle-aged men with chronic disease, frequently involving the testicles and prostate, and was associated with delayed diagnosis, surgical intervention (32.7%), and mortality (32.0%). Distinct epidemiological profiles, clinical forms and outcomes were consistently observed across atypical sites. CONCLUSION: Atypical extrapulmonary PCM manifestations follow recognizable clinical patterns associated with diagnostic delay and poor outcomes, supporting earlier investigation and timely antifungal therapy in endemic settings.
Kruschewsky et al. (Tue,) studied this question.