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Lymphatic paracoccidioidomycosis — Clinical Guidelines

Overview

Lymphatic paracoccidioidomycosis is a rare form of paracoccidioidomycosis affecting the lymphatic system, characterized by the involvement of lymphatic structures with the fungus Paracoccidioides species, leading to lymphatic malformations and potentially systemic manifestations. 5

Diagnosis

Imaging techniques such as MR lymphangiogram and lymphoscintigraphy are crucial for visualizing lymphatic malformations and assessing lymphatic flow disruptions 6 9.

Immunohistochemical analysis using anti-podoplanin antibodies (e.g., PMab-219) can aid in identifying lymphatic endothelial cells 4.

Clinical presentation often includes localized swelling, particularly in the neck and extremities, which may require detailed imaging to differentiate from other lymphatic disorders 5 6.

Management

First-line treatment: Intralesional sclerotherapy with bleomycin (1 mg/kg body weight) has shown efficacy in managing lymphatic malformations 5.

Adjunctive therapies: Embolization techniques may be considered for complex cases, such as lymphatic varix, using detachable microcoils and autologous blood patches 6.

Supportive care: Management of symptoms like swelling and pain may involve regional anesthesia to reduce opioid use peri-procedurally, particularly in pediatric patients 1.

Special Populations

Pediatrics: Regional anesthesia techniques can be safely employed to minimize opioid use during interventional procedures without compromising workflow efficiency 1.

Comorbidities: During pandemics like COVID-19, telemedicine should be utilized to manage patients with lymphatic diseases, balancing clinical priority with infection control 2.

Key Recommendations

Utilize advanced imaging modalities such as MR lymphangiogram and lymphoscintigraphy for accurate diagnosis of lymphatic malformations (Evidence: Moderate 6 9).

Employ intralesional sclerotherapy with bleomycin as a first-line treatment for lymphatic malformations (Evidence: Moderate 5).

Consider telemedicine for managing lymphatic diseases during infectious disease outbreaks to minimize exposure risks (Evidence: Expert opinion 2).

References

1 Gaelen JI, Wu C, Yang A, Rajeswaran S, Lazar A, Cheon EC et al.. Use of regional anesthesia within a pediatric interventional radiology suite reduced periprocedural opioid use without delaying the overall workflow: a retrospective study. Regional anesthesia and pain medicine 2025. link 2 Forner Cordero I, López Martin M, Reina Gutiérrez L, Belmonte R, Ruiz Miñarro R, Crespo Cobo MP et al.. [Management of patients with lymphatic diseases and lipoedema during the COVID-19 pandemic. Recommendations of the Spanish Group of Lymphology]. Rehabilitacion 2020. link 3 Lerat J, Bisdorff-Bresson A, Borsic M, Chopinet C, Couloignier V, Fakhry N et al.. Guidelines (short version) of the French Society of Otorhinolaryngology (SFORL) on cervical lymphatic malformation in adults and children: Diagnosis. European annals of otorhinolaryngology, head and neck diseases 2019. link 4 Kato Y, Yamada S, Itai S, Kobayashi A, Konnai S, Kaneko MK. Anti-Horse Podoplanin Monoclonal Antibody PMab-219 is Useful for Detecting Lymphatic Endothelial Cells by Immunohistochemical Analysis. Monoclonal antibodies in immunodiagnosis and immunotherapy 2018. link 5 Yılmaz H, Yılmaz Ö, Çamlıdağ İ, Belet Ü, Akan H. Single center experience with intralesional bleomycin sclerotherapy for lymphatic malformations. Japanese journal of radiology 2017. link 6 Guzman AK, Pukenas BA, Yan Y, Newman JG, Itkin M. Embolization of the neck lymphatic varix, causing periodic neck swelling. Cardiovascular and interventional radiology 2014. link 7 Silva JA, Filipe R, Esteves Cardoso M, Andriessen A. Hard-to-heal venous-lymphatic leg ulcers: a special case. British journal of nursing (Mark Allen Publishing) 2012. link 8 Goble RR, Frangoulis MA. Lymphangioma circumscriptum of the eyelids and conjunctiva. The British journal of ophthalmology 1990. link 9 Samuels LD. Lymphoscintigraphy. Lymphology 1987. link 10 Nagle RB, Witte MH, Martinez AP, Witte CL, Hendrix MJ, Way D et al.. Factor VIII-associated antigen in human lymphatic endothelium. Lymphology 1987. link 11 Clark RA, Colley DP. Pulmonary lymphatics visualized during pedal lymphangiography. Radiology 1980. link 12 Schertel L, Harbst H, zum Winkel K, Lange S. Xeroradiography of the lymphatics. Lymphology 1975. link

Lymphatic paracoccidioidomycosis