Lupus vorax: A rare case of facial tuberculosis in a patient with Noonan syndrome

Lupus vorax is a rare, aggressive ulcerative variant of lupus vulgaris, the most common form of cutaneous tuberculosis, characterized by rapid tissue destruction, extensive scarring, and potential disfigurement. We report the case of a 30-year-old man with Noonan syndrome who presented with chronic facial ulcerations initially suspected to represent pyoderma gangrenosum (PG). The lesion, involving the upper lip, nasal alae, and left eyelid region, had been present for two years with progressive worsening. Initial histology suggested PG or granulomatosis with polyangiitis; however, PCR and culture from a subsequent biopsy confirmed a multisensitive Mycobacterium tuberculosis strain. Quadruple anti-tubercular therapy led to complete lesion resolution within nine months, leaving residual scarring and functional sequelae. This case highlights the diagnostic challenge of differentiating PG from infectious mimickers, the need for multiple diagnostic modalities in atypical presentations, and the importance of considering rare etiologies, even in patients with genetic disorders potentially associated with immune dysregulation.

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Publication Details

Journal
IDCases
Published
2025-01-01
DOI
https://doi.org/10.1016/j.idcr.2025.e02409
Citations
1
Primary Topic
Infectious Diseases and Tuberculosis
Type
article
Field-Weighted Citation Impact
2.01
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article

Lupus vorax: A rare case of facial tuberculosis in a patient with Noonan syndrome

Carlo Alberto Maronese, Stefano Buffon, Federica Derlino, Giovanni Genovese et al.
1 citations
IDCases
Infectious Diseases and Tuberculosis
2.01
article

Lupus vorax: A rare case of facial tuberculosis in a patient with Noonan syndrome

Carlo Alberto Maronese, Stefano Buffon, Federica Derlino, Giovanni Genovese, Lorenzo Di Sarno, Francesco La Monaca
article en
1 citations

Abstract

Lupus vorax is a rare, aggressive ulcerative variant of lupus vulgaris, the most common form of cutaneous tuberculosis, characterized by rapid tissue destruction, extensive scarring, and potential disfigurement. We report the case of a 30-year-old man with Noonan syndrome who presented with chronic facial ulcerations initially suspected to represent pyoderma gangrenosum (PG). The lesion, involving the upper lip, nasal alae, and left eyelid region, had been present for two years with progressive worsening. Initial histology suggested PG or granulomatosis with polyangiitis; however, PCR and culture from a subsequent biopsy confirmed a multisensitive Mycobacterium tuberculosis strain. Quadruple anti-tubercular therapy led to complete lesion resolution within nine months, leaving residual scarring and functional sequelae. This case highlights the diagnostic challenge of differentiating PG from infectious mimickers, the need for multiple diagnostic modalities in atypical presentations, and the importance of considering rare etiologies, even in patients with genetic disorders potentially associated with immune dysregulation.

IDCasesVol. 42
University of Milan (IT), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (IT)
Openalex Percentile: Top 11%
Infectious Diseases and Tuberculosis
2.01
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Lupus vorax: A rare case of facial tuberculosis in a patient with Noonan syndrome — Carlo Alberto Maronese, Stefano Buffon, et al. · IDCases (2025) | TGRS Research Map | TGRS