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.

Lupus vorax: A rare case of facial tuberculosis in a patient with Noonan syndrome / F. La Monaca, S. Buffon, G. Genovese, G.A. Croci, C.A. Maronese, F. Derlino, A.V. Marzano, L. Sarno. - In: IDCASES. - ISSN 2214-2509. - 42:(2025 Nov), pp. e02409.1-e02409.3. [10.1016/j.idcr.2025.e02409]

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

F. La Monaca
Primo
;
S. Buffon
Secondo
;
G. Genovese
;
G.A. Croci;C.A. Maronese;A.V. Marzano
Penultimo
;
2025

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.
tuberculosis; lupus vorax; pyoderma gangrenosum
Settore MEDS-10/C - Malattie cutanee e veneree
nov-2025
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/1214937
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