Bilateral Breast Ulcers: Granulomatosis with Polyangiitis

Pierre-André Jarrot (1, 2) , Marie-Laure Pelletier (3) , Marion Brun (2) , Martin Penicaud (2) , Karin Mazodier (2) , Audrey Benyamine (1, 4) , Jean Del Grande (5) , Rodolphe Jean (2) , Dominique Casanova (2) , Gilles Kaplanski (1, 2) , Pierre-Jean Weiller (6)

1 C2VN - Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research
2 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
3 La Casamance
4 Hôpital Nord [CHU - APHM]
5 TIMONE - Hôpital de la Timone [CHU - APHM]
6 IPC - Institut Paoli-Calmettes

PRESENTATION An investigation into the patient's unusual dermatologic symptoms identified an underlying autoimmune disease. Fourteen days after breast-reduction surgery, the patient, a 25-year-old white woman, was completely recovered. But at 20 days after surgery, she had bilateral mammary ulcera-tive lesions and was admitted to another hospital. The lesions were painless, and she was afebrile. Negative pressure wound therapy failed to accomplish secondary intention healing. For 6 weeks, she was treated with oral prednisolone, 1 mg/kg/day, and hyperbaric oxygen therapy, but these had limited effect. She then underwent a biopsy of the right breast. The specimen showed perivascular inflammation of the superficial and deep dermis, as denoted by an infiltrate of numerous neutrophils, eosinophils, lymphocytes, and some giant cells. Areas of nonfibrinoid necrosis were evident in the superficial dermis. Bacterial cultures of the wound and blood were negative. A diagnosis of pyoderma gangreno-sum was suspected based on the necrotic ulceration and characteristic violaceous undermined borders of the patient's lesions, the high proportion of neutrophils in the biopsy specimen, and the appearance of lesions on the scar site after a complete recovery. Yet, though the biopsy findings suggested pyoderma gangrenosum, there was some atypia, such as the absence of a central necrotic zone. Treatment with prednisolone and hyperbaric oxygen was restarted 5 days after biopsy results returned. She was then referred to our internal medicine department for further work-up. ASSESSMENT On admission, the patient was afebrile. She had bilateral, painless , inframammary ulcerations with inflammatory borders (Figure 1A). These measured 5 cm in diameter and ran horizontally across the surgical scar line. She also had pain in the sinus region. She reported a 1-year history of rhinitis with crusting and bleeding in both nostrils. Treatment had consisted of several courses of corticosteroids and antibiotics, which offered transient relief from symptoms, and ultimately, she underwent nasal septum surgery. Interestingly, the patient's ear, nose, and throat symptoms improved while she was being treated with prednisolone for her mammary lesions. Laboratory tests revealed elevations in the plasma fibrin-ogen level (5.4 g/L) and the C-reactive protein levels Figure 1 On presentation, the patient had bilateral ulcera-tive mammary lesions.

Domaines: Sciences du Vivant [q-bio]

Pierre-André Jarrot, Marie-Laure Pelletier, Marion Brun, Martin Penicaud, Karin Mazodier, et al.. Bilateral Breast Ulcers: Granulomatosis with Polyangiitis. The American Journal of Medicine, 2019, 132 (2), pp.179-181. ⟨10.1016/j.amjmed.2018.09.008⟩. ⟨hal-02591551⟩