Coincidence of cheilitis granulomatosa and chronic spontaneous angioedema: successful treatment with dapsone and omalizumab

Gronostay, Alexandra; Hellmich, Luisa; Rasokat, H.; Hunzelmann, Nicolas

doi:10.1111/ddg.14104

Cited by 2 publications

(2 citation statements)

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“…The local reduction in IL‐2 in OFG lesions could be responsible for the down‐regulation of IL‐2 receptors on lymphocytes and the suppression of the inflammatory process 45 . Quite recently, the case of a patient with GCM coinciding with CSU‐related recurrent angioedema was reported, successfully treated with dapsone and omalizumab 46 . These two were the only reports describing the use of omalizumab in OFG.…”

Section: Discussionmentioning

confidence: 99%

“…45 Quite recently, the case of a patient with GCM coinciding with CSU-related recurrent angioedema was reported, successfully treated with dapsone and omalizumab. 46 These two were the only reports describing the use of omalizumab in OFG. Further studies on a large scale are needed to confirm this therapeutic effect and to better understand the mechanisms of action of anti-IgE monoclonal antibody in OFG.…”

Section: Trigeminal Neuralgiamentioning

confidence: 99%

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Orofacial granulomatosis: Clinical and therapeutic features in an Italian cohort and review of the literature

Galdiero

Maio

Arcoleo

et al. 2021

Allergy

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Background Orofacial granulomatosis (OFG) is characterized by granulomatous inflammation of the soft tissues of maxillofacial region. We explored OFG patients from 10 different Italian centers and summarized the most recent literature data. Methods A review of patients with OFG was carried out. An extensive online literature search was performed to identify studies reporting diagnosis and management of OFG. Results Thirty‐nine patients were recruited between January 2018 and February 2020. Most of them (97.4%) displayed involvement of the lips, and 28.2% suffered from Melkersson‐Rosenthal syndrome. Two patients received diagnosis of CD and one patient of sarcoidosis, suggesting secondary OFG. Oral aphthosis and cervical lymphadenopathy were also described. The mean diagnostic delay was 3.4 years. Histological evaluation was performed in 34/39 patients (87.2%); non‐caseating granulomas were found in 73.5% of them. Neurological symptoms (28.2%), gastrointestinal symptoms in absence of overt inflammatory bowel disease (IBD) (20.5%), and atopy (35.9%) were also identified. Therapeutic approaches varied among the centers. Steroids (51.3%) were used with good or partial results. Anti‐TNF‐α and anti‐IgE monoclonal antibodies were used in 6 (15.4%) and 1 (2.6%) patients, respectively, with variable results. Surgery was the choice for 2 patients with good response. Conclusions OFG is a rare and neglected disease showing multiple clinical phenotypes. While early diagnosis is crucial, management is difficult and highly dependent on the expertise of clinicians due to the lack of international guidelines. There is a need to establish registry databases and address challenges of long‐term management.

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Section: Discussionmentioning

confidence: 99%

Section: Trigeminal Neuralgiamentioning

confidence: 99%