Abstract Number: 107

Cutaneous granulomas arising in a patient with ataxia telangiectasia

N. Biswas, L. Wheller, M. Melo, T. Casey, J. Peake

Meeting: 2016 Dermcoll

Session Information

Date: -

Session Title: Poster Presentations

Session Time: -

Ataxia telangiectasia (AT) is an autosomal recessive pri- mary immunodeficiency disorder characterised by progres-sive cerebellar ataxia and telangiectasia. Rarely, it has been associated with cutaneous granulomas, with only 18 such cases reported to date.1 The diagnosis of cutaneous granulomas can be challenging in the context of AT, likely due to the wide clinical heterogeneity, numerous possible differential diagnoses and limited literature. We report a case of predominantly nasal cutaneous granulomas in a 16-year-old female with AT treated with potent topical cor- ticosteroid, infliximab and intravenous immunoglobulin. This regimen has shown progressive improvement in the skin lesions. This case highlights the importance of consid- ering cutaneous granuloma in patients with primary immunodeficiency presenting with refractory and unusual skin lesions.
Harp J, Cogshall K, Ruben BS et al. Cutaneous granulomas the setting of primary immunodeficiency: a report of four cases and review of the literature. Int J Dermatol. 2015 Jun; 54(6): 617–25.