QJM Advance Access published January 21, 2015 BIG LIPS. M. Reveillon, V. Eble, H. Levesque, I. Marie

Transcription

1 QJM Advance Access published January 21, 2015 BIG LIPS M. Reveillon, V. Eble, H. Levesque, I. Marie Mathilde Reveillon, MD; Vincent Eble, MD; Hervé Levesque MD, PhD; Isabelle Marie, MD, PhD. Department of Internal medicine, CHU Rouen, Rouen, France. Correspondence to: Professeur Isabelle Marie, Département de Médecine Interne, CHU de Rouen, Rouen Cedex. : ; Fax : ; isabelle.marie@chu-rouen.fr Key words: granulomatous cheilitis sarcoidosis - granulomatosis with polyangiitis Crohn's disease - tuberculosis - angioedema related to angiotensin-converting enzyme inhibitor and calcium channel blocker therapy - hereditary angioedema. The authors confirm that they do not have conflict interest. The Author Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please journals.permissions@oup.com

2 A 31-year-old man, with unremarkable previous medical history, presented with a 3- year history of painless swelling involving his lips. The swelling of his lips was initially episodic and recurrent, both frequency and duration of attacks increasing during this period of time, and being persistent since 9 months. On admission, physical examination showed swollen lips (Figure 1A) and gingival hypertrophy (Figure 1B); general physical examination was otherwise normal. Laboratory studies disclosed the following: erythrocyte sedimentation rate: 8 mm/hour, C-reactive protein: 3 mg/l; other routine biochemical tests, including renal and liver tests, blood protein electrophoresis and urinalysis, were normal. Other tests -that are autoantibody screening (rheumatoid factors, antinuclear antibodies, antineutrophil cytoplasmic antibodies, angiotensin converting enzyme level), complement profile and C1 esterase inhibitor, were negative; IgE level, prick tests and tuberculin skin test yielded negative results. Computed tomography of both lungs and abdomen were within normal limits. A biopsy of the lips was performed; histological analysis of lip biopsy specimens revealed non-caseating granulomas; Ziehl-Neelsen stains were negative. The diagnosis of granulomatous cheilitis was made. First described by Miescher, granulomatous cheilitis is an uncommon disorder, characterized by both chronic and potentially disfiguring swelling of the lip related to granulomatous inflammation; this condition belongs to the orofacial granulomatous group, including the Melkersson-Rosenthal syndrome associating swollen lips, plicated tongue and recurrent facial nerve palsy (1, 2). Granulomatous cheilitis has complex pathogenesis and likely multifactorial causes, notably genetic predisposition and environmental exposures (1, 2). Our case report emphasizes the importance of recognizing granulomatous cheilitis from the following disorders, which may also result in swelling of the lips, i.e.: 1) granulomatous diseases: infection (tuberculosis, syphilis, leprosy, histoplasmosis), sarcoidosis,

3 granulomatosis with polyangiitis, amyloidosis and Crohn's disease; 2) atopic reaction to allergens; 3) angioedema related to angiotensin-converting enzyme inhibitor and calcium channel blocker therapy; and 4) hereditary angioedema (1, 2). Indeed, accurate diagnosis of granulomatous cheilitis will lead to appropriate therapy, thereby avoiding unnecessary investigations.

4 References 1. Banks T, Gada S. A comprehensive review of current treatments for granulomatous cheilitis: current treatments for granulomatous cheilitis. Br J Dermatol 2012;166: Critchlow WA, Chang D. Cheilitis granulomatosa: a Review. Head Neck Pathol 2013;8:

5 Figure legend Figure 1. Swelling involving both the upper and lower lips (A) and gingival hypertrophy (B) related to granulomatous cheilitis.

6 Swelling involving both the upper and lower lips (A) and gingival hypertrophy (B) related to granulomatous cheilitis. 254x190mm (96 x 96 DPI)