Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Cheilitis Granulomatosa

  • Jacqueline E. van der WalEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_667-1

Synonyms

Definition

Cheilitis granulomatosa, first described by Miescher in 1945, is together with Melkersson–Rosenthal syndrome subsets of orofacial granulomatosis. Clinically, patients present with a nontender, persistent swelling of one or both lips. The labial swelling may be associated with orofacial swelling affecting the chin, eyelids, cheeks, gingiva, and oral mucosa.

Clinical Features

Incidence

It is estimated that the incidence is 0.08% in the population.

Age

Mostly adults, although it can occur in childhood. In children and adults under 30 years of age, an association with an asymptomatic, non-Crohn’s disease, gastrointestinal inflammatory process, or food allergy has been shown.

Sex

Cheilitis granulomatosa has a female predilection.

Site

Upper and lower lip.

Treatment

First, try to find the underlying cause by thorough clinical examination. Individual lesions have been treated with several different interventions as topical or intralesional corticosteroids, triamcinolone, radiotherapy, sulfasalazine, hydroxychloroquine sulfate, azathioprine, cyclosporine A, methotrexate, antibiotics, and others. Spontaneous remissions have been published as well.

Outcome

The prognosis is highly variable. It is a recurrent disease and may be episodic and intermittent. No therapy has been proven to be of benefit in every patient.

Microscopy

Microscopically, noncaseating granulomas are present in the connective tissue. The granulomas consist of epithelioid histiocytes and lymphocytes, with or without multinucleated giant cells. The superficial lamina propria shows edema with dilation of small lymphatic vessels and scattered lymphocytes. The granulomas seem to cluster around vessels. No foreign material, fungal organisms, or acid-fast bacteria are present (Figs. 1 and 2).
Fig. 1

Low power H&E stain with multiple noncaseating granulomas in the deep subepithelial connective tissue

Fig. 2

Detail of Fig. 1

Differential Diagnosis

Melkersson–Rosenthal syndrome, with fissured tongue and facial paralysis.

Allergic reaction to several agents.

References and Further Reading

  1. Critchlow, W. A., & Chang, D. (2014). Cheilitis granulomatosa: A review. Head and Neck Pathology, 8, 209–213.CrossRefPubMedGoogle Scholar
  2. El-Hakim, M., & Chauvin, P. (2004). Orofacial granulomatosis presenting as persistent lip swelling: Review of 6 new cases. Journal of Oral and Maxillofacial Surgery, 62, 1114–1117.CrossRefPubMedGoogle Scholar
  3. Kaminagakura, E., & Jorge, J., Jr. (2011). Melkersson–Rosenthal syndrome: A histopathologic mystery and dermatologic challenge. Journal of Cutaneous Pathology, 38, 241–245.CrossRefPubMedGoogle Scholar
  4. van der Waal, R. I. F., Schulten, E. A. J. M., van der Scheur, M. R., Wauters, I. M. P. M. J., Starink, T. M., & van der Waal, I. (2001). Cheilitis granulomatosa. Journal of the European Academy of Dermatology and Venereology, 15, 519–523.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of PathologyThe Netherlands Cancer Institute, Antoni van Leeuwenhoek HospitalAmsterdamThe Netherlands