Cheilitis granulomatosa G51.2

Authors: Prof. Dr. med. Peter Altmeyer, Dr. med. Lucian Cajacob

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Last updated on: 28.04.2021

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Synonym(s)

Granulomatosis orofacial; OFG; orofacial granulomatosis

History
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Miescher, 1945

Definition
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Cosmetically disfiguring, initially chronic recurrent, then permanently persistent, granulomatous inflammation of the lip, which can occur in isolation (idiopathic) or as a cutaneous partial manifestation of a granulomatous systemic disease. These include:

In children, especially in a constellation of oral aphthae and Cheilitis granulomatosa, a high percentage of Crohn's disease can be expected.

In individual cases, type I food allergies have also been blamed.

Manifestation
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Manifestation peak: 20-40 years; m:w=1:1;

Clinical features
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Initially changing, later permanent, diffuse, inflammatory swelling of the lips, especially the upper lip and the surrounding skin with bulging, sometimes trunk-shaped protrusion of the lips (tapir mouth) and eraser-hard consistency proliferation.

  • Associated lingua plicata, facial nerve palsy, or hyperplastic gingivitis are suggestive of Melkersson-Rosenthal syndrome.
  • If the cheilitic changes occur in combination with persistent (especially large and deep) aphthae, Crohn's disease must be excluded (therapeutic consequences- see below Cushing K et al.2021).
  • Ulcerative colitis may also be associated with these symptoms.

Histology
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In the early oedematous inflammatory phase, dilated lymphatic and blood vessels are found with spongy, perivascular, lymphocytic infiltrates. In a later stadium, small epithelial cell granulomas with giant cells may appear in addition to a distinct fibrosis of the dermis. In the case of association with ulcerative colitis or Crohn's disease, dense mixed-cell inflammatory infiltrates with shot-shot-like distributed, non-keratinizing granulomas with epithelial and giant cells are found. Remark: Epitheloid cell granulomas must be searched for in serial sections. Often they are missing completely.

Differential diagnosis
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Clinical:

  • Angioedema: only of short duration, swellings regress completely in the meantime even in recurrent course.
  • Herpes simplex recidivans: development of typical herpes vesicles. In close recurrences, firm swellings may persist for a long time.
  • Sarcoidosis: localization is unusual.
  • Foreign body granulomas after injection of filler substances: important differential diagnosis; injections may be longstanding.

Histologic:

  • Sarcoidosis: dense collection of "naked" noncaseating granulomas.
  • Foreign body granuloma: epithelioid cell granulomas with numerous giant cells of the foreign body type.
  • Infections (mycobacteriosis, leishmaniasis): mixed cell granulomatous infiltrates.

Therapy
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In Melkersson-Rosenthal syndrome: see there. Otherwise, if necessary, treatment of the underlying disease, e.g. sarcoidosis, Crohn's disease.

In monosymptomatic cheilitis granulomatosa, intralesional triamcinolone acetonide injections (e.g. Volon A diluted with Scandicaine in a ratio of 1:3) at 1- or 2-week intervals over a few weeks (depending on the clinic) are recommended as first-line therapy.

Partial success is possible with early use of clofazimine (e.g. Lampren) 100 mg/day p.o. for 6 months (longer if necessary) and a repeat cycle after a 3-month break.

Alternative: Positive casuistic experiences exist about the long-term (>6 months) high-dose use of fumaric acid esters .(Hauck G 2017). Dosage analogous to the schematic procedure for psoriasis therapy.

Alternative: In case of therapy resistance of the dermatological symptoms and in case of (negligible gastroenterological symptoms) a therapy with a TNF-alpha antagonist is indicated.

Alternative: In case of therapy resistance of the dermatological symptoms, the use of vedolizumab is recommended (approved for Crohn's disease). Isolated successes have also been described in the absence of success of a TNFα inhibitor (Gueutier A et al. 2019).

Alternative: In case of persistent swelling resistant to therapy, surgical reduction plasty to be performed enorally is recommended.

In case of association with regional enteritis (Crohn's disease), the underlying disease should be treated in collaboration with a gastroenterologist.

Note(s)
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When cheilitis granulomatosa occurs in childhood, a frequent association with Crohn's disease is found in combination with oral aphthae. This clinical picture is called orofacial granulomatosis in pediatric and international literature. This term also seems to be used for cheilitis granulomatosa occurring in adults.

Literature
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  1. Al-Hamad A et al (2015) Orofacial granulomatosis. Dermatol Clin 33:433-446
  2. Allen CM et al (1990) Cheilitis granulomatosa: report of six cases and review of the literature. J Am Acad Dermatol 23: 444-450
  3. Ciacci C et al (2015) Buccal localization of Crohn's disease with long-term infliximab therapy: a case report. J Med Case Rep 30:397
  4. Critchlow WA et al (2014) Cheilitis granulomatosa: a review. Head Neck Pathol 8: 209-213
  5. Cushing K et al.(2021) Management of Crohn's disease: a review. JAMA 325:69-80.
  6. Gueutier A et al. (2019) Efficacy of vedolizumab in granulomatous cheilitis refractory to TNFα inhibitors. Int J Dermatol 58:e236-e237.
  7. Hauck G (2017) Fumaric acid ester therapy bie Melkersson-Rosenthal syndrome. Akt Dermatol 43: 459-460
  8. Kano Yet (1992) Treatment of recalcitrant cheilitis granulomatosa with metronidazole. J Am Acad Dermatol 27: 629-630.
  9. Kleine R et al (2011) Fumaric acid ester therapy in a young patient with marked cheilitis granulomatosa. Dermatol 62: 940-942
  10. Rana AP (2012) Orofacial granulomatosis: A case report with review of literature. J
  11. Indian Soc Periodontol 16:469-474
  12. Rehor GE et al (2010) Cheilitis granulomatosa as an initial manifestation of Crohn's disease. Dermatologist 61: 691-693
  13. Saalmann R et al. (2009) Orofacial granulomatosis in childhood - a clinical entity that may indicate Crohn`s disease as well as food allergy. Acta Paediatr 98: 1162-1167
  14. Sakuntabhai A et al (1993) Intralesional steroid injection after nerve block anaesthesia in the treatment of orofacial granulomatosis. Arch Dermatol 129: 477-480
  15. Schuhmacher F et al (1991) Cheilitis et pareitis granulomatosa: spontaneous course. Dermatologist 42: 516-517
  16. Tuxen AJ et al (2010) Childhood and adolescent orofacial granulomatosis is strongly associated with Crohn's disease and responds to intralesional corticosteroids. Australas J Dermatol 51:124-127
  17. Vibhute NA et al (2013) Cheilitis granulomatosa: a case report with review of literature. Indian J Dermatol 58:242

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Last updated on: 28.04.2021