Capurro NJ, Zelaya SP, Protti L, Pèrez MP, Ottino A, Manuel PH

Language: Spanish
References: 18
Page: 346-350
PDF size: 318.24 Kb.

ABSTRACT

Rosenthal-Melkersson syndrome (RMS) is a chronic neurocutaneous disorder of unknown etiology, clinically characterized by a triad of persistent labial edema, recurring facial palsy and fissured tongue. It frequently starts as mono or oligosymptomatic. Classic triad takes place in only 25% of cases. Granulomatous Meischer cheilitis (GMCh) is the most frequent monosymptomatic expression of RMS, which is histologically characterized by non-caseating granulomas. We report the case of a 50-year-old female, with protracted facial palsy history and facial edema, who attended to consultation due to lower lip swelling. The biopsy submitted to the Pathology Department was an irregular fragment measuring 1.2 cm. It was routinely processed and stained with hematoxilin-eosin, PAS, Ziehl Neelsen and Grocott. Histologically it showed dermal non-caseating granulomas plentiful of epithelioid cells, with sporadic giant Langhans cells. PAS, Ziehl-Neelsen and Grocott were negative. The diagnosis was “granulomatous cheilitis with histological features consistent with RMS”. The unlikelihood of its occurrence and the frequent unawareness about this entity explain its low prevalence. In case of manifestations suggestive of RMS, a biopsy should be performed. Although complete forms should not be source of diagnostic trouble, partial forms can cause difficulty, and should mainly be distinguished from sarcoidosis and Crohn’s disease.

REFERENCES

1. Barnhill R. Textbook of dermopathology. 2nd ed. New York: Mc Graw Hill, 2004;p:1000.

2. Cocuroccia B, Gubinelli E, Annessi G, Zambruno G, Girolomoni G. Persistent unilateral orbital and eyelid oedema as manifestation of Melkersson Rosenthal Syndrome. J Eur Acad Dermatol Venereol 2005;19:107-11.

3. Aliagaoglu C, Yildirim U, Albayrak H, Goksugur N, et al. Melkersson Rosenthal syndrome associated with ipsilateral facial, hand, and foot swelling. Dermatol Online J 2008;14:7.

4. Hoexter DL. Melkersson Rosenthal syndrome. N Y State Dent J 2007;73:30-32.

5. Gonçalves DU, de Castro MM, Galvão CP, Brandao AC, et al. Cheilitis granulomatosa associated with Melkersson-Rosenthal syndrome. Rev Bras Otorrinolaringol 2007;73:132-33.

6. Elder DE. Lever’s Histopathology of the skin. 10th ed. Philadelphia: Lippincott Williams and Wilkins, 2009;pp:380-1.

7. Opala G, Krzystanek E, Siuda J, et al. Melkersson-Rosenthal syndrome as a rare cause of recurrent facial nerve palsy. Neurol Neurochir Pol 2005;39:335-8.

8. Nagel F, Foelster-Holst R. Cheilitis granulomatosa Melkersson Rosenthal syndrome. Hautarzt 2006;5:121-6.

9. Ratzinger G, Seep N, Vogetseder W, Tilg H. Cheilitis granulomatosaand Melkersson Rosenthal syndrome: evaluation of gastrointestinal involvement and therapeutic regimens in a series of 14 patients. J Eur Acad Dermatol Venereol 2007;21:1065-70.

10. Ang KL, Jones NS. Melkersson Rosenthal syndrome. J Laryngol Otol 2002;116:386-8.

11. Camacho-Alonso F, Bermejo-Fenoll A, Pía-López J. Queiltis granulomatosa de Miescher. Presentación de cinco casos. Med Oral Patol Oral Cir Bucal 2004;9:425-9.

12. Tucto S. Síndrome de Melkersson Rosenthal. Reporte de un caso. Folia Dermatol Perú 2004;15:176-82.

13. McGrath DS, Doyle C, Bredin CP. The Melkersson Rosenthal syndrome a diagnosis of facial sarcoidosis. Ir J Med Sci 1997;166:253-6.

14. Ilnyckyj A, Aldor TA, Warrington R, Bernstein CN. Crohn’s disease and the Melkersson Rosenthal syndrome. Can Gastroenterol 1999;13:152-154.

15. Pérez-Calderón R, Gonzalo-Garijo MA, Chaves A, de Argila D. Cheilitis granulomatosa of Melkersson Rosenthal syndrome: treatment with intralesional corticosteroid injections. Allergol Immunopathol 2004;32:36-38.

16. Weedon D. Piel. Patología. 1ª ed. Madrid: Marbán, 2002;pp:176-8.

17. Van der Waal RI, Schulten EA, Van der Meij EH, van de Scheur MR, et al. Cheilitis granulomatosa: overview of 13 patients with long-term follow-up-results of management. Int J Dermatol 2002;41:225-9.

18. Vigliola P. Queilitis granulomatosas y síndrome de Melkersson Rosenthal. Act Terp Dermatol 2006;29:193-7.