Rodríguez CTM, Vega MME, Mosqueda TA, Rangel GEL

Language: Spanish
References: 24
Page: 87-92
PDF size: 291.35 Kb.

ABSTRACT

Background: Pemphigus vulgaris is a chronic autoimmmune bullous disease that affects mainly adults with an age average of 50 years and it is uncommon in young people.
Objetive: to present the clinical data and therapeutic management of patients younger than 30 years, affected by pemphigus vulgaris.
Material and Methods: a retrospective study of pemphigus vulgaris cases in patients younger than 30 years recorded in 25 year period (1980-2006) in the DermatologyDepartment at the “Dr. Manuel Gea González” General Hospital in Mexico City.
Results: from a total of 135 cases of PV diagnosed during this study, there were 17 (12.6%) diagnosed before the age of 30. In eight of them (47%) the onset of the disease was before 24 years of age. There were 13 female (76.5%) and 4 male (23.5%) ratio: 1:3.2. Correct diagnosis was done in the first eight months (mean average: four months). Skin and mucosae were affected in all cases, but oral involvement was the initial clinical manifestation in 11 cases (64.7%). In all the cases diagnosis was confirmed by histopathology. Treatment with prednisone 1–2 mg/kg/day (16/17 cases), deflazacort 90 kg/day (1/17 cases) and in (15/17) azathioprin 100 mg/day was added to prednisone as initial therapy. Tapering of steroids was done in a dose-response fashion. Just four patients are still under control without medication for periods of up to nine years without reactivation of the disease.
Conclusión: in our service PV is an uncommon disease. It occurs primarily in women and it is not rare in patients younger than 30 years of age, which represent 12.6% of all our cases and we have observed remissions for longer periods without treatment.

REFERENCES

1. Vega Memije ME, Villatoro Ugalde V y Mosqueda Taylor A, Pén figo vulgar. Informe del manejo de 40 casos. Dermatología Rev Mex 1998; 42 (6): 244-249.

2. Calebotta A, Cirocco A, Rodríguez MA y Sáenz AM, Oral Pemphigus vulgaris antedating cutaneous lesions, Derma Venez 2002; 40: 6-10.

3. Aldama A, Correa J, Rivelli V y Mendoza G, Tipos y variantes de pénfigo en el Hospital Nacional de Paraguay. Revisión de 70 casos, Med Cutan Iber Lat Am 2000; 28 (5): 242-247.

4. Micali G, Musumeci ML y Nasça MR, Epidemiologic analysis and clinical course of 84 consecutive cases of pemphigus in eastern Sicily, Int J of Dermatol 1998; 37: 197-200.

5. Firth N, Rich A, Varigos G y Reade PC, Oral pemphigus vulgaris in young adults, Int J of Dermatol 1991; 30 (5): 352-356.

6. Scully C y Challacombe SJ, Pemphigus vulgaris: update on etiopathogenesis, oral manifestations, and management, Crit Rev Oral Biol Med 2002; 13(5): 397-408.

7. Amrinder J, Kanwar y Surrinder Kaur, Pemphigus in children, Int J of Dermatol 1991; 30: 343-346.

8. Brenner S, Tur E, Shapiro J, Ruocco V, D’Avino M, Ruocco E, Tsankov N, Vassileva S, Drenovska K, Brezoev PM, Branadas A, González MJ, Grant Anhalt N, Ramos H, Silva M, Teixeira Pinto K y Miranda MF, Pemphigus vulgaris: environmental factors. Occu pational, behavioral, medical, and qualitative food frequency questionnaire, Int J of Dermatol 2001; 40: 562-569.

9. Olivares LM, Pizzariello GEA, Starck F, Jaled M y Restito EJ, Estudio retrospectivo de pacientes con pénfigo, en un periodo de diez años, Dermatol Argent 1998; 1: 27-32.

10. 10.Weston WL, Morelli JG y Huff JK, Misdiagnosis, treatments, an outcomes in the immunobullous diseases in children, Pediatric Dermatol 1997; 14 (4): 264-272.

11. Herbst A y Bystryn J, Pattern of remission in pemphigus vulgaris, J Am Acad Dermatol 2000; 42: 422-427.

12. Bezold G, Sander CA, Flaig MJ, Peter RU y Messer G, Lack of detect - ion of human herpesvirus (HHV)-8 DNA in lesional skin of Ger man pemphigus vulgaris and pemphigus foliaceus patients, J Invest Dermatol 2000; 114: 739-741.

13. Loannides D, Chrysomallis F y Bystryn JC, Ineffectiveness of cyclosporine as an adjuvant to corticosteroids in the treatment of pemphigus, Arch Dermatol 2000; 136: 868-872.

14. Camacho Alonso F, López Jornet P y Bermejo Fenoll A, Pemphigus vulgaris. A presentation of 14 cases and review of the literature, Med Oral Patol Oral Cir Bucal 2005; 10: 282-288.

15. Black M, Mignogna MD y Scully C, Pemphigus vulgaris, Oral Diseases 2005; 11: 119-130.

16. Rosenberg FR, Sanders S y Nelson CT, Pemphigus. A 20 year review of 107 patients trated with corticosteroids, Arch Dermatol 1976; 112: 962-970.

17. Razzaque AA y Salm M, Juvenile pemphigus, J Am Acad Dermatol 1983; 8: 799-807.

18. Graff, Lonnevig y Kaaman, T Juvenile pemphigus vulgaris, Acta Pediatric Scand 1991; 80: 262-265.

19. Hahn K, Kippes W, Amagai M, Rzany B, Broker EB y Zillikens D, “Clinical aspects and immunopathology in 48 patients with pemphigus, Hautarzt 2000; 51 (9): 670-677. 20.Claude B y Rudolph JL , Pemphigus, The Lancet, 2005; 366: 61-67. 21. Beutner EH y Chorzelski TP, Studies on etiologic factors in pemphigus, J. Cutaneous Pathology. 1976; 3: 67-74. 22.Weinberg MA, Insler MS y Campen RB, Mucocutaneous features of autoimmune blistering diseases, Oral Surg, Oral Med, Pathol Oral Radiol Endod 1997; 84: 517-534. 23. Bezold G, Sander CA, Flaig MJ, Peter RU y Messer G, Lack of detection of human herpesvirus (HHV)-8 DNA in lesional skin of German pemphigus vulgaris and pemphigus foliaceus patients, J Invest Dermatol 2000; 114: 739-741. 24.Mignogna MD, Lo Muzio L, Galloro G, Satriano RA, Ruocco V y Bucci E, Oral pemphigus: clinical significance of esophageal involvement: report of eight cases, Oral Surg, Oral Med, Pathol Oral Radiol Endod 1997; 84: 179-184.

20. Parlowky T, Welzel J, Amagai M, Zillikens D y Wygold T, Neonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns, J Am Acad Dermatol 2003; 48 (4): 623-625.

21. Thivolet J, Pemphigus: past, present and future, Dermatol 1994; 189 (suppl 2): 26-29.

22. Vega Memije ME, Sáez de Ocariz M, Cortés M, Franco R, Domínguez-Soto L y Granados Arriola J, Análisis de HLA-DR en pacientes mexicanos con pénfigo, Gac Med Méx 2001; 137 (6): 535-540.

23. Contran RS, Kumar V, Robbins SL. “La piel, pénfigo vulgar”. En Robbins SL, Patología estructural y funcional, 6a ed., McGraw-Hill- Interamericana, Madrid, 2000, 1289-1330.

24. Lever WF y Schaumburg-Lever Gundula, Treatment of pemphigus vulgaris, Arch Dermatol 1984; 120: 44-47.