2007, Number 6
<< Back Next >>
Med Cutan Iber Lat Am 2007; 35 (6)
Pemphigus vulgaris in a 14 year old girl
Chanussot-Deprez C, Vega-Memije ME, Mosqueda-Taylor A, Vallejo-Vega B, Barrezueta-Chea S, Hernández-Delgado L, Vázquez-Zavala G
Language: Spanish
References: 40
Page: 285-289
PDF size: 236.18 Kb.
ABSTRACT
A 14 years old with severe pemphigus vulgaris is reported. She received treatment with oral prednisone but new lesions developed and the 90% of the body surface was affected. Infusion of fresh frozen plasma was started and high dosis of oral prednisone was given for five days without improvement. On the sixth day, an intravenous pulse dose with methylprednisolone for five days was administered and followed by oral prednisone and oral azathioprine. With this treatment modality, there was no formation of new blisters, however, the patient presented infectious complications with pneumoniaand septicemia which resolved with intravenous antibiotics. The patient was discharge from hospital without active lesions. The dermatosis was difficult to control and failed to tolerate tapering of oral prednisone. She presented Cushing syndrome as a complication of steroid therapy and oral cyclosporine A was initiated with oral prednisone. Pemphigus vulgaris is rare in children and it needs to be considerate within the differential diagnosis of bullousdisease of childhood. There are no reported randomized control trials in the literature to provide evidence for the best therapeutic approach. Pemphigusvulgaris in childhood has been reported as case series and there are no well established standard recommendations for treatment.
REFERENCES
Bjarnason B, Flosadottir E. Childhood, neonatal, and stillborn pemphigus vulgaris. Int JDermatol 1999;38:680-8.
Harangi F, Varszegi D, Schneider I, Zombai E. Complete recovery from juvenile pemphigus vulgaris. Pediatr Dermatol 2001;18:51-3.
Singalavanija S, Limpongsanurak W. Immunobullous diseases in Thai Children: report of 24 cases. J Med Assoc Thai 2003;86:S681-8.
Weinstein M. Pemphigus vulgaris: the eyes have it. Pediatrics 2003;112:183-5.
Kong HH,Prose NS, Ware RE, Hall RP 3rd. Successful treatment of refractory childhood pemphigus vulgaris with anti CD20 monclonal antibody (rituximab). Pediatr Dermatol2005;22:461-4.
Antaya RJ, Prieto VG, Prose NS. Mucosal erosions and bullae in a child. Pemphigusvulgaris in a child with tinea capitis. ArchDermatol 2000;136:666-70.
Harangi F, Varszegi D, Schneider I, ZombaiE. Complete recovery from juvenile pemphigus vulgaris. Pediatr Dermatol 2001;18:51-3.
Thami GP, Kaur S,Kanwar AJ. Severe childhood pemphigus vulgaris aggravated byenalapril. Dermatology 2001;202:341.
Burgan SZ, Sawair FA, Napier SS. Case report: oral pemphigus vulgaris with multiple oral polypsin a young patient. Int J Dent 2003;53:37-40.
Cetovska P, Pizinger K. Childhood pemphigus associated with montelukast administration. Clin Exp Dermatol 2003;28:328-9.
Mahajan VK, Sharma NL, Sharma RC, Garg G. Twelve year clinico-therapeutic experience in pemphigus: a retrospective study of 54cases. Int J Dermatol2005;44:821-7.
Ariyawardana A, Tilakaratne WM, Dissanayake M, Vitana arachchi N, Basnayake LK, Sitheeque MA, et al. Oral pemphigus vulgaris in children and adolescents: a review ofthe litterature and a case report. Int J PediatrDent2005;15:287-93.
Roca-Miralles M, Repiso T, Borona M, Kanitakis J, Vidal-Lliteras J, Castells-Rodellas A. Pemphigus vulgaris in a 18 monthold-boy.Ann Dermatol Venereol1993;120:777-9.
Hup JM, Bruinsma RA, Boersma ER, DeJong M C. Neonatal pemphigus vulgaris: transplacental transmisión of antibodies. Pediatr Dermatol 1986;3:468-72.
Campo Voegeli A, Muniz F, Mascaró JM, García F, Casals M, Arimany JL, et al. Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphi-gus vulgaris. Br J Dermatol 2002;147:801-5.
Parlowsky T, Welzel J, Amagai M, Zillikens D, Wygold T. Neonatal pemphigus vulgaris: Ig G4 autoantibodies to desmoglein 3 induce skin blisters in newborns. J Am Acad Derma-tol2003;48:623-5.
Starzycki Z, Chorzelski TP, Jablonska S. Familial pemphigus vulgaris in mother and daughter. Int J Dermatol 1998;37:211-4.
Fainaru O, Mashiach R, Kupferminc M, Shenhav M, Pauzner D, Lessing J. Pemphigus vulgaris in pregnancy: a case report and review of theliteratura. Human Reprod 2000;15:1195-7.
Vega ME, Villatorro VG, Mosqueda A. Pénfigo vulgar. Informe del manejo de 40 casos. Dermatología Rev Mex 1998;42:244-9.
Kanwar AJ, Dhar S. Oral pemphigus vulgaris (letter). Pediatr Dermatol 1995;12:195-7.
Laskaris G, Stoufi E. Oral pemphigus in a 6 year old girl. Oral Surg Oral Med Oral Pathol 1990;69:609-611.
Hamlet KR, Stevens SR, Gushurst C, Karabin G, Cooper KB. Juvenile pemphigus vulgaris associated with Graves Disease. J Am Acad Dermatol 1995;33:132-4.
Wananukul S, Pongprasit P. Childhood pemphigus. Int J Dermatol 1999;38:29-35.
Firth N, Rich A, Varigos G, Reade PC. Oral pemphigus in young adults. Int J Dermatol 1991;30:352-6.
Keinman N. New immunomodulating drugs in autoimmune bullous disease. Dermatol Clin 2001;19.
Ahmed R, Salm M. Juvenile pemphigus. J Am Acad Dermatol 1983;8:799-807.
Kanwar A, Kaurr S. Pemphigus in children. Int J Dermatol 1991;30:343-6.
Herbst A, Bystryn JC. Patterns of remission in pemphigus vulgaris. J Am Acad Dermatol 2000;42:422-7.
Ahmed R, Moy R. Death in pemphigus. J Am Acad Dermatol 1982;7:221-8.
Mimouni D, Anhalt GJ, Lazarova Z, Aho S, Kazerounian S, Kouba DJ, et al. Paraneoplastic pemphigus in children and adolescents. Br J Dermatol 2002;147: 725-32.
Nikolskaia OV, Nousari CH, Anhalt GJ. Paraneoplastic pemphigus in association with Castleman’s disease. Br J Dermatol 2003;149:1143-51.
Lane J, Woody C, Davis L, Guil M, Jerath R. Paraneoplastic autoimmune multiorgansyndrome (paraneoplastic pemphigus) in a child: case report and review of the literature. Pediatrics 2004;114:513-16.
Bystryn JC. Steinman N. The adjuvant therapy of pemphigus: an update. Arch Dermatol 1996;132:203-212.
Turner M, Sutton D, Sauder D. The use of plasmapheresis and immunosupresion in the treatment of pemphigus vulgaris. J Am Acad Dermatol 2000;43:1058-64.
Shich S, Fang YV, Becker JL, Holm A, Beutner EH, Helm TN. Pemphigus, pregnancy, and plasmapheresis. Cutis 2004; 73:327-9.
Aberer W, Wolff-Schreiner E, Stingl G, Wolff K. Azathioprine in the treatment of pemphigus vulgaris. A long term follow up. J Am Acad Dermatol 1987;16:527-33.
Alijotas J, Pedregosa R, Bosch J, Vilardel M. Prolongued remission after cycloporine therapy in pemphigus vulgaris: report of two young siblings. J Am Acad Dermatol 1990;23:701-3.
Bjarnason B, Skoglund C, Flosadottir E. Childhood pemphigus vulgaris treated with dapsone: a case report. Pediatr Dermatol 1998;15:381-3.
Mimouni D, Anhalt GJ, Cummins DL, Kouba DJ, Thorne JE, Nousari HC. Treatment of pemphigus vulgaris and pemphigus foliaceous with mycophenolate mofetil. Arch Dermatol 2003;139:739-42.
Kong HH, Prose NS, Ware RE. Successful treatment of refractory childhood pemphigus vulgaris with CD 20 monoclonal antibody (rituximab). Pediatr Dermatol 2005;22:461-4.