•Localized mild disease with oral or skin lesions alone may initially be treated with local corticosteroids.
•The side effects of systemic corticosteroid therapy must always be taken into account and the patients should be regularly examined both clinically and by laboratory testing for this possibility.
•Patients with oral lesions should avoid mechanical injuries and should be advised to maintain good oral hygiene.
Suggested Therapies
Systemic Treatment
Oral Corticosteroids
Oral corticosteroids are (he mainstay of therapy for bullous pemphigoid. The majority of patients with generalized disease usually respond well to 40-80 mg/day of prednisone or prednisolone. It usually takes 2-3 weeks to stop new bullae formation and for old lesions to heal. Once the disease is controlled, the dose of the corticosteroid is tapered slowly over months and finally the drug is withdrawn. Recurrences are not unusual.
Immunosuppressive Drugs
Azathioprine 50-100 mg/day appears to be effective and it is the most commonly used corticosteroid-sparing agent for bullous pemphigoid. Cyclophosphamide 100-200 mg/day or cyclo-sporine 5-8 mg/kg per day also appears to be effective. Recently, mycophenolate mofetil 2 g/day has been used either in combination with corticosteroids or as monotherapy in the treatment of bullous pemphigoid with considerable success.
Dapsone/Sulfapyridine
Dapsone 50-100 mg/day or sulfapyridine 2-4 g/day might be effective in some cases (10-15%) of bullous pemphigoid, in particular in younger patients or patients with localized oral lesions.
Topical Treatment
Localized oral or skin lesions may be successfully treated with topical corticosteroids alone. This treatment may be given either in the form of oral paste or ointment, or in the form of an intra-lesional injection.
Alternative Therapies
•Tetracycline/niacinamide: Tetracycline 1.5-2.0 g/day alone or in combination with niacinamide 1.5 g/day are useful, particularly for controlling limited disease.
•Plasmapheresis: This can be used as an adjuvant to corticosteroids in selected severe cases of bullous pemphigoid.
•Future therapies of bullous pemphigoid might be directed toward the induction of compensating BP180 and BP230 isoforms.
•Topical use of tacrolimus in an adhesive paste form may also be useful for localized oral lesions.
References
Böhm M, Beissert S, Schwarz T, et al Bullous pemphigoid treated with mycophenolate mofetil. Lancet 1997, 349:541.
Grundmann-Kollmann M, Kaskel P, Leiter U, et al. Treatment of pemphigus vulgaris and bullous pemphigoid with mycophenolate mofetil monotherapy. Arch Dermatol 1999;135:724–725.
Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002;346:321–327.
Korman NJ. New immunomodulating drugs in autoimmune blistering diseases. Dermatol Clin 2001;19:637–648.
Liu Z, Diaz LA. Bullous pemphigoid: End of the century overview. J Dermatol 2001;28:647–650.
Stern RS. Bullous pemphigoid therapy—think globally, act locally. N Engl J Med 2002;346:364–367.
Wojnarowska F, Kirtschig G, Khumalo N. Treatment of sub-epithelial immunobullous diseases. Clin Dermatol 2001;19:768–777.
Burkitt Lymphoma
Burkitt lymphoma is a high-grade malignancy of B-lymphocyte origin that usually affects the jaws.
Epstein-Barr virus has been implicated in the pathogenesis of Burkitt’s lymphoma.
There are three types of Burkitt lymphoma: a) African, b) endemic, and c) American or sporadic.
•Pain, tenderness, paresthesia, bone destruction, and tooth mobility and loss are common symptoms and signs
•A large ulcerating or nonulcerating soft tissue mass may also be present in the oral mucosa and gingiva
•The maxilla is more frequently affected than the mandible
•Usually affects children 2-12 years of age
The clinical diagnosis should be confirmed by a biopsy and histopathologic examination.
•Non-Hodgkin lymphoma
•Osteosarcoma
•Chondrosarcoma
•Ewing’s sarcoma
•Multiple myeloma
•Cherubism
•Central giant cell granuloma
•Odontogenic tumors
•Chemotherapy—CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) regimens or more complex chemotherapy-is the treatment of choice, either alone or in combination with radiotherapy and surgery. This regimen has improved the prognosis of Burkitt lymphoma.
•Radiation therapy is the second treatment choice, either alone or in combination with chemotherapy.
•Surgery can also be used for early localized lesions.
References
Hesseling PB, Broadhead R. Molyneux E, et al. Malawi pilot study of Burkitt lymphoma treatment. Med Pediatr Oncol 2003;41:532–540.
Levine AM. Challenges in the management of Burkitt’s lymphoma. Clin Lymphoma 2002;3(suppl 1):S19-S25.
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