[Mucous membrane pemphigoid: clinical manifestations and treatment with corticosteroids, dapsone and cyclophosphamide in 5 patients].
BACKGROUND
Cicatricial pemphigoid includes several processes which are characterized by the presence of subepidermal bullae, and which affect the mucous membranes and, more rarely, the skin. At present, the term mucous membrane pemphigoid (MMP) is more accepted than other names used in the past, as they do not clearly define the broad spectrum presented by this disease. MMP can cause significant dysfunctions, primarily in the mucous membranes. Therefore, it is necessary to diagnose the disease as soon as possible, in order to quickly initiate systemic immunosuppressive treatment.


METHODS
We present our experience with 5 patients with MMP. We analyze the clinical manifestations and the response to immunosuppressive treatment during the evolution of the disease.


RESULTS
The patients were aged 41 to 69 years. The most frequent location of the lesions was the oral mucosa (80 %) and the ocular mucosa (80 %), followed by the pharyngeal mucosa (60 %), laryngeal mucosa (40 %), skin, anal mucosa (20 %) and genital mucosa (20 %). Three patients received systemic corticosteroids, dapsone and cyclophosphamide, and several sessions of plasmapheresis were also associated in one patient. One patient was controlled with topical corticosteroids and dapsone.


CONCLUSIONS
Many patients with MMP can present with severe secondary complications. For this reason, the diagnosis must be confirmed quickly and the appropriate treatment started as soon as possible. The association of corticosteroids, dapsone and cyclophosphamide is a combination that gives very good results.
Servicio de Dermatología, Clínica Universitaria de Navarra, Pamplona, Spain. aespana@unav.es
Agustín
A
España
Julio
J
del Olmo
Miren
M
Marquina
Secundino
S
Fernández
Carlos
C
Panizo
Miguel
M
Maldonado
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D003520:Cyclophosphamide / Q000627:therapeutic use*; D003622:Dapsone / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000473:pathology*
D000305:Adrenal Cortex Hormones; D003520:Cyclophosphamide; D003622:Dapsone