Acquired Epidermolysis Bullosa


Ceylan N., Gurel M. S., Kiremitci U., Erdemir A., Demirkesen C.

ISTANBUL MEDICAL JOURNAL, cilt.9, sa.2, ss.100-104, 2008 (ESCI) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 9 Sayı: 2
  • Basım Tarihi: 2008
  • Dergi Adı: ISTANBUL MEDICAL JOURNAL
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI)
  • Sayfa Sayıları: ss.100-104
  • Anahtar Kelimeler: Acquired epidermolysis bullosa, autoimmune vesiculobullous diseases
  • Acıbadem Mehmet Ali Aydınlar Üniversitesi Adresli: Hayır

Özet

Acquired epidermolysis bullosa is achronic autoimmune bullous disease which is characterized by formation of subepidermal blister formatian on the traumatized skin and mucosa. A 48 year old male patient was admitted to hospital with the compliant of blister formation on the trunk and oral cavity. In the dermatological examination, there are erosions covered with crusts and Nikolsky (-), intaci bullae on erythematous skin of the palmoplantar area, dorsum of the hands and feet, fateri aspect of the fingers. thorax and umblicus and atrophic scars on the scrotum and inguinal area. A punch biopsy was taken with the prediagnosis of bullous pemphigoid, paraneoplastic pemphigus, bullous SLE. There were subepidermal bullae formation on the histopathological examination and linear lg G and C3 accumulation along the basement membrane on the direct immunoflorescent assay. The findings are correlated with bullous pemphigoid. Treatment with methyl prednisolon 80 mg/day was started. As new bullae formatian continued beside the therapy, a new punch biopsy from perilesianal area was taken for salt split skin test. lg G and C3 accumulation on the bullae floor and no accumulation on the ceiling of the bultae was observed. So. the patient was accepted as acquired epidermolysis bullosa. At the end of two years with this therapy regimen, there is no new bullae formatian so steroid dosage is tapered and stopped. In case of unresponsiveness to treatment in the bullous pemphigoid and the other vesiculobullous diseases as in our patient, the clinical properties and diagnosis should be reviewed.