Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid
Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement an...
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| Veröffentlicht in: | Dermatology (Basel) 1999-01, Vol.198 (3), p.310-313 |
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| container_title | Dermatology (Basel) |
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| creator | Wieme, N. Lambert, J. Moerman, M. Geerts, M.L. Temmerman, L. Naeyaert, J.M. |
| description | Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either. |
| doi_str_mv | 10.1159/000018139 |
| format | Article |
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The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.</description><identifier>ISSN: 1018-8665</identifier><identifier>EISSN: 1421-9832</identifier><identifier>DOI: 10.1159/000018139</identifier><identifier>PMID: 10393462</identifier><language>eng</language><publisher>Basel, Switzerland: Karger</publisher><subject>Aged ; Biological and medical sciences ; Bullous diseases of the skin ; Case Report ; Dermatology ; Diagnosis, Differential ; Epidermolysis Bullosa Acquisita - pathology ; Fatal Outcome ; Female ; Humans ; Medical sciences ; Pemphigoid, Benign Mucous Membrane - pathology ; Pemphigoid, Bullous - pathology ; Skin - pathology</subject><ispartof>Dermatology (Basel), 1999-01, Vol.198 (3), p.310-313</ispartof><rights>1999 S. Karger AG, Basel</rights><rights>1999 INIST-CNRS</rights><rights>Copyright (c) 1999 S. 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The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.</abstract><cop>Basel, Switzerland</cop><pub>Karger</pub><pmid>10393462</pmid><doi>10.1159/000018139</doi><tpages>4</tpages></addata></record> |
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| subjects | Aged Biological and medical sciences Bullous diseases of the skin Case Report Dermatology Diagnosis, Differential Epidermolysis Bullosa Acquisita - pathology Fatal Outcome Female Humans Medical sciences Pemphigoid, Benign Mucous Membrane - pathology Pemphigoid, Bullous - pathology Skin - pathology |
| title | Epidermolysis bullosa acquisita with Combined Features of Bullous Pemphigoid and Cicatricial Pemphigoid |
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