Images in Emergency Medicine

Abstract

DiagnosisPemphigus vulgaris. This patient’s bullous dermatitis is an example of pemphigus vulgaris. Pemphigus vulgaris is an autoimmune disorder caused by autoantibodies directed against the cell surface of keratinocytes.1Amagai M. Klaus-Kovtun V. Stanley J.R. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion.Cell. 1991; 67: 869-877Abstract Full Text PDF PubMed Scopus (861) Google Scholar Autoantibody binding results in loss of cell adhesion, which manifests clinically as fragile intraepidermal bullae. The mortality associated with this disease is 10% to 15%, which can be mainly attributed to secondary infection, dehydration, and complications of therapy.2Kanwar A.J. Dhar S. Factors responsible for death in patients with pemphigus.J Dermatol. 1994; 21: 655-659PubMed Google ScholarPemphigus vulgaris is characterized by flaccid bullae that rupture easily. Oral lesions tend to precede cutaneous findings.3Ruocco E. Baroni A. Wolf R. et al.Life-threatening bullous dermatoses pemphigus vulgaris.Clin Dermatol. 2005; 23: 223-226Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar This disorder is commonly associated with neoplastic4Anhalt G.J. Kim S.C. Stanley J.R. et al.Paraneoplastic pemphigus an autoimmune mucocutaneous disease associated with neoplasia.N Engl J Med. 1990; 223: 1729-1735Crossref Scopus (889) Google Scholar and rheumatologic disorders but may also be caused by certain medications (eg, angiotensin-converting enzyme inhibitors). Lesions will not heal without immunosuppressive therapy.This patient underwent biopsy with immunofluorescence, which confirmed the diagnosis. A complete metabolic profile, CBC count, chest radiograph, prostate-specific antigen, and erythrocyte sedimentation rate were also performed to screen for common malignancies. He began taking prednisone and mycophenolate mofetil in the emergency department after consultation with dermatology. DiagnosisPemphigus vulgaris. This patient’s bullous dermatitis is an example of pemphigus vulgaris. Pemphigus vulgaris is an autoimmune disorder caused by autoantibodies directed against the cell surface of keratinocytes.1Amagai M. Klaus-Kovtun V. Stanley J.R. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion.Cell. 1991; 67: 869-877Abstract Full Text PDF PubMed Scopus (861) Google Scholar Autoantibody binding results in loss of cell adhesion, which manifests clinically as fragile intraepidermal bullae. The mortality associated with this disease is 10% to 15%, which can be mainly attributed to secondary infection, dehydration, and complications of therapy.2Kanwar A.J. Dhar S. Factors responsible for death in patients with pemphigus.J Dermatol. 1994; 21: 655-659PubMed Google ScholarPemphigus vulgaris is characterized by flaccid bullae that rupture easily. Oral lesions tend to precede cutaneous findings.3Ruocco E. Baroni A. Wolf R. et al.Life-threatening bullous dermatoses pemphigus vulgaris.Clin Dermatol. 2005; 23: 223-226Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar This disorder is commonly associated with neoplastic4Anhalt G.J. Kim S.C. Stanley J.R. et al.Paraneoplastic pemphigus an autoimmune mucocutaneous disease associated with neoplasia.N Engl J Med. 1990; 223: 1729-1735Crossref Scopus (889) Google Scholar and rheumatologic disorders but may also be caused by certain medications (eg, angiotensin-converting enzyme inhibitors). Lesions will not heal without immunosuppressive therapy.This patient underwent biopsy with immunofluorescence, which confirmed the diagnosis. A complete metabolic profile, CBC count, chest radiograph, prostate-specific antigen, and erythrocyte sedimentation rate were also performed to screen for common malignancies. He began taking prednisone and mycophenolate mofetil in the emergency department after consultation with dermatology. Pemphigus vulgaris. This patient’s bullous dermatitis is an example of pemphigus vulgaris. Pemphigus vulgaris is an autoimmune disorder caused by autoantibodies directed against the cell surface of keratinocytes.1Amagai M. Klaus-Kovtun V. Stanley J.R. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion.Cell. 1991; 67: 869-877Abstract Full Text PDF PubMed Scopus (861) Google Scholar Autoantibody binding results in loss of cell adhesion, which manifests clinically as fragile intraepidermal bullae. The mortality associated with this disease is 10% to 15%, which can be mainly attributed to secondary infection, dehydration, and complications of therapy.2Kanwar A.J. Dhar S. Factors responsible for death in patients with pemphigus.J Dermatol. 1994; 21: 655-659PubMed Google Scholar Pemphigus vulgaris is characterized by flaccid bullae that rupture easily. Oral lesions tend to precede cutaneous findings.3Ruocco E. Baroni A. Wolf R. et al.Life-threatening bullous dermatoses pemphigus vulgaris.Clin Dermatol. 2005; 23: 223-226Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar This disorder is commonly associated with neoplastic4Anhalt G.J. Kim S.C. Stanley J.R. et al.Paraneoplastic pemphigus an autoimmune mucocutaneous disease associated with neoplasia.N Engl J Med. 1990; 223: 1729-1735Crossref Scopus (889) Google Scholar and rheumatologic disorders but may also be caused by certain medications (eg, angiotensin-converting enzyme inhibitors). Lesions will not heal without immunosuppressive therapy. This patient underwent biopsy with immunofluorescence, which confirmed the diagnosis. A complete metabolic profile, CBC count, chest radiograph, prostate-specific antigen, and erythrocyte sedimentation rate were also performed to screen for common malignancies. He began taking prednisone and mycophenolate mofetil in the emergency department after consultation with dermatology.