Toxic epidermal necrolysis and paraneoplastic pemphigus

Abstract

Although we found David Becker's seminar (May 9, p 1417)1Becker DS Toxic epidermal necrolysis.Lancet. 1998; 351: 1417-1420Summary Full Text Full Text PDF PubMed Scopus (142) Google Scholar on toxic epidermal necrolysis (TEN) informative, more emphasis should have been given to excluding alternative diagnoses. This point is important because systemic corticosteroids may be harmful in TEN, 2Halebian P Madden M Finglestein G Corder V Shires G Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids.Ann Surg. 1986; 204: 503-512Crossref PubMed Scopus (328) Google Scholar but are highly effective in disorders that enter the differential diagnosis, such as neoplastic disease.In January, 1996, a 56-year-old-man was referred to the dermatology service with a history of malaise, fever, and intraoral, perioral, and periorbital sloughing for 2 days. He had a 3-year history of chronic lymphocytic leukaemia and had received fludarabine and allopurinol before the onset of signs. The preliminary diagnosis was TEN, secondary to one of these two drugs, and initial treatment was supportive. During the next 4 days, he developed dusky red patches, bullae, and sloughing of the skin which rapidly extended over the neck, trunk, and limbs to involve more than 60% of his skin surface. Nikolsky's sign was positive. Light microscopy and direct immunofluorescence examination of a skin biopsy specimen indicated pemphigus. Indirect immunofluorescence from a serum sample on monkey oesophagus and rat bladder epithelium showed paraneoplastic pemphigus. He was given five pulses of methyl prednisolone 1 g and received oral prenisolone up to 200 mg daily in combination with cyclosporin 5 mg/kg daily. Despite a course complicated by life-threatening sepsis he recovered and is alive and well.Paraneoplastic pemphigus is an increasingly recognised disorder, which occurs most frequently in patients with haematological malignant disease.3Anhalt GJ Paraneoplastic pemphigus.Adv Dermatol. 1997; 12: 77-96PubMed Google Scholar As well as being at risk of developing paraneoplastic pemphigus, these patients are commonly given treatments such as allopurinol which cause TEN. The onset of paraneoplastic pemphigus can be as rapid as TEN and the clinical picture is initially indistinguishable. Paraneoplastic pemphigus should therefore be rigorously excluded by skin biopsy and indirect immunofluorescence so that appropriate treatments can be selected. Although we found David Becker's seminar (May 9, p 1417)1Becker DS Toxic epidermal necrolysis.Lancet. 1998; 351: 1417-1420Summary Full Text Full Text PDF PubMed Scopus (142) Google Scholar on toxic epidermal necrolysis (TEN) informative, more emphasis should have been given to excluding alternative diagnoses. This point is important because systemic corticosteroids may be harmful in TEN, 2Halebian P Madden M Finglestein G Corder V Shires G Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids.Ann Surg. 1986; 204: 503-512Crossref PubMed Scopus (328) Google Scholar but are highly effective in disorders that enter the differential diagnosis, such as neoplastic disease. In January, 1996, a 56-year-old-man was referred to the dermatology service with a history of malaise, fever, and intraoral, perioral, and periorbital sloughing for 2 days. He had a 3-year history of chronic lymphocytic leukaemia and had received fludarabine and allopurinol before the onset of signs. The preliminary diagnosis was TEN, secondary to one of these two drugs, and initial treatment was supportive. During the next 4 days, he developed dusky red patches, bullae, and sloughing of the skin which rapidly extended over the neck, trunk, and limbs to involve more than 60% of his skin surface. Nikolsky's sign was positive. Light microscopy and direct immunofluorescence examination of a skin biopsy specimen indicated pemphigus. Indirect immunofluorescence from a serum sample on monkey oesophagus and rat bladder epithelium showed paraneoplastic pemphigus. He was given five pulses of methyl prednisolone 1 g and received oral prenisolone up to 200 mg daily in combination with cyclosporin 5 mg/kg daily. Despite a course complicated by life-threatening sepsis he recovered and is alive and well. Paraneoplastic pemphigus is an increasingly recognised disorder, which occurs most frequently in patients with haematological malignant disease.3Anhalt GJ Paraneoplastic pemphigus.Adv Dermatol. 1997; 12: 77-96PubMed Google Scholar As well as being at risk of developing paraneoplastic pemphigus, these patients are commonly given treatments such as allopurinol which cause TEN. The onset of paraneoplastic pemphigus can be as rapid as TEN and the clinical picture is initially indistinguishable. Paraneoplastic pemphigus should therefore be rigorously excluded by skin biopsy and indirect immunofluorescence so that appropriate treatments can be selected.