
A 79 year-old male patient presented clinically as a typical case of pemphigus foliaceus. He displayed a seborrhoeic pattern of crusting and erosions with an absence of oral involvement. Surprisingly, a biopsy showed suprabasilar acantholysis and direct immunofluorescence consistent with pemphigus vulgaris. Indirect pemphigus antibodies were positive for desmoglein 3 (DSG-3) but negative for DSG-1 antibodies. Most cases of cutaneous pemphigus are reported to have both DSG-1 and DSG-3 antibodies. A rare cutaneous subtype of pemphigus vulgaris is reported in the literature that may present clinically as seen in our patient. Our patient is clinically similar to these previous reported cases but with a negative DSG-1 antibody titre. The extended DSG compensation theory explains the differences of clinical expression in pemphigus based on the variable pathogenicity of DSG antibodies as well as the distribution of DSG-1 and DSG-3. We discuss this case, highlighting the theories of the pathogenesis of pemphigus and, in particular, the cutaneous pemphigus subtype.
Male, Desmoglein 3, Desmoglein 1, Foliaceus, Phenotype, Fluorescent Antibody Technique, Direct, 616, Lesions, Humans, Antibody, Pemphigus, Aged, Autoantibodies
Male, Desmoglein 3, Desmoglein 1, Foliaceus, Phenotype, Fluorescent Antibody Technique, Direct, 616, Lesions, Humans, Antibody, Pemphigus, Aged, Autoantibodies
| selected citations These citations are derived from selected sources. This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically). | 13 | |
| popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network. | Top 10% | |
| influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically). | Top 10% | |
| impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network. | Average |
