Pemfigo ad esordio anomalo

Pemphigus vulgaris is a dermatosis with an autoimmune pathogenesis usually easy to recognise. In fact it is characterized by several clinical aspects which may allow the diagnosis of this disease, later comfirmed by MO and IFD studies. Sometimes, however, the disease doesn't show this 'classical' aspect and a peculiar unfrequent onset can mislead the dermatologist for a long time. The case of a 35 year-old man, who came to our attention with pustulo-bullous painless periungual lesions, localised in both hands and feet is described. These lesions, which occurred about one year before on the first toes of one foot were diagnosed as pustulosis psoriasis and treated with etetrinate and PUVA therapy without success. These lesions were the only symptom of the disease for about one year. During hospitalization, the patient developed erosions of oral mucosa, so a pemphigus was hypothesized; the diagnosis was confirmed by MO and IFD study. The treatment with betametasone and azatioprina lead to a quickly improvement of the lesions and to the clinical recovery in about 30 days. This case is significative for the periunguealis unusual onset (there is only another case described in the letterature) which together with the relative young age of our patient lead for a long time to a diagnostic mistake. Furthermore, the recent acquisitions on pemphigus etiology are discussed.