Introduction. Subepidermal autoimmune bullous dermatoses form a clinic
al entity for which there is not always an individualized clinical and
pathological description. Case report. A patient presented with bullo
us skin disease of atypical nature. There was an almost total desepide
rmization of the legs, vast areas of erosion on the trunk and arms wit
h a Nikolski sign in an area of healthy skin, buccal involvement, mult
iple milium cysts and ungueal dystrophies with nail loss.Discussion. T
his clinical presentation in this patient suggested acquired bullous e
pidermolysis. However, according to the recently defined clinical crit
eria for pemphigoid, the probability of correct diagnosis of pemphigoi
d was greater than 95 p. 100 since nearly three fourths of the major c
riteria were present. This diagnosis was confirmed by reference techni
ques (electron microscopy, indirect electron immunomicroscopy and immu
noblotting). Thus, bullous autoimmune diseases of the dermoepidermal j
unction can be reliably differentiated on the bases of the clinical fe
atures, together with direct and indirect immunofluorescence on salt-s
plit skin.