Background: The nosologic field of aphthosis is not well defined. While cri
teria for Behcet's disease (BD) are lacking in severe unipolar or bipolar a
phthosis, these disorders however lead to an important impairment of qualit
y of life. The aim of this study was to assess the relationship between sev
ere aphthosis and BD. Methods: Records of all patients hospitalized for aph
thosis or BD during a 20-year period were reviewed. Results: According to c
linical data, cases (n = 104) were classified as unipolar aphthosis (group
I, n = 53), bipolar aphthosis (group II, n = 30) or BD (group III, n = 21).
The characteristics of aphthae (number, localization, recurrences, presenc
e of scars) were similar in all groups. Specific cutaneous manifestations (
erythema nodosum, pseudofolliculitis, papulonodules) were present in 15, 17
and 95% of patients of groups I, II and III. Cutaneous puncture hypersensi
tivity was present in 14 out of 18 patients of group III and no patient of
the two other groups. At least one extramucous specific manifestation (cuta
neous, ophthalmologic, neurologic, articular, vascular) was present in 43%
of patients of group I and II, and 100% in group ill. Conclusion: These dat
a support the hypothesis of a continuous spectrum linking severe aphthosis
to BD.