Background Perforating granuloma annulare (PGA) is considered an histo
logic subtype of granuloma annulare (GA) and it is described as a very
rare disease, usually of children, affecting the dorsum of the hands.
Mechanisms leading to perforation are unknown. Our experience suggest
ed a different clinical presentation, so we decided to review our pati
ents and the cases published. Methods We present six cases of PGA and
review 52 PGA cases reported in the literature. Data regarding sex, ag
e, time of evolution of disease, clinical features, laboratory finding
s, treatment, and follow-up were collected, The significance level was
determined by the chi(2) or Student t test when appropriate, Results
The prevalence of PGA could be up to 5% of GA. Pustular-like lesions c
an be found in 26% of cases, and scars in 37%; papular, umbilicated, a
nd crusted lesions being the most common finding, While PGA appears as
a single lesion in only 9% of cases, and half of the patients are old
er than 30 years, in GA 50% of cases present as a single lesion and 80
% of patients are younger than 30 years. Conclusions PGA is different
to GA not only histologically but also clinically, It is a disseminate
d disease, affecting both children and adults, which is characterized
by the presence of multiple papules, most of them umbilicated and/or c
rusted, and characteristically pustular lesions and scars. Histology s
uggests that the superficial localization of the necrobiotic granuloma
leads to the epidermal perforation. Treatment is disappointing.