Background: Even though only 33 cases of Erythromelanosis follicularis faci
ei et colli (EFFC) hare been reported around the world, there have been no
quantitative clinicopathological studies yet. The purpose of this study is
to examine quantitatively clinical and histopathological evaluations of EFF
C using image analysis system.
Method: Clinical grading of its severity was assessed. Many parameters of t
he horny layer, granular layer, epidermis and the dermal components were me
asured and compared to controls by image analysis. The correlation between
the histological findings and clinical grading was assessed.
Result: Thickness and compactness of the horny layer was significantly incr
eased in EFFC. There were no significant differences in the number of melan
ocytes and severity of pigmentation between EFFC and controls; however, mel
anization was slightly increased in EFFC. The percent area of the superfici
al blood vessels was significantly increased and correlated with visual gra
ding of erythema. Diameters of hair shafts and outer root sheaths, and thic
kness of inner root sheaths were decreased. Basal layer pigmentation was co
rrelated with the clinical features of pigmentation.
Conclusion: This study supported the findings of hyperkeratosis and dilatat
ion of superficial dermal blood vessels in EFFC. The pigmentation of the ba
sal layer and the percent area of the inner spaces of the superficial derma
l blood vessels may reflect the clinical severity more than the other param
eters. Although follicular plugging is a characteristic clinical feature of
EFFC, enlargement of hair follicles and hair shafts were not characteristi
c pathologic changes EFFC in our study.