We report a Japanese case of Darier's disease with brown and white pap
ules or maculae distributed on the neck, trunk and dorsa of hands. Bot
h brown papules and leukoderma showed typical histological features of
Darier's disease. Moreover, there were much fewer melanocytes and mel
anosomes in the epidermis of both lesions. The corneal layer of the br
own papules was far thicker than that of the leukoderma. Therefore, th
e thick corneal layer of the brown papules may prolong the retention o
f a few melanosomes or a little melanin to induce hyperpigmentation, w
hile the thin corneal layer of leukoderma may not do so, thereby produ
cing hypopigmentation. The difference in clinical and histological cou
rses after involvement of melanocytes was proposed to be the cause of
the discrepancy between the previous reports. We conclude that leukode
rma was the primary lesion, the postinflammatory depigmented spots, or
the atypical or subclinical eruption of Darier's disease.