Histologic examination using hematoxylin-eosin stain demonstrated compacted collagen, scattered lymphocytes, and ectatic blood vessels. Immunohistochemical analysis of CD1a in lesional biopsy specimen revealed an almost complete absence of epidermal Langerhans cells (Figure 2) compared with a normal control (Figure 3). The combination of clinical and immunohistochemical findings indicated Huriez syndrome. The patient was treated with acitretin and showed minimal improvement. Examination of his sister revealed identical clinical and immunohistochemical findings.