Normolipemic Papuloeruptive Xanthomatosis in an Infant

Abstract

A 8-month-old boy showed several yellow to brownish yellow, papular and/or nodular eruptions predominantly on the face. Initial clinical and histologic findings indicated possible juvenile xanthogranuloma or papular xanthoma. Since his second year, the papulonodular lesions have gradually merged into large confluent plaques, particularly on the face, nape, and axillae. Consequently, it was considered that his condition had terminated in diffuse plane xanthoma. Serum lipid panels showed normolipemia. Finally, a diagnosis of papuloeruptive xanthomatosis with normal lipid metabolism and no associated systemic disorders was made. The patient's papulonodular lesions have continued to involute spontaneously since the age of three.

Congenital papular xanthoma

“…Sanchez et al . 2 and Horiuchi and Ito 9 suggested the use of iron staining and PAS staining for differential diagnosis, but these were negative in our case. Papular xanthoma may sometimes be difficult to differentiate clinically from the chronic progressive form of histiocytosis X.…”

Normolipemic Papuloeruptive Xanthomatosis in a Child

“…The skin lesions are small papules with some confluence. Horiuchi and Ito (2) reported an 8-month-old boy with several yellowish to brownish yellowish papules and nodular eruptions predominately on the face. Since the age of 2, the papulonodular lesions have gradually merged into large confluent plaques, especially on the face, nape, and axillae.…”

“…Aplasia cutis may be defined as a congenital absence of skin due to localized failure of its development (1,2). Aplasia cutis of the scalp is by far its most common form.…”

Papular xanthoma: a clinicopathological study of 10 cases

“…Of further differential diagnostic concern is “normolipemic papuloeruptive xanthomatosis” 36 . This disease, also known in the literature as diffuse normolipemic plane xanthoma, is easily distinguishable as it shows macules and patches, rarely papules with a frequently sparse macrophage infiltrate in superficial location.…”