Progressive nodular histiocytoma

Abstract

Extensive evaluation of the condition of a 9-year-old girl with a previously undescribed proliferative histocytic syndrome showed normal serum and tissue lipid values, which rule out the known lipid storage diseases. Clinically and histologically the case is inconsistent with any of the recognized xanthomatoses or histiocytic abnormalities.

Progressive nodular histiocytosis

“…The latter may also occur sporadically [1,12]. In our patients, the nodules were the predominant clinical feature.…”

“…Taunton and colleagues also described conjunctival involvement as seen in our patient 2. A unique fi nding in patient 2 was ocular involvement with epibulbar infi ltrates, which has only been reported once thus far [1]. An association with retinitis pigmentosa, without an evident causal relationship has been reported in one patient [24].…”

“…There have been no reports of involvement of internal organs. The disease can also affect children [1,13,14]. Except for one patient with an association with leukemia [15], there seems to be no association with malignancies or chronic infl ammatory diseases.…”

“…Only one other patient had involvement of the palms and soles [22]. Mucosal involvement has been occasionally reported, including the publication by Taunton and colleagues [1], who were the fi rst to describe this disease. In one patient, extensive laryngeal involvement led to respiratory diffi culty and resulted in laser removal of the nodular infi ltrates, which was also necessary in patient 1 [23].…”

“…It is characterized by the clinical appearance of yellow papules and larger nodules on the head, trunk, and extremities; mucous membrane involvement can occur on occasion as well [1]. There have been reports of severe and disfi guring forms of disease, but not of involvement of internal organs or systemic complications.…”