Juvenile xanthogranuloma (JXG) is a benign Non-langerhancian histiocytic proliferation of uncertain histogenesis which usually resolves spontaneously. Herein, we report a new case of diffuse juvenile xanthogranuloma.

A 17-month-old infant was presented with two months history of a diffuse asymptomatic popular eruption. The dermatological examination found orange papules well limited, firm, infiltrated, rounded to oval, 0.5 to 2 cm diameter, located in the face, trunk, upper and lower limbs (Figures 1 and 2). The immuno-histological study showed a Non-langerhansian histiocytosis type juvenile xanthogranuloma CD68+, PS100+, CD1a- (Figure 3). Biological and morphological explorations did not reveal a systemic localization. No treatment was administered because of the spontaneously resolving nature of this dermatosis. The evolution was marked by the disappearance of skin lesions after 3 years of follow-up.

Figure 1: Orange papules in the face.

Figure 2: Orange papules well limited, firm, infiltrated, rounded to oval.

Figure 3: Non-langerhansian histiocytosis type juvenile xanthogranuloma.

JXG is the most frequent Non-langerhancian histiocytosis (NLH). It usually occurs in infants and children in 80% of cases before the age of 2 years [1]. The lesions preferentially sit at the level of the upper part of the body but may also appear at the limbs. It is characterized by a single papule or a single yellow-orange nodule [2]. The JXG is rarely multiple which makes the originality of our observation. We should distinguish between the micro-nodular form which is the most frequent, and the macro nodular form that could have a particular risk of systemic involvement (lung, bones, kidneys, testicles, pericardium, eye, etc.).

The diagnosis is histological, showing a dense dermal infiltration of epithelioid cells with vacuolated and foamy histiocytes S100-, CD1-, CD68+ and touton cells [3]. The JXG prognosis is generally benign with self-regression in a few months to a few years [4]. Therapeutic abstention may be proposed. For non-regressive forms, treatment is not well codified due to the scarcity of the different entities.

In summary, our observation illustrates a clinically atypical observation of JXG by the multi-lesional and multifocal nature of lesions. It illustrates also the difficulties of classifying benign forms of NLH, because of the existence of forms of passage between different entities.

None.