Clinical and Experimental Dermatology (1978) 3, 327.

Clinical meeting of the St John's Hospital Dermatological Society: i December 1977

Juvenile xanthogranuloma (naevoxantho-endothelioma)

K.GRICE Watford General Hospital, Peace Memorial Wing, Watford, Herts, WDI 7HH

Accepted for publication 6 February 1978

History K.B., a 20-month-old boy developed a sudden eruption of multiple papules on the face at the age of 6 months, and lesions appeared on the trunk at the age of 15 months. He was born in England of Pakistani parents and had previously had a Ramstedt's pyloromyotomy for congenital hypertrophie pyloric stenosis.

Examination Multiple dusky erythematous papules were scattered over the face (Fig. i), and the margins of the nares and the lower eyelids were outlined by multiple pin-head sized papules, while larger (2-3 mm diameter) round or oval lesions were present elsewhere on the face. On the trunk, there were a few macular pigmented lesions (2-5 mm diameter) and many erythematous yellowish papules. Darier's sign (friction urticaria) was negative. There was no evidence of systemic involvement.

Investigation Skin biopsy of a nodule on the face showed a subepidermal infiltrate consisting of foamy histiocytes (Fig. 2). A few mast cells were present in the deeper dermis. The histology was considered to be consistent with an early or pre-xanthomatous stage of naevoxantho-endothelioma (E. Wilson Jones). The haemoglobin was 10-4 g/dl with an iron deficient picture. Normal investigations included haemoglobin electrophoresis (EP), fasting plasma lipids, lipoprotein EP and chest X-ray. 0307-6938/78/0900-O327$02.00 ©1978 Blackwell Scientific Publicatiotis

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Figure i. Multiple papules of juvenile xanthogranuloma.

Figure 2. Skin biopsy from face showing a foamy histiocytic infiltrate in the papillary dermis (H& Ex360).

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Discussion This child was unusual in showing the large number of papular lesions and the lentigo-like hyperpigmented macules that have been described in juvenile xanthogranuloma. Juvenile xanthogranuloma has been found in association with neurofibromatosis, Nieman-Pick disease (Sibulkin & Olichney, 1973), myeloproliferative diseases such as leukaemia (Conejo Garcia, Martinez Sahuquillo & Navarrete Ortega, 1974) and urticaria pigmentosa. De Villez & Limmer (1975) described a girl with two identical yellow papules and a negative Darier's sign. The histology of one ofthe lesions was characteristic of mastocytosis with a tumour like aggregation of mast cells; the other lesion showed the histology of juvenile xanthogranuloma, electron microscopy showing fat laden histiocytes with no Langerhans' granules. The 'flush syndrome' has been described in juvenile xanthogranuloma but it has been pointed out that finding some mast cells in the infiltrate does not justify a diagnosis of urticaria pigmentosa. Juvenile xanthogranuloma is said to be localized to the skin in about 75",, of the cases but systemic involvement has been reported in the eyes, lungs, liver, spleen, pericardium, stomach, colon, testeSj ovaries, kidneys and mucous membrane ofthe tongue (Conejo Garcia et ah, t974). References CONEJO GARCIA, E . , MARTINEZ SAHUQUILLO, A. & NAVARRETE ORTEGA, M . (1974) Xanthogranuloma

juvenil difuso papulotuberoso. Actas Dermo-sifihograficas, 65, 421-450. SIBULKIN, D . & OLICHNEY, J.J. (1973) Juvenile xanthogranuloma in a patient with Nieman-Pick disease. Archives of Dermatology, 108, 829-831. D E VILLEZ, R.L. & LIMMER, B . L . (1975) Juvenile xanthogranuloma and urticaria pigmentosa. Archives of Dermatology, i i i , 365-366.