Congenital multiple juvenile xanthogranuloma

Juvenile xanthogranuloma (JXG) is a rare condition which belongs to a group of histiocytoses. It is also described as the most common non-Langerhans cell histiocytosis (non-LCH). Considering the large variability of its clinical presentation, histopathological verification of cutaneous lesions is necessary. The management of JXG involves observation, surgical treatment or systemic treatment. We present a case of a 2.5-month-old male patient with diffuse skin lesions in course of JXG, present since birth.
The 2.5-month-old male infant presenting yellow-orange skin nodules localized on his upper and lower limbs and trunk, was admitted to the Department of Dermatology. Lesions have been already present since the child’s birth and have become gradually larger. New nodules have also appeared. The boy was born on time, with perinatal history burdened with posthypoxic status and paralysis of the facial nerve, born by Caesarean section, and scored 10 points in the Apgar scale. The child was not treated for any other diseases before the hospitalization. He was vaccinated according to the valid calendar but the cycle of vaccinations was interrupted because of skin lesions occurrence. Physical examination at the admission demonstrated presence of numerous yellow-orange, dense nodules and papules (Figures 1 A–C). Skin lesions were localized on the right forearm, in the area of the right iliac ala, on the second finger of the left hand, on the third toe of the left foot and on the sole of the right foot. Basic laboratory blood tests conducted during hospitalisation revealed minor macrocytosis (90.7 fl), minor monocytosis (1.38 × 103/µl) and a slightly extended activated partial thromboplastin time (37.7 s). Other laboratory tests were normal. The infant had ophthalmological and neurological consultations. The examinations did not reveal any pathological symptoms. There were no abnormalities in abdominal ultrasonography and ultrasound examination of soft tissues within skin nodules demonstrated the typical presentation for JXG. The largest nodule located close to the right iliac ala was excised for histopathological examination with the shave biopsy method. The lesion manifested microscopically as a dense histiocytic proliferation in the papillary and reticular dermis. Epidermis was thin with focally elongated rete ridges (Figure 2 A). Foamy histiocytes and giant cells were dominating in the infiltration. Some of the giant cells were the Touton-type (Figure 2...


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