Progressive jaundice, draining ears and seborrheic dermatitis in a 25-month-old boy
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Prepared by...
Pimlak Charoenkwan, M.D.
Department of Pediatrics, Chiang Mai University
 
Diagnosis: Langerhans cell Histiocytosis
    Laboratory studies showed the following results: hemoglobin 10.6 g%, WBC count 16,800/cu
mm., platelet count 778,000/cu mm.; alkaline phosphatase 1,587 u/L, total and direct bilirubin 8.35
and 6.78 u/L respectively; chest x ray was normal; skull x ray showed multiple osteolytic lesions at
parietal bone and mastoid region. Skeletal survey showed another osteolytic lesion at right scapula.
Urine output was 3.5 cc/kg/hr and the first morning urine specific gravity was 1.023. Wright stain of
pus smear from skin leions at forehead showed numerous mononuclear cells with fine chromatin
and prominent blue nucleoli compatible with histiocytes.
 
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skullfilm02.jpg (41103 bytes)
 
    Biopsy specimens of skin at back and gingiva revealed mononuclear cells, immunohistochemistry
investigation showed that these cells were s-100 positive compatible with the diagnosis of
Langerhans cell histiocytosis.
   
    The patient was treated with chemotherapy with vinblastine, methotrexate, cyclophosphamide and prednisolone with improvement of his skin and gum lesions in the first six weeks, the nail lesions
improved slower. His liver function test also showed improvement.  
 

Further readings:
1.
 Kulapong P, ed. Histiocytosis. In: Clinical Pediatric Oncology. Chiangmai: Department of
Pediatrics, Faculty of Medicine, Chiangmai University, 1997: 177-96.

2.

 Arico M, Egeler RM. Clinical aspects of Langerhans cell histiocytosis. Hematology/Oncology Clinics of North America 1998;12: 247-58.

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