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380 Think out of the box! lymphadenopathy, skin rashes and skull lesions; all ended up with langerhans cell histiocytosis ?
  1. Lay Chin Tan,
  2. Siew Sing Chua,
  3. Fong Chiao Lai,
  4. Norsarwany Mohammad,
  5. Chin Hong Ding,
  6. Noraini ab rahman
  1. Malaysia


Background Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a rare paediatric disorder. The abnormal proliferation of specialized dendritic cells found in skin and mucosa, which resembles langerhans cells, account for the underlying pathology of LCH (1). The vast clinical presentations of LCH, from self-limiting lesion to fulminant disseminated disease, make its diagnosis a challenge for doctors. Its incidence stands at around 1–5 per million children. Some literatures showed that children of parents who were exposed to certain chemicals such as benzene have an increased risk for developing LCH. We presented 3 cases, 2 of whom aged 6 months and below.

Objectives To provide a wider perspective in diagnosing LCH.

Methods We are presenting 3 case reports, 2 of whom aged 6 months and below.

Results 1st case: Inguinal swelling mimicking inguinal abscess

This was a case report of a 6-month-old girl who presented with multiple lymphadenopathies over the right inguinal, axillary and cervical region. She was otherwise asymptomatic. A detailed history revealed presence of the right inguinal swelling noted since 2 weeks old and progressively increasing in size. Detailed investigation for infection were all normal. She received few courses of antibiotics but symptoms persisted. The father works in road construction industry. Excision biopsy of the right inguinal lymph node showed positive staining of CD1a, confirming the diagnosis of LCH.

2nd case: Skin rashes mimicking eczematous rashes

This was a 5-month-old girl who was admitted for fever with petechiae rashes, while the blood investigations revealed pancytopenia. 3 weeks prior to admission, she developed skin rashes mimicking eczema over the genitalia and bilateral inguinal. Systemic examinations showed hepatosplenomegaly, ascites with multiple petechiae rashes over the groin and neck region. Full blood picture reported pancytopenia with leucoerythroblastic blood film and circulating blasts. Her mother works in company selling household cleaning detergent. Skin biopsy of the inguinal region confirmed the diagnosis of LCH.

3rd case: Scalp swelling mimicking scalp abscess

This was a 2-year-5-month old ‘Orang Asli’ (native people), whose stays in rubber estate in a remote area (regular exposure to pesticides), presented with multiple scalp swellings. Other systemic examination was unremarkable. Blood investigations and septic parameters were normal. Computed tomography (CT) brain showed multiple soft tissue mass with osteolytic bony destruction, involving outer and inner table of occipital, left parietal and left frontal bone. Excision biopsy of the scalp confirmed the diagnosis of LCH.

Conclusions LCH has a wide spectrum of clinical manifestations. Although it is a very rare disease, LCH should always be considered as differential diagnosis in children with generalised lymphadenopathy, scalp swelling or any disorder with multisystemic organs involvement. A thorough history to identify risk, physical examination and laboratory investigation are vital to aid in diagnosing patient.

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