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Updated: Dec 29 2021

Langerhans Cell Histiocytosis

  • Snapshot
    • A 1-year-old boy is brought to the emergency room for a new rash in the diaper area along with fever. On physical exam, along the inguinal folds, there are pink eroded papules on background of petechiae and purpura, as well as pink greasy discrete papules on the scalp and trunk. A radiograph of the cranium shows punched out lytic lesions.
  • Introduction
    • Overview
      • Langerhan cell histiocytosis (LCH) describes a group of conditions caused by proliferation of dendritic (Langerhan) cells
        • traditionally classified by 4 subtypes, but more recent classification is based on extent of dissemination
    • Langerhan Cell Histiocytosis (LCH)

      Letterer-SiweHand-Schuller-ChristianHashimoto-Pritzker (Congenital Self-Healing Reticulohistiocytosis)
      Eosinophilic Granuloma
      • < 2 years of age
      • 2-6 years of age
      • Neonatal period
      • 7-12 years of age
      • Pulmonary involvement seen in ages 30-40 years
      • Skin, bone, and viscera
        • pulmonary infiltrates, lymph nodules, liver, and spleen
      • Skin, bone, and viscera
        • liver, spleen, and lymph nodes
      • Skin-limited
      • Primarily bone > skin
      • Pulmonary involvement
        • Poor prognosis with disseminated disease
        • Chronic and progressive
        • Rapid spontaneous resolution is likely
        • Good prognosis
    • Epidemiology
      • Incidence
        • rare
      • Demographics
        • males > females
      • Risk factor
        • smoking
          • pulmonary involvement
      • Pathogenesis
        • mechanism
          • proliferation of Langerhan cells, which are immature and are unable to stimulate T-cells via antigen presentation
            • thought to be reactive or neoplastic
          • BRAF V600E mutation can be found in LCH
    • Presentation
      • Clinical Presentation of LCH

        Letterer-SiweHand-Schuller-ChristianHashimoto-Pritzker (Congenital Self-Healing Reticulohistiocytosis)
        Eosinophilic Granuloma
        • Acutely disseminated lesions affecting the skin, bone, and internal organs
        • Fever
        • Bone pain
        • Triad of diabetes insipidus, osteolytic bone lesions (cranium), and exophthalmos
        • Skin lesions that crust and resolves spontaneously
        • Solitary indolent bone lesions (cranium most common) with spontaneous bone fractures
        Physical exam
        • Pink papules, pustules, vesicles, petechiae, purpura, and erosions on the scalp, flexural surfaces, and trunk
        • Secondary impetiginization common
        • Skin findings similar to Letterer-Siwe
        • Multipe red to brown papulonodules, some with erosions
        • Tenderness at site of bone lesions
      • Other symptoms
        • recurrent ear infections
        • dizziness
        • headache
        • limping (due to leg pain)
        • failure to thrive
        • developmental delay
    • Imaging
      • Bone radiograph
        • views
          • cranium or other skeletal survey
        • findings
          • punched out lytic lesions
      • Chest radiograph
        • findings
          • may show pulmonary nodules or infiltrate
    • Studies
      • Serum studies
        • liver function test
          • to evaluate for liver involvement
        • baseline complete blood count
      • Urine studies
        • to evaluate for diabetes insipidus
      • Pulmonary function test
        • to evaluate for pulmonary involvement
      • Histology
        • S100+, CD1a+, and Langerin+
        • proliferation of histiocytes with mixed inflammatory infiltrate
      • Electron microscopy
        • Birbeck granules or “tennis rackets
    • Differential
      • Multiple myeloma
        • key distinguishing factors
          • may also have “punched out” lytic bone lesions
          • typically affects older adults, and protein electrophoresis of the serum (SPEP) will reveal monoclonal gammopathy
    • Treatment
      • Medical
        • corticosteroids
          • indications
            • to slow autoimmune reaction
        • chemotherapy
          • indications
            • for severe cases in children
          • drugs
            • cyclophosphamide, etoposide, methotrexate, and vinblastine
        • radiation therapy
          • indications
            • bone lesions
        • vasopressin
          • indications
            • diabetes insipidus
        • bisphosphonates
          • indication
            • prevent bone destruction from bone lesion
      • Surgical
        • curettage or excision
          • indications
            • bone lesions
    • Complications
      • Bone fractures
      • Hypercalcemia (from lytic bone lesions)
      • Spontaneous pneumothorax
      • Infections
      • Hearing impairment
    • Prognosis
      • Worse with BRAF V600E mutation
      • Worse if there is failure to respond to 6 weeks of treatment
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