Letterer – Siwe Disease

  • Rare, acute aggressive disseminated proliferative lethal disease of Langerhans cells that affects infants <2 years old.
  • Accounts for 10% of cases of Langerhans cell histiocytosis

Clinical features:-

  • Initial manifestation often skin rash involving trunk, scalp & extremities.
  • Recurrent pyoderma – like lesions with crusting and scaling, vesicopustular and purpuric eruption; resembling seborrhoeic dermatitis.
  • Denuded skin may facilitate microbial invasion, leading to sepsis.
  • Persistent low grade spiking fever with malaise & irritability.
  • Splenomegaly, hepatomegaly & lymphadenopathy are early manifestations.
  • Nodular lesions in body flexures and intraoral haemorrhage.
  • Myelophthisic anemia , leukopenia and thrombocytopenia may also be seen.
  • Bone lesions are not common initially.
  • Diffuse involvement of skeletal system occurs later in the disease.
  • The uvea may be affected; orbital involvement is most unusual
  • The pustules are sterile and Tzanck smear of the vesicopustules shows pale histiocytes which can be used as a rapid screening test.

Oral manifestations:-

  • Ulcerative lesions, gingivitis, loose teeth, ectopic teeth also may be seen.
  • Parents frequently report precocious eruption of teeth, when in fact the gums are receding to expose immature dentition
  • Diffuse destruction of maxilla & mandible.
  • Some cases extreme rapid course of disease; oral involvement does not occur.

Histologic Features:-

1.Very similar to HSC; histiocytic proliferation with or without eosinophils.

2.These histiocytes do not contain significant amounts of cholesterol.

3.‘Foam cells’ not a feature.

Treatment & Prognosis:-

  • Extremely poor; Course of disease rapid & terminates fatally in short time.
  • Poor prognostic features include younger age; dissemination of lesions; involvement of lungs, liver, CNS and RE system; associated infection; anaemia, thrombocytopenia and purpura
  • Chemotherapy coupled with radiation to localised bony lesions and supportive measures are useful in treatment.
  • Thymic extracts have been successfully used to treat LS disease

References:-

Shafer’s 8th edition

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