One of the acute variant of Langerhans cell histiocytosis i.e. Eosinophilic Granuloma is Abt Letterer Siwe disease or syndrome. It is a rare and fatal condition in which there is Acute, disseminated and rapidly progressive form of Langerhans-cell histiocytosis is found. Langerhans cell histiocytosis is characterized by proliferation and accumulation of a specific type of white blood cell known as histiocyte in the various tissues and organs of the body leading to various lesions which if contains some granules are termed as Langerhans Cells.

There is proliferation of nonlipid histiocytes in the viscera and bones.It is most commonly seen in children less than two years old. The five-year survival rate with treatment is fifty percent. If untreated the disease is fatal. Disease may affect the adults.The etiology of the disease is unknown.

Signs and symptoms

• Yellowish brown skin lesions

• Maculopapular lesions on skin
• Skin folds eroding
• Bruises
• Anemia
• Edema
• Lymphadenopathy
• Tooth problems
• Tooth decay
• Gum necrosis
• Fatigue
• Anorexia
• Irritability
• Wasting
• Persistent fever
• Palpable Liver

• Palpable Spleen

• Palpable Lymph nodes

Complications

• Lymphadenopathy
• Bone metastases
• Neutropenia
• Suppurative otitis media
• Splenomegaly
• Breathlessness
• Pulmonary fibrosis
• Bone cyst
• Hepatomegaly
• Mastoiditis
• Pneumothorax
• Periosteal reaction
• Hepatocellular jaundice
• Hypopituitarism
• Exophthalmos
• Decreased red cell production
• Short stature
• Loss of tooth

• Mass in orbit

• Mouth ulcers

Diagnosis and investigations

• X- Ray
• Computed Tomography Scans

• Magnetic Resonance Imaging Scans

• Bone Biopsy

Treatment and Drugs

• Antibiotics depending on case
• Steroids like Prednisolone
• Antimetabolite drugs like vinbastine and vincristine