Causes and risk factors
The exact cause of Letterer-Siwe disease is not known. It is believed to be due to an autoimmune reaction [(immune system over produces histiocytes that attacks body’s healthy tissue]).
Clinical presentation
The signs and symptoms present itself depending on the system or organ infiltrated by histiocytes. It may present with lung infiltrate, skin manifestation, bone lesions, ; endocrine, hepatic, hematopoetic symptoms. Patient presents with symptoms like lethargy, unintentional weight loss. There is slow growth, delayed puberty. Skin lesions are seen. Scaly, eczematous, purpural rash over scalp, ear, abdomen can be observed. Broken skin may lead to infection and further to sepsis. Painful bone swelling, swollen gums, loose teeth are observed. Generalized rash with fever occurs. There can be anaemia. There may be swollen lymph nodes [(lymphadenopathy]). Hepatomegaly can occur. There may be trouble in breathing. There may be cough, tachypnea, . Pneumothorax can occur. Diabetes insipidus may occur.
Investigations
Medical history by the patient and clinical examination by the doctor helps in diagnosis. Complete blood count is estimated. Liver function test is required. Bone marrow aspiration and biopsy is recommended.
Treatment
Treatment depends upon the extent of disease. Treatment includes steroids, immune suppressing medications. Chemotherapy may be required.
Complications
Complications such as diabetes insipidus, hypothyroidism occur.
When to Contact a Doctor
One must consult a doctor if patient presents with symptoms such as unintended weight loss, lethargy, etc.
Prevention
There is no sure way to prevent Letterer-Siwe disease.
Facts and figures
The disease occurs in 1: among 50,000 to 2, 00,000.
Systems involved
Circulatory system, immune system, endocrine system, integumentary system.
Organs involved
Bones, teeth, skin
