Langerhans cells histiocytosis
Introduction
Langerhans cell histiocytosis is a rare condition affecting children and occasionally adults too. A langerhans cell is an immune cell, that usually helps body fight infection. In LCH more than the normal number of langerhans cells are present in different body parts such as the skin, bone and kidneys and these can then cause damage to that body part. It is still unclear whether this condition is an immune disorder or a type of cancer.
It has a wide range of presentations from a mild disease just affecting the skin to a severe multisystem disease affecting many organs (e.g. lymph nodes, bone, lungs, thymus, brain, liver, spleen). It is usually classified into 2 groups:
Single system Langerhans cell histiocytosis
Multisystem Langerhans cell histiocytosis
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Vesicles and red papules on the abdomen and groin of a child with langerhans cell histiocytosis (LCH)
Who gets it?
LCH is a rare disorder reported to occur in 1 in every 200 000 children. It can occur in children of all ages but is most common in children 1-3 years old and seems to occur more in boys than girls.
What causes it?
The cause of LCH is not well understood. There is debate amongst experts whether this is a cancer or an immune disorder. Recent studies have found a genetic mutation in children that develop this condtion supporting the theory that this is a cancerous process.
What does it look and feel like?
This will depend on your child’s disease and what organs are involved. It tends to mainly affect the bones, skin and lymph nodes. Other less common sites include liver, spleen and lungs, brain and spinal cord.
Looks like…
In babies - Scaly and lumpy rash on scalp, skin folds, or body (can look like seborrhoeic dermatitis or “cradle cap” or fungal infection)
One or multiple brown-purple lumps
Red rash (which can look like eczema or psoriasis) on the body or in skin folds or in the nappy area
Small fluid-filled bumps which can disappear
Red gums with mouth ulcers
Children may fail to grow normally or be late reaching puberty
Feels like…
Symptoms of the condition will depend on your child’s disease. If there is multisystem LCH your child may:
Feel tired
Have bone pain or swelling
Feel very thirsty and urinate a lot
Feel short of breath or develop a cough
What tests might be needed?
Your dermatologist or specialist will decide how many tests your child needs but these are some they might need:
Blood tests (including blood count, kidney and liver function)
X-rays (including skull, chest x-ray, or full body x-ray for skeletal survey)
Ultrasound (liver and spleen)
Biopsy (skin, lymph node or bone)
MRI brain
Lung function tests
What treatments are available?
What can I do?
It is important to attend a specialist appointment for your child and if you notice any changes in your child report these to the doctor as they could be a sign of the disease progressing.
When the skin is affected regular moisturisers might be sufficient to manage very mild skin disease.
Medical therapies
Depending of the body systems affected, your child's specialists may recommend various treatment options. These could include treatments that help treat cancer such as radiation and chemotherapy.
For skin disease:
Sometimes just close monitoring by your dermatologist is sufficient
Creams - these are usually immune modifying creams which target skin disease e.g. these include corticosteroids, nitrogen mustard, imiquimod
Oral medications e.g. methotrexate, mercaptopurine, hydroxyurea, thalidomide, azathioprine, isotretinoin, acitretin
Phototherapy - UV light treatment to target the skin immune cells
If your child has multisystem LCH, they may require multiple different treatments, specific to the organs involved including chemotherapy (e.g. vinblastine & prednisone) but this will be decided by your child’s haematologist or oncologist.
What happens after treatment?
This will depend on your individual child and whether their disease is low risk or high risk. Some children have only skin disease and it self resolves. Some children require treatment for their skin disease. Other children have multi-system disease which requires a lot of investigation and treatments by a team of doctors including dermatologists, orthopaedic surgeons and haematologists or oncologists. Ask your specialist for a better indication about your child’s disease.
What support is available?
Your GP will refer you to a specialist (if it is involving the skin), likely a dermatologist. If your child’s Langerhans cell histiocytosis involves many organs they may need to be referred to a haematologist or oncologist or other specialist depending on the organ involved e.g. paediatrician, orthopaedic surgeon, endocrinologist, respiratory physician, etc.
Support for families is available from Histiocytosis Association, a global organization dedicated to addressing the unique needs of patients and families dealing with the effects of histiocytic disorders such as LCH.
https://histio.org/histiocytic-disorders/langerhans-cell-histiocytosis-in-children/
Authors/Reviewers:
Author: Dr Chelsea Jones
Paediatric Reviewer: Dr Mayuri Sivagnanam
Editor in Chief: Dr Tevi Wain
