Langerhans cell histiocytosis in Adult
LCH is a rare condition, occurring most commonly in children aged 1-4 years, but it may present from birth to adulthood. The 3-year survival rate is approximately 80%, with age younger than 2 years, multiorgan involvement, and organ dysfunction portending a worse prognosis.
While 4 clinical variants were previously designated (Letterer-Siwe disease, Hand-Schüller-Christian syndrome, self-healing reticulohistiocytosis of Hashimoto-Pritzker [congenital self-healing histiocytosis], and eosinophilic granuloma), these categories have been largely abandoned in recent years due to significant overlap between the clinical entities and lack of prognostic relevance. Currently, patients are classified based on the number of organ systems involved (ie, single-system or multisystem LCH).
LCH can affect any organ of the body, with bones, skin, oral mucosa, bone marrow, lungs, liver, spleen, gastrointestinal tract, lymph nodes, and the central nervous system being the most common sites of involvement. The skin is affected in approximately 33% of LCH cases. The spleen, bone marrow, and liver are considered high-risk organs. Involvement of one or more of these organs is associated with a higher risk of dying from the disease. Although the lung was previously considered a high-risk organ, it is now known to be a rare cause of death. Skull bone lesions are considered to be a risk factor for central nervous system involvement. These patients may have a higher risk of developing diabetes insipidus and/or neurodegenerative complications.
LCH may recur after resolution and has an unpredictable course. LCH has further been associated with the development of leukemias and lymphomas. Additionally, disease sequelae, including endocrine (diabetes insipidus, growth delay), skeletal (scoliosis, abnormal dental development, hearing dysfunction), neuropsychological (cerebellar ataxia, learning disabilities), pulmonary, and hepatic (sclerosing cholangitis, cirrhosis) involvement can present up to decades after diagnosis.
LCH often presents in the first year of life as a rash in the diaper area. Other findings may include lymphadenopathy, diffuse lung involvement, osteolytic involvement of the mastoid presenting with a picture of otitis media, and gastrointestinal involvement. Failure to thrive may be due to malabsorption. There is wide variation in the clinical spectrum. The congenital form presents with papules, nodules, or ulcers at birth; nodules may be solitary or few.
LCH is even rarer in adults than children. The skin, bone, and lungs are most frequently involved. Systemic and progressive forms are rare.
For more information, see OMIM.
C96.5 – Multifocal and unisystemic Langerhans-cell histiocytosis
C96.6 – Unifocal Langerhans-cell histiocytosis
65399007 – Langerhans cell histiocytosis
- Erdheim-Chester disease
- Rosai-Dorfman disease
- Juvenile xanthogranuloma or adult xanthogranuloma
- Necrobiotic xanthogranuloma
- Multiple myeloma
- Primary tumors
- Interstitial lung disease
- Seborrheic dermatitis usually lacks distinct papules. The presence of eroded, ulcerated, or purpuric lesions in a child thought to have seborrheic dermatitis should prompt a biopsy to rule out LCH.
- Psoriasis – Also erythematous and scaly, but not papular or accompanied by systemic symptoms, and usually there is a family history of psoriasis.
- Benign cephalic histiocytosis – Occurs between 2 months and 2 years; on the face and other portions of the head. It is caused by a non-Langerhans cell histiocytosis. Lesions regress spontaneously.
- Scabies may resemble LCH clinically and histologically. Inquire about potentially affected family members.
- Atopic dermatitis is much more pruritic and rapidly responds to topical steroids.
- Diaper dermatitis is localized to the diaper area and responds to gentle skin care and topical steroids.
Last Updated: 09/19/2017