Langerhans’ cell histiocytosis

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Langerhans’ cell histiocytosis

A group of conditions characterised by proliferation of Langerhans cells, which are lymphoreticular cells. Langerhans cell aggregates are nodular, especially in the lungs, and are variably accompanied by eosinophils, foamy cells, neutrophils, and fibrosis.

Prognosis
Good if limited to a single system. Multi-system involvement carries a 10–20% mortality rate due to organ failure; 50–60% have chronic disease; 30–40% have long-term clinical remission.

Langerhans cell histiocytosis types 
Pulmonary LCH
A lesion virtually exclusive to cigarette smokers, which is a form of smoking-related interstitial lung disease. Smoking cessation may lead to reversal of changes or evolution to pulmonary fibrosis and pulmonary hypertension.
 
Unifocal LCH
Eosinophilic granuloma, solitary bone involvement
A lesion affecting younger patients; may affect any bone, most commonly the cranial vault, jaw, humerus, rib and femur (often spares the hands and feet).

Imaging
Mimics Ewing sarcoma.
 
Treatment
Curettage.
 
Prognosis
Excellent.
 
Multifocal unisystem
LCH Hand-Schüller-Christian disease, multiple bone involvement.

Polyostotic eosinophilic granuloma 
A lesion that may affect the skin, accompanied by proptosis, diabetes insipidus, or chronic otitis media or combination thereof, marked by a chronic course with waxing and waning symptoms.

Prognosis
Relatively good.
 
Multifocal multisystem LCH
Letterer-Siwe disease, multiple organ involvement
A lesion that affects bone, lung and skin; while histologically indistinct, it is more aggressive than the other forms.

Prognosis
Poor if < 18 months at time of diagnosis; haemorrhagic skin lesions; hepatomegaly, anemia; thrombocytopenia; bone marrow involvement.
References in periodicals archive ?
LCH consists of a constellation of clinical presentations, which include Letterer-Siwe syndrome, a multisystem disease of young children characterized by cutaneous lesions containing infiltrates of Langerhans cells and a poor prognosis; eosinophilic granuloma, a solitary bone lesion; and Hand-Schuller-Christian disease that manifests as a characteristic triad of cranial bone lesions, diabetes insipidus, and exophthalmos (2).
Acute disseminated form is known as the Letterer-Siwe syndrome and chronic multifocal form is known as the Hand-Schuller Christian syndrome.
Hand-Schuller-Christian Disease and Letterer-Siwe Disease are more global and life-threatening diseases.
Three clinical forms of LCH have been identified ranging from localized LCH (eosinophilic granuloma), chronic recurring LCH (Hand-Schuller-Christian disease), and acute disseminated LCH (Letterer-Siwe disease).
Previously classified into three different clinical entities including eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease, the current classification of LCH is made according to dissemination of the disease: single-system and multisystem LCH [2, 5].
Vesiculopustular disorders of neonates are common; HSV infection, herpes zoster, congenital syphilis, neonatal acne, staphylococcal infections, bullous impetigo, epidermolysis bullosa simplex, Letterer-Siwe disease, transient pustular melanosis, neonatal dermatitis herpetiformis, and IP all have vesiculopustular cutaneous manifestations.
This group is comprised of three diseases, eosinophilic granuloma, Hand-Schilller-Christian disease, and Letterer-Siwe disease, which describe progressively more aggressive and widespread manifestations of the same underlying pathology.
Letterer-Siwe Disease is an association of cutaneous, systemic and bone lesions.
(1) The broad clinical spectrum that is encompassed by LCH is reflected in the many synonyms for this disease, which include eosinophilic granuloma (unifocal LCH), Hand-Shuller-Christian disease (multifocal unisystem LCH), and Letterer-Siwe disease (disseminated multifocal multisystem LCH).
The disease spectrum includes many pathological conditions, ranging from multisystem disease (Letterer-Siwe and Hand-Schuller-Christian disease) to a single-system disease confined to the skeleton (eosinophilic granuloma-EG).
[1-3] Letterer-Siwe disease is an acute disseminated and rapidly progressive form of LCH that is mostly seen in infants.