All are true about Langerhans’s Histiocytosis except? (AIIMS May 2009)
|A||Common before 3 years of age|
|B||Letterer Siwe disease is systemic manifestation|
|D||Testis is commonly involved|
Testis is commonly involved
1. Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow–derived Langerhans cells (LCs) and mature eosinophils.
2. LCH encompasses a number of diseases.
a. Letterer-Siwe disease →An acute fulminant, disseminated disease (Option B)
b. Eosinophilic granulomas → Solitary or few, indolent and chronic, lesions of bone or other organs.
c. Hand-Schüller-Christian disease →The intermediate clinical form classically presents as the triad of diabetes insipidus, proptosis, and lytic bone lesions.
d. Hashimoto-Pritzker disease →A congenital self-healing form has also been described.
3. Age of onset
a. Letterer-Siwedisease occurs predominantly in children younger than 2 years. (Option A)
b. Hand-Schüller-Christian syndrome, has a peak of onset in children aged 2-10 years. (Option A)
c. Localized eosinophilic granuloma occurs mostly frequently in those aged 5-15 years.
4. Clinical presentation.
a. Unifocal LCH is characterized by solitary bony lesions at any site. It is least common in the hands and the feet. These lesions are often asymptomatic and are found as incidental findings during investigation for unrelated disorders.
b. Multifocal disease has a history depending on the location of osteolytic lesions and the degree of organ dysfunction.
c. Hand-Schüller-Christian syndrome often present with otitis media and mastoiditis or with polyuria and polydipsia.
d. Letterer-Siwe disease presents with symptoms suggestive of a systemic infection or malignancy with a generalized skin eruption, anemia, and hepatosplenomegaly.
e. The congenital form of LCH (Hashimoto-Pritzker disease) manifests as skin lesions at birth or during the early postnatal period.
i. Cutaneous nodules and ulceration occur early in life.
ii. Rarely, purpuric lesions occur with a blueberry muffin presentation.
iii. Symptoms of organ involvement may also occur.
a. Single-system disease
i. Solitary bone lesions are treated locally with curettage or excision, intralesional steroid injection (triamcinolone acetonide).
ii. Polyostotic bone lesions are best treated with indomethacin or a short course of systemic steroids.
iii. Radiation may be required in lesions that are large, painful & occur in inaccessible sites or involve vital structures. (Option C)
iv. Localized skin disease is best treated with moderate-to-potent topical steroids.
v. Severe cutaneous involvement, topical nitrogen mustard (20% solution) may be used.
vi. Psoralen plus ultraviolet A (PUVA) is another excellent treatment for cutaneous only LCH or for cutaneous disease in multisystemic disease.
vii. For single lymph node infiltration, excision is the treatment of choice.
b. Multisystem disease
i. Systemic chemotherapy is indicated.
ii. The combination of cytotoxic drugs and systemic steroids is effective. Low-to-moderate doses of methotrexate, prednisone, and vinblastine are used.