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Langerhans cell histiocytosis (LCH); histiocytosis X
A group of disorders characterized histologically by proliferation & infiltration of Langerhans histiocytes that fuse into multinucleated giant cells & form granulomas with eosinophils.
Classification:
1) eosinophilic granuloma (unifocal LCH)
2) Hand-Schuller-Christian syndrome (multifocal LCH)
3) Letterer-Siwe syndrome (most aggressive form)
4) pulmonary Langerhans cell granulomatosis
Staging:
1) localized disease
- bone (1 or 2 adjacent lesions)
- lymph node
- skin
2) disseminated disease
- bone, multifocal
- bone & soft tissue or soft tissues alone (except skin & isolated lymph node)
- organ dysfunction (liver, lungs, hematopoietic system)
Etiology:
- abnormal proliferation of histiocytes
Epidemiology:
1) most commonly occurs in children (toddlers) [3]
2) males > females
3) incidence: rare
Pathology:
1) proliferation of Langerhans cells
a) abundant, pale eosinophilic cytoplasm with indistinct cell borders
b) folded, indented, kidney-shaped nucleus, with finely dispersed chromatin
2) stimulation for proliferation of Langerhans cells unknown
Clinical manifestations:
1) lytic bony lesions
2) cutaneous abnormalities
a) scalp, chest, upper back [3]
b) soft tissue swelling
c) eczema
d) seborrheic dermatitis-like changes
e) erythematous papules & nodules with purpura, crusting & ulceration, necrosis
3) lymphadenopathy
- posterior cervical, submandibular, supraclavicular [3]
4) serous drainage from ears [3]
Laboratory:
- immunohistochemistry (biopsy: skin, bone, soft tissue)
1) diagnosis (2 or more)
a) ATPase+
b) S-100 protein+
c) alpha-D-mannosidase
d) peanut agglutinin+
2) definite diagnosis
a) CD1a+
b) Birbeck granules
- complete blood count may show anemia of chronic inflammation
Management:
- prednisone, vinblastine, & mercaptopurine [3]
Related
eosinophilia
vinca alkaloid
Specific
eosinophilic granuloma
Hand-Schuller-Christian syndrome (lipoid histiocytosis)
Letterer-Siwe syndrome
pulmonary Langerhans cell granulomatosis
General
granulomatous disease
histiocytic neoplasm
histiocytosis
References
- Color Atlas & Synopsis of Clinical Dermatology, Common
& Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY,
1997, pg 556-561
- Medical Knowledge Self Assessment Program (MKSAP) 14,
American College of Physicians, Philadelphia 2006
- Mehta B, Venkatramani R
Langerhans'-Cell Histiocytosis
N Engl J Med 2014; 371:1050September 11, 2014
PMID: 25207768
http://www.nejm.org/doi/full/10.1056/NEJMicm1312532
- Allen CE, Merad M, McClain KL
Langerhans-Cell Histiocytosis.
N Engl J Med 2018; 379:856-868. Aug 30, 2018
PMID: 30157397
https://www.nejm.org/doi/full/10.1056/NEJMra1607548