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Differential Diagnosis

Author(s): Euro Histio Net Work Group for LCH Guidelines (see introduction page), Created: 2011/03/10, last update: 2012/01/23

Sometimes making the diagnosis of LCH may be difficult due to other diseases that may present with similar findings.

Table 6 shows a list of differential diagnoses for the different manifestations of Langerhans Cell Histiocytosis.


Tab. 6. Differential Diagnosis for Manifestations of Langerhans Cell Histiocytosis


Involvement	Manifestation	Possible other condition
Bone	Vertebra plana	Ewing sarcoma Septic osteomyelitis Chronic relapsing multifocal osteomyelitis (CRMO) Leukemia Lymphoma Aneurysmal bone cyst Juvenile xanthogranuloma Myeloma (only described in adults) Osteoporosis
	Temporal bone	Chronic otitis media Mastoiditis Cholesteatoma Soft tissue sarcoma
	Orbit	Acute infection (preseptal cellulitis) Dermoid cyst Rhabdomyosarcoma Neuroblastoma Erdheim-Chester disease Pseudoinflammatory tumor
	Other lytic lesions of the long bones	Osteomyelitis Chronic recurrent multifocal osteomyelitis (CRMO) Aneurysmal bone cyst Bone angiomatosis (Gorham disease) Fibrous dysplasia Atypical mycobacterial infection Osteogenic sarcoma Ewing’s sarcoma
Skin	Vesicles and bullae (most common in early infancy)	Erythema toxicum Herpes simplex Chickenpox infection
	Dermatitis (most frequently scalp, diaper or axilla, may occur up to late infancy)	Seborrheic dermatitis (eczema; usually no petechiae and marked scaling)
	Nodules	Mastocytosis Juvenile xanthogranuloma Hashimoto-Pritzker’s self-healing reticulohistiocytosis Neuroblastoma Infant leukemia
	Pruritus (more common), Petechiae (uncommon)	Scabies (other family members may be affected)
Lung	In particular systemic symptoms and cavitated pulmonary nodules	Pneumocystis Carinii cavitated infection Mycobacterial or other pulmonary infections Sarcoidosis Bronchiolar-alveolar carcinoma (only described in adults) Lymphangio-Leiomyosarcoma (only described in young adult women) Septic emboli
Liver	Jaundice with direct hyperbilirubinemia Hypo albuminemia	Chronic destructive cholangitis Metabolic disease Hepatitis Neoplasia obstructing biliary tract Inherited deficient conjugation of bilirubin Toxic (Reye syndrome) Chronic inflammatory bowel disease Neonatal hemochromatosis