Langerhans Cell Histiocytosis
| Figure 2.14
A: Juvenile xanthogranuloma
(Courtesy of Dr. Michelle
B. Bain)
B: Juvenile xanthogranuloma
(Courtesy of Dr. Paul Getz)
C: LCH
(Reprint from Morgan MB,
Smoller BR, Somach SC.
Deadly Dermatologic Diseases.
New York, NY: Springer;
2007) |
- Current classification by number of organ systems involved (single vs. multisystem), but historically grouped as follows:
| | | | | | | | Letterer–Siwe Disease | | – Multisystem involvement, (acute disseminated form); onset typically before 2 years of age
– Small, pink papules, pustules, vesicles with scale/crust/petechiae in seborrheic distribution | | | Hand–Schuller–Christian Disease | | – Onset between 2 and 6 years of age
– Typical triad: diabetes insipidus, bone lesions, exophthalmos
– Osteolytic bone lesions (cranium) | | | Eosinophilic Granuloma | | – Onset in older children, localized LCH variant
– Asymptomatic granulomatous lesions involving bone (cranium), spontaneous fractures | | | Congenital Self-Healing
Reticulohistiocytosis | | – Onset at birth or soon after, limited to skin; also known as Hashimoto-Pritzker disease
– Widespread, red-brown papulonodules
– Self-healing within weeks to months |
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