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Hand-Schuller-Christian syndrome (lipoid histiocytosis)
Multifocal, unisystem Langerhans cell histiocytosis; several sites in one organ system, usually bone. Hand-Schuller-Christian syndrome is the triad of:
1) lytic skull lesions
2) proptosis
3) diabetes insipidus
Clinical manifestations:
1) swelling & necrosis of gums & loose teeth with infiltration of maxilla &/or mandible
2) skin lesions
a) papular, seborrheic, purpuric or petechial rash
b) small xanthomatous nodules
c) raised yellow to brown lesions in axillae or neck
d) lesions may become necrotic
e) intertriginous lesions may be exudative, become infected & ulcerate
f) papulosquamous lesions on scalp, face, trunk, abdomen & buttocks
g) moist scalp & intertriginous lesions have an offensive odor
3) erosive lesions are exudative, pruritic or painful, with poor response to local treatment
4) anogenital lesions
5) bony lesions occur in the calvarium, sphenoid bone, sella turcica, mandible, long bones of extremities & vertebrae
6) otitis media +/- aural discharge resulting from destruction of mastoid &/or temporal bones
7) ocular proptosis/exophthalmos due to orbital masses
8) pituitary dysfunction with involvement of sella turcica
a) growth retardation
b) delayed sexual development
c) diabetes insipidus
9) chronic cough & pneumothorax with lung involvement
Laboratory:
1) complete blood count (CBC): anemia
2) skin biopsy: fat & cholesterol in skin lesions
Radiology:
1) plain radiographs: sharply demarcated bony defects
2) chest X-ray: pulmonary fibrosis
3) bone scan to assess extent of osseous involvement
Management:
1) prognosis:
a) spontaneous remissions occur
b) prognosis poorer with lung involvement
2) standard treatment of diabetes insipidus & growth hormone deficiency
3) low-dose (300-600 rad) radiotherapy to bony lesions
4) pharmaceutical agents:
a) corticosteroids
b) vinblastine
c) mercaptopurine
d) methotrexate
e) agents a-d ujsed alone or in combination with epipodophyllotoxin (etoposide) for treatment of diabetes insipidus
5) cutaneous lesions
a) topical corticosteroids (discrete cutaneous lesions)
b) PUVA
c) nitrogen mustard
General
Langerhans cell histiocytosis (LCH); histiocytosis X
References
- DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 891
- Color Atlas & Synopsis of Clinical Dermatology, Common
& Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY,
1997, pg 556-561