Langerhans cell histipcytosis

Question 1

Age of onset

Gender

Answer 1

Unifocal LCH. Mostcommonly, childhood and early adulthood.

Multifocal unisystem LCH. Most commonly, childhood.

MultifocalmultisystemLCH (previously Letterer-Siwe disease). Mostly seen in children under 2years of age.

PulmonaryLCH. Mostly in cigarette smokers. The triad of diabetes insipidus, exophthalmos and lytic bone lesions was previously referred to as Hand-Schuller Christian disease

Males

Question 2

clinical manifestations

Unifocal and multifocal.

Answer 2

UNIFOCAL LCH Systemic symptoms uncommon. Pain and/or swelling over underlying bony lesion. Disruption of teeth with mandibular disease,fracture,and otitis media due to mastoid involvement.

MULTIFOCAL LCH Erosive skin lesions are exuda- tive,pruritic,or painful and may have an offensive odor. Otitis media caused by destruction of temporal and mastoid bones, proptosis

Question 3

Histopath

Answer 3

Proliferation of Langerhans cells with abundant pale eosinophilic cyto- plasm and indistinct cellborders;a folded, indented, kidney-shaped nucleus with finely dispersed chromatin; epidermotropism.

S-100 protein, CD1a, and CD207 (Langerin)

Question 4

treatment

Answer 4

UNIFOCALLCH Curettage with or without bony chip packing. Low-dose (300 to 600 rad) radio- therapy. Intralesional corticosteroids. Extraos- seousso -tissuelesions:Surgicalexcisionor low-dose radiotherapy.
MULTIFOCALLCH Diabetes insipidus and growth retardation treated with vasopressin and human growth hormone. Low-dose radiother- apy to bony lesions. Systemic treatment with glucocorticoids and/or vinblastine, given as single agents or in combination and etoposide. Nonresponders: Polychemotherapy (vincristine and cytarabine and prednisone or vincristine and doxorubicin and prednisone), cladribine (2-chlorodeoxyadenosine). Bone marrow transplantation is an option. CUTANEOUSLESIONS Glucocorticoids or discrete cutaneous lesions