eosinophilic granuloma

Unifocal Langerhans' cell histiocytosis; single site of involvement. Pathology: 1) single osteolytic bony lesion is most common 2) skin & soft tissue lesions Clinical manifestations: 1) pain &/or swelling over underlying bony lesion 2) cutaneous or subcutaneous nodule, generally yellow; may be tender & breakdown; generalized distribution 2) disruption of teeth with mandibular disease 3) fracture 4) otitis media due to mastoid involvement 5) sharply marginated, recalcitrant ulcer on oral mucosa or genital region (necrotic, draining, tender) 6) lesions often asymptomatic Radiology: -> plain radiographs: -> osteolytic lesion -> adults: most commonly rib -> children: most commonly calvarium, femur Management: 1) prognosis: benign course with good prognosis for spontaneous recovery 2) curettage with or without bony chip packing 3) low-dose (300-600 rad) radiotherapy 4) surgical excision of extraosseous soft tissue lesions

References

Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 556-561