A 38-year old woman with a dural based lesion.

[erdheim-chester disease]

Isolated intracranial xanthogranulomas arising from the dura mater are extremely rare .We present a case of a symptomatic large right frontoparietal dura based intracranial xanthogranuloma in a 38 -year -old female . Xanthogranulomas are benign non-Langerhans cell histiocytic lesions . They are frequently described in the skin of infants and children . Extracutaneous manifestations especially in the central nervous system are highly uncommon . Dural xanthogranulomas usually arise in association with familial hypercholesterolemia , with Erdheim Chester disease (ECD ), and with Weber-Christian disease . Our case however , had no such associations . In this report , the authors describe the clinical , radiological and microscopic presentation of this case and the differential diagnoses of intracranial xanthogranuloma .

Diseases
Validation

Diseases presenting "central nervous system" symptom

22q11.2 deletion syndrome

adrenomyeloneuropathy

alexander disease

aniridia

aromatase deficiency

canavan disease

child syndrome

classical phenylketonuria

congenital toxoplasmosis

cowden syndrome

cushing syndrome

cystinuria

dracunculiasis

erdheim-chester disease

fabry disease

gm1 gangliosidosis

hereditary cerebral hemorrhage with amyloidosis

hirschsprung disease

hodgkin lymphoma, classical

kabuki syndrome

kallmann syndrome

kindler syndrome

krabbe disease

lamellar ichthyosis

legionellosis

liposarcoma

malignant atrophic papulosis

monosomy 21

neonatal adrenoleukodystrophy

phenylketonuria

proteus syndrome

scrub typhus

severe combined immunodeficiency

sneddon syndrome

triple a syndrome

von hippel-lindau disease

waldenström macroglobulinemia

well-differentiated liposarcoma

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

zellweger syndrome

You can validate or delete this automatically detected symptom