Background: Nijmegen Breakage Syndrome(NBS) is a rare autosomal
recessive disorder with specific clinical features, characteristic
chromosomal breakage and combined imunodeficiency. Patients with this
condition also associate growth retardation with microcephaly,
predisposition to malignancy and specific skin manifestations. Case
Presentation: Here we report a 3-year old girl known with NBS
associated with cutaneous sarcoid-like lesions. She presented with one
year history of squamous lesions on the face and upper and lower limbs.
The lesions were biopsied and histopatological examination revealed
nonnecrotizing epitheloid granulomas and raised the suspicion of a
sarcoid-like entity. Conclusion: The interest of this case will serve
to better understand clinical manifestations in a rare genetic entity.
Close follow-up is advised as cutaneous granulomas may be the first
manifestation of systemic granulomas