Buschke–Ollendorff syndrome

Buschke–Ollendorff sign
Other namesDermatofibrosis lenticularis disseminata
Buschke–Ollendorff syndrome has an autosomal dominant pattern of inheritance.
SymptomsPapules in skin
CausesMutations in the LEMD3 gene.
Diagnostic methodX-ray, ultrasound
TreatmentSurgery for hearing loss(or complications)

Buschke–Ollendorff syndrome (BOS) is a rare genetic skin disorder associated with LEMD3 that typically presents with widespread painless papules.

It is inherited in an autosomal dominant manner. Conditions that may appear similar include tuberous sclerosis, pseudoxanthoma elasticum, neurofibroma, and lipoma, among others.

Its frequency is almost 1 case per every 20,000 people, and it is equally found in both males and females. It is named for Abraham Buschke and Helene Ollendorff Curth, who described the condition in one female in 1928.