BUSCHKE-OLLENDORFF SYNDROME

Multiple yellow dermal papules.
Buschke-Ollendorff syndrome


Buschke-Ollendorff syndrome, also known as dermatofibrosis lenticularis disseminata, is a rare autosomal dominant disease characterized by cutaneous connective tissue nevi (collagenomas or elastomas) and osteopoikilosis (sclerotic densities at the ends of long bones). Inheritance is AD and affected patients may have only skin or only bone lesions. Mutations in the LEM domain-containing protein 3 gene (LEMD3) are causative.


Clinical

Multiple yellow to flesh-colored papules distributed symmetrically on the thighs, buttocks, and elsewhere is characteristic. Onset is from birth to adulthood.

Treatment

There is no specific treatment for the skin lesions. Genetic counseling is important. A normal lifespan is expected.

Additional Pictures

Buschke-Ollendorff syndrome Buschke-Ollendorff syndrome

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