Buschke Ollendorff syndrome

Synonym(s)

Dermatofibrosis lenticularis; disseminated lenticular dermatofibrosis; dermatofibrosis lenticularis disseminata with osteopoikilo-

Definition

Ectodermal dysplasia of connective tissue, consisting of osteopoikilosis and connective tissue nevi

Pathogenesis

Possibly resulting from abnormal regulation of extracellular matrix, leading to increased accumulation of elastin in the dermis

Clinical manifestation

Asymptomatic, slightly elevated, yellowish papules and nodules coalescing to form plaques; arising over several years; oste-opoikilosis of the epiphysis and the meta-physis of long bones

Differential diagnosis

Pseudoxanthoma elasticum; tuberous sclerosis; connective tissue nevus; morphea

Therapy

Surgical excision of skin lesions for cosmetic purposes

References

Woodrow SL, Pope FM, Handfield-Jones SE (2001) The Buschke-Ollendorff syndrome presenting as familial elastic tissue naevi. British Journal of Dermatology 144(4^890-893

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