Bahemuka Brown Syndrome

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Synonyms

Bahemuka Brown syndrome, Bahemuka-Brown syndrome, Bahemuka-Brown综合征

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Introduction

A complex form of hereditary spastic paraplegia with characteristics of delay in motor development followed by a slowly progressive spastic paraplegia (affecting mainly lower extremities) associated with a desquamating facial rash with butterfly distribution (presenting at around two months of age) and dysarthria. There have been no further descriptions in the literature since 1982.

Pemphigus foliaceus is a superficial autoimmune blistering disorder of the skin. Localized forms of pemphigus foliaceus are rare. Only a few cases have been described. We report a case of an 81-year-old man with pemphigus foliaceus who initially presented with localized dermatomal facial lesions. Due to this atypical presentation, making the diagnosis was challenging. To our knowledge, this is the first case of localized pemphigus foliaceus with a dermatomal distribution to be recorded.

01 Feb 2022·Skeletal Radiology

Test Yourself-Question: Multiple facial skin lesions associated with gingival hypertrophy in a pair of siblings

Author: Boitsios, Grammatina ; Marroun, Nour ; Segers, Valérie ; Franck, Diane

Two brothers, completely asymptomatic until their first year of life, started to complain from gingival hypertrophy, progressive development of painful soft tissue masses on the fingers and toes, on the face and on the scalp. There were no neurological symptoms or mental delay for both brothers.

01 Feb 2022·Skeletal Radiology

Test yourself-answer: multiple facial skin lesions associated with gingival hypertrophy in a pair of siblings

Author: Boitsios, Grammatina ; Marroun, Nour ; Segers, Valérie ; Franck, Diane

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