Report a rare hemifacial atrophy-romberg disease

ABSTRACT

Romberg disease is a rare progressive disease with hemifacial atrophy of skin, soft tissue and bone. It was first described by parry in 1825 and later in 1846 by Romberg and in 1871 named as a progressive hemifacial atrophy by Eulenbery. First signs of this rare disease usually appear before the age of 20 and initially skin and subcutaneous parts are involved and later, there is muscle and bone involvement. In 95% of cases there is unilateral atrophy. Different theories are mentioned about the exact cause of this rare disease such as Rubeolla infection, trigeminal neuralgia, sclerodermia, cervical sympathetic anomaly, but the exact cause is not known yet. Progression of disease usually lasts between 2 to 10 years, but in some cases it takes longer. Treatment includes skeletal and soft tissue reconstruction, although in most of cases soft tissue rehabilitation is enough. The patient is a 20 year old woman who was suffering from atrophy of soft tissue and skull in the right frontal and parietal areas plus loss of eyebrow for the past 9 years. In biopsy skin and adnexal atrophyed was identified. The progression of disease ceased after 2 years and reached a steady state. The patient was a scheduled for tissue expander insertion in scalp area [left side] to expand hair full area and flap rotation and covering hairless area of scalp [right side]. In 2 steps operation minimal surgical scar with satisfaction of patient was achieved and this way may be an effective way for limited form of this disease