Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of the skin followed by unilateral craniofacial atrophy of su bcutaneous tissues, including fat, muscle and bone. Hereditary factors have been postulated to be involved in the aetiology of PFH. Yet, the occurrence of PFH i n one of two identicalmale twins reported heremakes this possibility unlikely. P FH usually occurs in the first two decades of life, and the clinical presentatio n resembles linear scleroderma. PFH may be complicated by autoimmune, neurologic al, ocular and dental disorders. Management of PFH comprises a long term follow -up of somatic disorders, and prevention of psychological problems. Treatment o f PFH is symptomatic and consists of plastic surgery after the disease activity has stopped. Conclusion: The occurrence of PFH in one of a monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosi s of PFH and accurate follow-up is essential to disclose the occurrence of comp lications.
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