ReportClinical and serological characteristics of progressive facial hemiatrophy: A case series of 12 patients
Section snippets
Patients and methods
A retrospective chart review of all records of patients suffering from localized scleroderma (LS) at the Outpatient Clinic for Connective Tissue Diseases, Department of Dermatology, Ruhr-University Bochum, and a university-affiliated private clinic was performed. From 2000 to 2004, a total of 278 patients with LS, including 149 cases of morphea, 55 cases of linear scleroderma, 30 cases of LSCS, 21 cases of generalized LS, 11 cases of deep morphea, and 12 cases of PFH, had been treated in one of
Dermatologic features
The clinical data of all patients are summarized in Table I. PFH was accompanied by cutaneous induration in 7 patients (58%, group 1), whereas in 5 patients (42%) atrophy of subcutaneous structures was present without cutaneous sclerosis (Fig 1, Fig 2, group 2). In 5 patients (71% of group 1) cutaneous indurations appeared as a linear lesion of the scalp and forehead with underlying atrophy as seen in LSCS, and accompanying cicatricial alopecia of the affected scalp was observed in 4 of the
Discussion
The relationship between PFH and LSCS and their association with neurologic involvement has been discussed since the 19th century. In classic PFH, the entire side of the face is affected and atrophy mainly involves the subcutaneous soft tissue. However, the first case of PFH described by Romberg1 presented typical features of LSCS. Overlapping sclerodermic lesions, as seen in LSCS, are frequently observed in PFH and the clinical distinction of the two conditions is often difficult. Neurologic
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Funding sources: None.
Conflict of interest: None identified.