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Clinical and serological characteristics of progressive facial hemiatrophy: A case series of 12 patients

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Background

Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders.

Objective

We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS).

Method

A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings.

Results

Five patients (42%) presented with complete PFH, and 7 patients (58%) with partial PFH involving either cheek or forehead. Five of the patients (42%) had a coexisting LSCS lesion, and 3 of them (25%) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders.

Limitations

There were a relatively small number of patients in a retrospective study.

Conclusion

The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes, one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.

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.

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