Scleroderma ‘en coup de sabre’ and progressive facial hemiatrophy. Is it possible to differentiate them?

Abstract

The aim was to be able to evaluate the diagnosis of two diseases by a consensus of clinical opinion used in the Department of Dermatology of the National Institute of Paediatrics in Mexico City. To differentiate between scleroderma 'en coup de sabre' (SCS) and progressive facial hemiatrophy (PFH), colour slides of 13 patients diagnosed as SCS and nine as PFH were examined by two dermatologists and microscopic slides by two pathologists. In both cases, the slides were randomly presented and no clinical information was given. The clinical and histopathological findings were statistically compared with two-tailed tests and alpha = 0.05. Kappa coefficients were obtained to evaluate the concordance between dermatologists, pathologists, and in terms of the consensus diagnosis. The usefulness of photographic assessment is limited by the inability to palpate the consistency of lesions. The most important clinical feature that differentiated both conditions was cutaneous sclerosis present in eight of 13 patients with SCS and in none of the PFH patients (P < 0.005). Other clinical features more frequently found in SCS were cutaneous hyperpigmentation and alopecia. The more frequent clinical features in PFH were total hemifacial involvement and ocular changes. Statistically significant histopathological features were: connective tissue fibrosis present in all cases with SCS and two of nine patients with PFH (P < 0.0002); adnexal atrophy present in 11 of 13 patients with SCS, and in three of nine with PFH (P < 0.02), and mononuclear cell infiltrates in all patients with SCS cf. six with PFH (P < 0.05). Our results suggest that in most cases it is possible to differentiate SCS from PFH based on clinicopathological findings.

En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients

“…[3][4][5][6][7] Our data support the theory that they are in fact on the same spectrum of disease for the following reasons. First, of the 28 patients in our study with Parry-Romberg syndrome, 20 (71.4%) of them had some cutaneous facial sclerosis, 15 (53.6%) of them with en coup de sabre lesions.…”

“…2 The relationship between these two entities is not entirely clear, although studies have suggested that a close relationship exists. [3][4][5][6][7] As en coup de sabre morphea and Parry-Romberg syndrome are relatively rare entities, virtually all information regarding demographics and clinical findings is based on case reports, small case series, and one Internet-based survey. 3 Similarly, not much is known about the efficacy of agents commonly used in treatment of these diseases.…”

A review of Parry-Romberg syndrome

“…147 Moreover, both disorders have comparable neurologic and ophthalmologic findings, 147 and both may respond to immunosuppressive treatment. 76,112,151,152 Furthermore, both conditions may show overlap or transition 14,17,28,43,44,58 ; although LSCS and PRS were found to coexist in many patients, [153][154][155][156] some have described patients with LSCS converting with time to PHA (Fig 4). 155,157 The prevalence of LSCS in conjunction with PRS is uncertain, but has been reported to range from 36.6% to 53.6%.…”

Characterization of interfacial reactions between connective tissue and allogenous implants used for subdermal soft tissue augmentation

“…Congenital malformations 6 , autoimmune disease 26 , HIV protease inhibitor associated lipodystrophy syndrome 3 , aging 1,11 , trauma and radical operations in cancer treatment result in extraoral and intraoral soft tissue deficiencies of varying size. Current treatment mainly uses autogenous tissue transfer from the adjacent 42 area or another part of the body 19 .…”