Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications
© Gambichler et al; licensee BioMed Central Ltd. 2001
Received: 12 November 2001
Accepted: 4 December 2001
Published: 4 December 2001
Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. We describe an unusual case of LSC.
A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented.
The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and LSCS actually represent different spectra of the same disease.
Variants of localized scleroderma recently classified by Tuffanelli 
I. Localized scleroderma
a) Morphea [plaque-type; guttate; profound; nodular; bullous; Atrophoderma of Pasini and Pierini]
b) Linear scleroderma ["en coup de sabre"; with facial atrophy (Parry-Romberg syndrome); acral; with arthritis, myositis, and growth defects of bone; melorheostotic]
c) Generalized morphea [pansclerotic; edematous; with lichen sclerosus]
II. Localized scleroderma as a component of an overlap syndrome
a) Mixed connective tissue disease
b) Undifferentiated connective tissue disease
III. Localized scleroderma as a component of eosinophilic fasciitis (Shulman's syndrome)
In the present case, band-like scleroderma and facial atrophy of the frontoparietal area was observed. The skin involvement did not extend below the forehead. These features were consistent with a diagnosis of LSCS with facial atrophy [1–3]. In classic progressive facial hemiatrophy or Parry-Romberg syndrome (PRS), cutaneous inflammation, induration, and adhesion are absent or minimal, and atrophy usually involves the one entire side of the face [4–6]. It has been suggested that the term PRS should only be used for progressive hemifacial atrophy occurring without features of scleroderma . However, a clear differentiation between PRS and involutionary LSCS is often not possible, and it is rather an arbitrary approach to differentiate the two diseases because of their extend on the face and more or less sclerosis, despite of all their common features observed. Although preceding sclerosis was never noticed in many cases of PRS, Jablonska and Blaszczyk  observed several patients with typical LSCS in children converting within several years into facial hemiatrophy. Thus the time of investigation is of significance when diagnosing either LSCS or PRS. There are no reliable histological criteria to differentiate PRS from involutionary scleroderma. Recently, Blaszczyk et al.  reported three cases of primary atrophic profound linear scleroderma with involvement of the subcutis and deeper tissues. In accordance with a previous report of Malandrini et al. , deep skin biopsies showed inflammatory infiltrates in the endomysium and perimysium. Interestingly, atrophy was not preceded by clinical evidence of inflammation, discoloration of the skin, or sclerosis. Blaszczyk et al.  concluded that PRS could be regarded as a variety of deep linear scleroderma [7, 9].
Common theories on the etiology of LSCS are: firstly, that the localized forms of scleroderma are related in the same manner as discoid and systemic lupus erythematosus and thus belonging in the collagen-vascular group of diseases with autoimmune phenomena or secondly, that localized scleroderma is a developmental disease, occasionally associated with other (predetermined) defects . Other theories implicate that viral or bacterial infections (e.g. B. burgdorferi) and genetic factors may play a role in the etiology of LSCS . However, a multifactorial pathogenesis of hemiatrophies is most likely. Ophthalmological (e.g. iridocyclitis, enophthalmus, exophthalmus) and/or neurological (e.g. intracerebral calcification, hemiplegic headaches, epileptic seizures) manifestations have frequently been observed in patients with LSCS and PRS [2–4, 10, 11]. Besides, non-neurogenic myopathy associated with LSCS has previously been reported [11, 12]. However, cranial nerve palsy coexisting with LSCS or PRS is a rarity. The involved areas in LSCS do usually not cross the midline and may resemble innervated fields in a cranial nerve distribution, in particular the upper trigeminal dermatome, raising the possibility of a neurogenic origin. It has therefore been suggested that LSCS and PRS result from hyper- or hypoactivity of sympathetic nervous system or abnormality of the trigeminal nerve. Pathological evidence of intracerebral inflammation in a patient with LSCS has recently been observed by Stone et al. . Accordingly, we suggest that computed tomography and magnetic resonance imaging are useful diagnostic tools for the investigation of patients with LSCS, in particular for detecting clinically inapparent intracranial changes [13, 14]. In the present case, cutaneous atrophy involved both frontal sites. Bilateral lesions of LSCS have been considered to be extremely rare . Lateralization of LSCS possibly indicates a neuropathological etiology of the disease. Notably, there were no preceding clinical features of scleroderma in the atrophic area affecting the right forehead of our patient indicating that the atrophic cleft on the right forehead was caused by profound linear scleroderma .
The management of localized scleroderma is still unsatisfactory. Various therapeutic modalities (e.g. topical and pharmacological agents, immunosuppresion, physiotherapy, and phototherapy) have been suggested . In an uncontrolled study, we have recently observed that childhood scleroderma can successfully be treated with topical calcipotriol and low-dose UVA1 phototherapy . However, management of facial atrophy is a challenge. As shown in the presented case, palliative reconstruction surgery is potentially beneficial for patients with disfiguring facial atrophy, and the employment of 3-dimensional reconstuction imaging may be helpful in preoperative scheduling of bony reconstruction surgery .
List of abbreviations
linear scleroderma "en coup de sabre"
We are very grateful to the patient who gave us written consent for publication of her details and figures.
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