lichen sclerosus et atrophicans ( lsa ) is a chronic inflammatory muco - cutaneous disorder , characterized by sclerotic and atrophic lesions , most commonly found in adult women .
it affects the anogenital area in 8598% of the cases and less frequently the extrgenital area .
the most commonly affected extragenital areas are the neck and the shoulders , but also the inner thighs , the submammary area , the wrist and occasionally the oral mucosa .
the involvement of the scalp is not frequent and its outcome could be similar to scarring alopecia , which could be the result of different diseases .
the etiology of lsa is still unknown . besides the genetic and local factors ( koebner phenomenon ) and the autoimmune hypothesis , supported by the association of different autoimmune disorders , especially of thyroid origin ( 30% of cases ) , an infectious hypothesis has also been proposed .
a 57-year - old caucasian woman presented with a history of asymptomatic frontoparietal lesion . such lesion , which had been developing over the 3 previous years , was initially erythematous and became progressively atrophic and sclerodermic .
the patient had been living in a highly endemic area for borrelia burgdorferi , but she could not recall any tick bite or erythema chronicum migrans .
she also reported the simultaneous onset of migrating diffused myoarthralgias involving knees , hands , ankles , elbows , shoulders , as well as short - term memory impairment for two years , worsened previous seasonal insomnia for one year .
moreover , she presented migrating paresthesias involving the left side of the body for one month .
the dermatological examination revealed an atrophic - sclerodermic lesion of about 10 cm in length and 3 cm in width , from the scalp to the centre of the forehead as reported in figure 1 .
the skin was thin , inelastic , mother - of - pearl in shade , with erythematous margins of the lesion with subsequent scarring alopecia as well ( figure 1 ) . 

figure 1lichen sclerosus et atrophicans with frontoparietal distribution , mimicking scleroderma en coup de sabre . 
 
a skin biopsy was obtained from the scalp and after histological examination three different pathologists confirmed independently the diagnosis of lsa .
it consisted of epidermal atrophy , oedema with superficial layer , hyperkeratosis and collagen production with cell rarefaction .
lymphoid infiltrate was observed even in the dermal - subepidermal junction indicating a possible later evolution of the lsa toward morphoea ( figure 2 ) . 

figure 2h&e stained section of the lesion ( a ) 2,5  magnification of the entire histological section , ( b ) 20 magnification of the lichen sclerosus et atrophicans features ( c ) 40 magnification of the lymphatic infiltrate . 
  h&e stained section of the lesion ( a ) 2,5  magnification of the entire histological section , ( b ) 20 magnification of the lichen sclerosus et atrophicans features ( c ) 40 magnification of the lymphatic infiltrate .
serological igm and igg for borrelia burgdorferi with enzyme - linked immunoassay test ( elisa ) confirmed by western blot analysis was negative .
anti - nuclear antibodies ( ana ) , extractable nuclear antigen antibodies ( ena ) , anti - native dna antibodies ( n - dna ) , anti - neutrophil cytoplasmic antibodies ( anca ) and erythrocyte sedimentation rate were negative .
pcr analysis for the detection of borrelia burgdorferi was performed on dna obtained from formalin - fixed paraffin - embedded skin biopsy , blood and urine as previously reported .
the diagnosis of lyme borreliosis was made on the basis of clinical data , supported by the pcr positivity for borrelia genome .
the patient underwent antibiotic treatment with 2 cycles ceftriaxone 2 gr / day i.v for 21 days .
after about 6 months since the beginning of antibiotic therapy , the lesion had not progressed , all other clinical symptoms improved and blood pcr resulted negative .
the patient received vitamin e 400 mg 2x / day per os for 3 months and applied topic vitamin e prior to uva-1 therapy .
thanks to this combined therapy a remarkable regression of the atrophic - sclerodermic lesion was observed .
this report describes a case of lsa , which was unusual for the involvement of the scalp
. the lesion was mimicking scleroderma en coup de sabre , which is a frontal or frontoparietal linear morphoea characterized by a linear band of depressed atrophy on skin and scalp .
the classical features of lsa are represented by hypopigmented papules that coalesce into white plaques with epidermal atrophy .
although anogenital lsa is associated with a risk of 45% of squamous cell carcinoma , extragenital lesions do not seem to carry any risk of malignant degeneration .
the isolated linear frontoparietal involvement was described in few cases and may clinically simulate scleroderma en coup de sabre , requiring careful histopathological recognition . in this case
it has already been reported that overlap of histologic features between lsa and morphoea may occur , however in the reported case the clinical and the histologic features were not ambiguous of lsa .
the possible later evolution toward morphoea in this case , due to the migration of the lymphatic infiltrate , is not unusual since morphoea and lsa may be closely related such that the latter could be considered the superficial expression of the same disease process which results in morphoea . regarding the possibility of an infectious etiology of lsa , since the first proposal by aberer and stanek in 1987 several european studies
some atrophic skin diseases have been proposed as manifestation of lyme borreliosis with contradictory results .
the detection methods , the examined specimens , such as sera , skin biopsies and urine , together with the different geographic region could explain the conflicting results on the association of borrelia with morphoea and lichen sclerosus et atrophicus .
moreover in the manifestation of long standing infection of borrelia the paucity of microorganisms could lead to a low detection rate by pcr , especially when the analysis is performed on archival biopsies .
no decision can be made to date as to whether bb plays a role as causative agent of different types of circumscribed scleroderma and lsa . with regard to the disparate findings in different geographic areas
, it can be speculated that lsa may be caused in some cases by bb genotypes which are present in that area only . to support this infectious etiology in endemic regions ,
the fact that in this case borrelia dna was detected both on dna obtained from the biopsy and from blood and urine , strongly supports the hypothesis that borrelia has a causative role on the onset of this unusual lsa .
moreover , the geographical location , middle europe , has been highly associated with borrelia prevalence .
indeed , it is well known that there are significant geographic differences in borrelia infections with a higher prevalence in areas in middle europe . to further support this theory ,
lyme disease affects mostly the skin : about 80% of all lyme borreliosis cases present skin manifestations .
we recognise that classical dermatological events include erythema chronicum migrans ( ecm ) , lymphadenosis benigna cutis ( labc ) or borrellial lymphocytoma ( bl ) and acrodermatitis cronica et atroficans ( aca ) , but there is growing evidence that some cases of other cutaneous manifestations could be related to borrelial late infection , mostly borrelia afzelii .
common laboratory tests are not usually revealing for the diagnosis of lyme borreliosis and serologic tests support the diagnosis , but are not always essential in this regard . in this case of lsa mimicking scleroderma en coup de sabre , an associaton with lyme borreliosis
is proposed . to our knowledge , this is the first case of lsa , mimicking scleroderma en coup de sabre , which was associated with lyme borreliosis .
our findings indicate an association between this particular form of lsa and borrelia , suggesting that borrelia burgdorferi itself could represent a causative agent of this atypical form of lsa , however it can not be excluded that borrelia could be only one of the predisposing agent triggering it .
in conclusion we propose that tissue pcr for dna of borrelia should be performed in patients with lsa in endemic area , because it could represent a rare manifestation of borreliosis , and in those cases lsa should be treated with proper antibiotic therapy in order to eradicate the microorganism .
 lichen sclerosus et atrophicans ( lsa ) is a chronic , inflammatory skin disease of unknown etiology , characterized by atrophy.we report a case of lsa with frontoparietal distribution , mimicking scleroderma en coup de sabre , causing scarring alopecia . 
 the case was associated with borrelia infection . 
 the lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr / day i.v for 21 days associated with uva-1 therapy and local and systemic vitamin e supply ( 400 mg 2x / day per os for 3 months ) . 
 this case stresses the importance of identifying clinical manifestations associated with lyme disease and the use of tissue pcr to detect borrelial dna in patients with these lesions , but characterized by negative serology for borrelia . 