frontal fibrosing alopecia ( ffa ) is increasingly considered as a distinct variant of lichen planopilaris ( lpp ) , with scarring alopecia of frontotemporal hairline and nonscarring alopecia of the eyebrows .
ffa and graham  little  piccardi syndrome ( glps ) can be considered as spectrum of a disease as per the current literature due to the presence of overlapping features .
a 49-year - old female presented with hair loss from both center and sides of the scalp for the last 1 year which did not improve following minoxidil therapy .
her family history was unremarkable , and she did not suffer from any chronic disease . no history of chronic medication could be elicited .
personal and family history of photosensitivity was absent . on examination , we found scarring alopecia with follicular papules and mild scaling over the vertex [ figure 1a and b ] .
she had definite recession of frontotemporal hairline with bilaterally symmetrical band - like scarring alopecia of the same region [ figure 2 ] .
there were numerous violaceous follicular spinous papules distributed over the abdomen , back , arms , and legs [ figure 3a  c ] .
( b ) close - up view recession of frontotemporal hairline with symmetrical band - like scarring alopecia ( a ) violaceous follicular spinous papules over the abdomen .
( c ) violaceous follicular spinous papules over the leg nonscarring alopecia over the axilla routine investigations with complete hemogram , blood sugar , liver function test including hepatitis profile and thyroid screening were within normal limit .
there was definite basal cell degeneration with band - like chronic inflammatory infiltrate around perifollicular region .
all these features were consistent with lpp [ figure 5a and b ] . ( a )
follicular plugging with band - like chronic inflammatory infiltrate around perifollicular region , occasional loss of hair follicles , and perifollicular fibrosis ( h and e , 10 ) .
( b ) follicular plugging with definite basal cell degeneration with band - like chronic inflammatory infiltrate around perifollicular region ( h and e , 40 ) based on above findings , a diagnosis of glps with ffa was made , and the patient was put on oral prednisolone ( 1 mg / kg / day ) and oral retinoid ( 0.5 mg / kg / day ) for initial 2 weeks .
dose of the steroid was tapered to 20 mg / day and continued along with the same dose of oral isotretinoin ( 20 mg ) for another 2 weeks with strict monitoring of blood parameters without any alteration . at the end of 1 month ,
the patient had significant improvement of pruritus and hair loss with flattening of the follicular papules .
doses of steroid and isotretinoin were further reduced to 10 mg / day and 15 mg / day , respectively , for another 1 month with further flattening of follicular papules and improvement of ffa patch .
now , the patient is on only 15 mg oral retinoid every alternate day and with regular follow - up on a monthly basis .
the entity , glps was born with a lot of controversies as far as proper clinical definition is concerned .
our observation of simultaneous presence of two variants may further add fire to the unsolved issues of this strange entity .
glps is an unusual variant of lpp characterized by multifocal cicatricial alopecia of the scalp , noncicatricial alopecia of the axillae , and/or pubis and follicular lichen planus ( lp ) involving the trunk and extremities . on the other hand ,
this progressive form of fibrosing alopecia has failed to show any significant difference from other forms of lpp as far as histopathology and immunophenotyping are concerned .
if we look into the pathogenesis , glps is generally considered of having an autoimmune origin . in ffa ,
the lymphocytic infiltrate and fibrosis affect selectively the intermediate and the vellus - like follicles of the frontal margin and eyebrows .
although the reason for this selective involvement is still an enigma , it is hypothesized that it may represent a variety of lpp with selective involvement of certain androgen - dependent areas .
the affected follicles may have typical biologic markers that could explain typical characteristic feature of ffa .
there was several existing literatures where ffa was found to be associated with different forms of lp . in one report , authors concluded ffa as a phenotypic variation of glps where additional features are nonscarring alopecia of the eyebrow and axillae and follicular lp over the face .
other authors described ffa in the association of cutaneous lp and oral mucosal lp . to the best of our knowledge , like our case
, no such case has been reported till date where ffa is found along with full blown glps with all features .
there are several treatment options available for lpp such as oral steroid , oral retinoid , cyclosporine , mycophenolate mofetil , hydroxychloroquine , and psoralen plus ultraviolet a therapy .
surprisingly , our case responded very well in terms of symptomatic improvement , clearing of lesions , and halt of progressive alopecia .
the classical and extensive presentation of glps along with concomitant ffa in the index case portrays a rare and unique presentation of follicular lp .
such a coexistence of these two variants of lpp may be a mere overlap , a phenotypic variation of the same entity , or it may help in future to frame a newer nosology for lpp .
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the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s )
has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal .
the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .
 graham  little  piccardi syndrome ( glps ) is a rare form of follicular lichen planus and comprises cicatricial alopecia of the scalp , noncicatricial alopecia of the axillae , and/or pubis and spinous follicular papules involving the trunk and extremities . 
 we herein report a classic case of glps in a 49-year - old female . 
 uniqueness of our case is due to its additional features of frontal fibrosing alopecia ( ffa ) . 
 although overlap between glps and ffa has been mentioned in literature , such numbers are scanty . 
 therapeutic response with oral steroid and retinoid is also far better in contrast to what is described in the existing literature . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION
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