in 1963 , rowell et al . described a distinctive subset of patients with lupus erythematosus ( le ) consisting of erythema multiforme - like skin lesions , anti - la ( ss - b)/anti - ro ( ss - a ) antibodies , positive serum rheumatoid factor and a speckled pattern of antinuclear antibodies .
though its progression to acute le has been documented , progression to anti - double - stranded dna ( ds - dna ) negative lupus nephritis has not been reported till date .
an 18-year - old female presented with fever since 1 month and rash over face and forearms since 3 weeks .
the patient was apparently all right prior to 1 month , when she developed high - grade continuous fever .
three days later she developed blisters and red lesions over face and forearms and blackish lesions over forearms .
she did not give any history of joint pains , decreased urine output and hematuria , chest pain , dyspnea , cough or no history of spontaneous bleeding tendencies .
lesions over the face are in the form of palpable purpura , scars and targetoid lesions [ figure 1a and b ] .
purpuric and targetoid lesions were also seen over the arms [ figure 2a ] and chest .
non - tender erythematous macules and targetoid lesions were seen over palms [ figure 2b ] .
nail - fold capillaroscopy revealed proximal nail fold erythema , ragged cuticles , and capillary telangiectasia and nail - fold infarcts [ figure 4a  c ] .
( a ) clinical photograph showing scars and targetoid lesions over face and crusting over lower lip .
( b ) clinical photograph of face showing scars and targetoid lesions on closer view ( a ) clinical photograph showing scars and targetoid lesions and palpable purpura over arm .
( b ) clinical photograph showing non tender erythematous macules and targetoid lesions were seen over palms crusting erosions and edema were seen over lips ( a ) nail fold capillaroscopy showing erythema , telangiactasia , ragged cuticles and infarcts over proximal nail fold .
( c ) nail - fold capillaroscopy showing nail fold infact on higher magnification systemic examination was normal .
complete hemogram and urine examination were normal except for a raised esr of 30 mm / hour .
anti - nuclear antibody was positive with a titer of 1:160 and showed a speckled pattern .
anti- ro antibody was found to be negative with a titer of 11 u / ml ( titer of > 10 u / ml is considered positive by immunofluorescence method ) .
histopathological examination of the biopsy specimen taken from targetoid lesion over the forearm revealed orthokeratosis , focal hypergranulosis , basal cell vacuolar degeneration , lymphohistiocyic infiltrate at the dermo epidermal junction with pigment incontinence [ figure 5a and b ] .
( a ) section from targetoid lesion showing orthokeratosis , focal hypergranulosis , basal cell vacuolar degeneration , lymphohistiocyic infiltrate at the dermo epidermal junction with pigment incontinence ( h and e 10x ) .
( b ) section from targetoid lesion showing basal cell vacuolar degeneration with lymphohistiocytic infiltrate at dermoepidermal junction ( h and e 40x ) a diagnosis of rowell 's syndrome was made on the basis of clinical and laboratory findings .
the patient was duly started on tablet hydroxychloroquine 200 mg twice daily and tablet prednisolone at 30 mg which was gradually tapered was gradually tapered to 5 mg over a period of 6 months .
four months later , she developed recurrence of lesions on face , chest , back , arms and ears .
erythematous papules with dusky red hue were seen over the concha of ears and forearms .
twenty - four hour urine protein examination revealed 472 grams of protein per 24 hours(normal range is less than 80 mg per day ) .
complement c3 levels were low with a titer of 83.4 mg / dl.(normal range is 75 - 135 mg / dl ) .
histopathology of the kidney revealed increased mesangial cellularity and increased cellular proliferation in few glomeruli with mild basement membrane thickening .
there was no evidence of tubular casts , interstitial inflammation or blood vessel thickening [ figure 6 ] .
immunofluorescence showed moderate peripheral coarse granular igg , iga and c3 deposits with weak igm deposits .
the patient was started on prednisolone 40 mg , tacrolimus 1.5 mg twice daily and ramipril10 mg for one month . with
the above treatment 24 hour urine protein decreased to 744 mg of protein per 24 hours .
histopathological section from kidney showing increased mesangial cellularity and increased cellular proliferation in few glomeruli with mild basement membrane thickening . interstitial inflammation and blood vessel thickening was seen .
an association of erythema multiforme and le was first noted by scholtz in 1922 . in 1963 , during a study of 120 patients with discoid le , rowell et al . found four patients to have distinctive clinical and immunologic findings that included erythema multiforme - like lesions , chilblain - like lesions , speckled pattern of antinuclear antibodies , positive serum rheumatoid factor , and antibodies to saline extract of human tissues ( anti sjt positivity ) . of the characteristic immunological abnormalities speckled pattern of ana was the
anti - sjt positivity has been replaced by anti - ro and anti - la positivity . in 2000 , zeitouni et al . redefined rowell 's syndrome with major and minor criteria .
major criteria included 
 i)le : acute , subacute or discoidii)erythema multiforme like lesions ( with / without involvement of the mucous membranes),iii)speckled pattern of ana . 
 
le : acute , subacute or discoid erythema multiforme like lesions ( with / without involvement of the mucous membranes ) , speckled pattern of ana . 

i)chilblains , ii)anti - ro antibody or anti - la antibody , iii)positive rf . 
 
anti - ro antibody or anti - la antibody , our patient satisfied all three major and one of the minor of the above criteria and also satisfied four of the acr criteria for sle . though originally described in cases of dle , there is now evidence to classify rowell 's syndrome as a type of subacute le rather than a separate entity .
the early annular polycyclic lesions of scle may resemble erythema multiforme with similar histopathological and immunologic findings .
rowell 's syndrome has been described with all subtypes of le ( systemic , acute , subacute or discoid ) .
unidentified hsv , ebv or other viral infections may cross - react with lupus autoantigens and they can initiate the immunologic response , hence triggering the em like lesions in sle .
similarly , immunopathogenetic mechanisms described in both diseases may be responsible for the co - occurrence .
anti - smith ( sm ) and anti - ds - dna antibodies are considered to be nephritogenic and their correlation with lupus nephritis ( ln ) has been well established .
patients with drug - induced ln ( hydralazine ) have been described in the literature to have negative ds - dna and anti - sm antibodies on serological screening .
only seven cases of non - drug - induced severe ln with negative ds - dna antibodies are reported .
progression of rowell 's syndrome to ds - dna negative ln has not been reported in the literature .
nail changes seen at presentation when the patient only had features of rowell 's syndrome have not been documented in previously reported cases . 

nail changes as seen in our case have not yet been described with rowell 's syndromeour case developed lupus nephritis and was ds - dna negative .
ds - dna negativity has not been described in non - drug - induced lupus with cutaneous features . 
 
nail changes as seen in our case have not yet been described with rowell 's syndrome our case developed lupus nephritis and was ds - dna negative .
ds - dna negativity has not been described in non - drug - induced lupus with cutaneous features .
 rowell 's syndrome is now identified as a subtype of subacute lupus erythematosus ( le ) with erythema multiforme - like skin lesions , positive serum rheumatoid factor , anti - ro la positivity and speckled pattern of antinuclear antibodies . 
 here we describe a case of rowell 's syndrome in an 18-year - old female who was found to be ds - dna negative , who later progressed to develop stage v lupus nephritis ( ln ) over a course of 4 months . 
 although extremely rare , most cases of ln are drug induced . 
 of only seven cases of non - drug induced ln with negative dsdna , none had cutaneous involvement . 
 ours was a unique case of progression of rowells syndrome to ds - dna negative ln . 
Introduction
Case Report
Discussion