British Journal of Dermatologff i\ 991) 124, 498-502.
ADONIS 0OO7O96391(M)119P
A case report of a patient with features of systemic lupus erythematosus and linear IgA disease M.LAU. I . K A U F M A N N - G R C N Z I N G H K * AND M.RAGHUNATH Division of tmmutioikithologn. Institute of Immunalogif and 'Department of DermatolosH. Vnivenitfi of Heidelberg. Germany Accepted for publication 14 Novt-mbor 1990
Summary
A case is reported of a 5S-yeur-()ld woniiin with a 22-year history of systemic lupus erythemutosus (Sli:) who later developed a blistering eruption compatible with the diagnosis of linear IgA disease.
Since 1979 linear IgA disease has heen differentiated as a distinct entity trom that of dermatitis herpetiformis hy the homogeneous linear deposition of IgA at the basement membrane zone (BMZ) of the epidermis and with a lack ofan associated gluten-dependent enteropathy.'"*
hospital in Heidelberg. She was pyrexial and there were urticated areas of erythema and large tense blisters arranged in a herpetiform manner on her abdomen and left thigh (Fig. 1). There was no involvement of the mucous membranes.
Case report
Utboratorii investigalious
A 3 5-year-oId woman had from the age of 15 a history of intermittent arthralgia. fever and anaemia. She was diagnosed as having systemic lupus erythematosus and in April 19S5 she was seen at the Mannheim Clinic with fever and having had a haemoptysis. A peri myocarditis and pericardial effusion with an alveolitis and hilateral pieural effusions were present, and 5 days after admission she developed an erythematous rash on the trunk and thighs with several blisters on the right thigh. Vesicles later appeared on the biiccal mucous membrane and on the tongue. Oedema of both legs and purpura was associated with a vascuiitis and a thrombotytcjpenia. Anti-ONA antihodies and antinuclear antihodies in a titre of 1 :640 were present. The serological findings were compatible with a diagnosis of systemic lupus erythematosus (SIJi). The patietit was initially treated with 100 mg prednisone daily and with an improvement of the symptoms the dose was reduced to 60 mg daily and she was then discharged from hospital. She remained well over the next 4 years until March 1989. when the dose of prednisone was reduced further to 2- 5 mg daily, hi midJuly 1989 she developed blisters in itchy areas of erythema on the abdomen and thighs. She later had an arthralgia with fever and diarrhoea and was admitted to
Haemoglobin was 11-7 g/dl. WBC 24-9 x 10^1. with 50% neutrophils. ?6% lymphocytes. 6% monocytes, 2% atypical lymphocytes. The platelet count was 4S1.000/ ml and the liSK was 37 mm/h. The serum immunoglobulins were IgG 20-S g/l, IgA 2-14 g/l and IgM la'i g/i. Kchocardiography revealed a small pericardial effusion and there was also a small left-sided pieural diffusion.
Corrcspondemc: l> M.Uu. llnivcrsitHtskHnikum Stcgiilz. Mautklinlk WF 07. HindcnbiirKdamni JU. U-IOOO Berlin 45. Uemiany.
498
A skin biopsy from the medial aspect of the thigh showed a subepidermal hulla. the lumen of which was filled with fibrinoid material and inflammatory cells that consisted mainly of lymphocytes and eosinophils (Fig. 2). There was a marked lymphohistiocytic inliliralion of the upper dermis with numerous eosinophils und a perivascularmlxed infiltrate in the dermis. hnmunopathologii I'our separate skin biopsies were examined by direct immunotluorescence (I)ll") and the findings are shown in Table 1. In all four biopsies there was a homogeneous linear deposition of IgA at the dermo-epidermal junction zone (Fig. 3). This was most intense in the biopsy taken of a fresh lesion from non-sun-exposed skin and showed only subclass IgAl. In the lesional non-sun-exposed skin there was also an additional faint homogeneous deposi-
SLE AND LINEAR IGA DISEASE
499
Figure 1. Vesiculobullous eruptions on the medial aspect of the thtgh.
tion of IgM at the BMZ. This was confirmed using the more sensitive APAAP method.'* The BMZ showed deposits of C4d. C3b. C3d in an unusual pattern with involvement of the epidermal basal layer (Fig. 4). The capillaries in lesional skin from the papillary dermis showed deposits of C4d. C3d (Fig. 4). CS and C9 together with focal deposits of fibrin in the upper dennis away from the BMZ. The serum of the patient was screened for autoantibodies and the only significant finding was the presence of IgA autoantibodies to the BMZ using monkey oesophagus and in a titre of 1 : 5. The presence of circulating
Figure 2. Cryostat section of subepidermal bulla in lesional sun-exposed skin showing a large leucocytic intiltration in the upper dennis consisting largely of eosinophilic granuloeytes (Haematoxylin and eosin. X 100; insert x 4t)0).
IgA antibodies directed to the BMZ was confirmed on cryostat sections of human skin.'' A high titre of 1 :20 was found. The bound IgA was localized in the split skin exclusively to the epidermal side (Fig. 5). An identical pattern was obtained with the serum from another patient with linear IgA disease. The HliA haplotypes of our patient were A23. A24. B35. B57. Cw4 and Cw7. Progress
Our patient was treated with prednisone at a dosage of 50 mg daily for 1 week with some improvement of her
500
M.LAU et al.
Biopsy LNEX
LEX
NHX
Histology No hullii or cleft, multiple dense foci of infiltriiting cells in upper dermis Subepidermal blister. multiplt' den,se perivascular foci of inliltratiny cells in upper dermis Few perivascular foci of jiililiratin^ cells, in upper dermis
IgG
IgM
IgA
BMZ
Few discrete perivascular cellular intiltrations. in upper demiis only
TaWe I. DIF findings in four biopsies from different sites
BMZ r e s of BK. PAPCA
l&c BMZ
pseudolineiir BMZ. ICS of BK
l&d BMZ
l&c BMZ ICSofBK
only NNEX
C3d
l&d BMZ
Ll'.X. lesional, sun-exposed: LNEX, lesional. non sun-exposed; NEX. nonnal. sun-exposed; NNEX. nonnal. mm .sun-exposed: ICS. intercellular Hp«ce: BK. basal keratinocytes; PAPCA. papillary capillaries; l&c. linear and continuous; l&d. linear and discontinuous: linear, sharply defined deposits: pseudo linear, homogeneous, but ill defined deposits along the DE|.
Figure 3. Homogeneous linear deposition oflgA at the BMZ in lesional. non-sun-exposed skin as revealed by DIF ( x 2 SO).
general symptoms and slight improvement of her skin eruptions. On stopping prednisone new blisters occurred and with the tindings from DIF she was then treated with topical eorticosteroids and 100 mg daily of dapsone. Within 1 week there was a marked improvement of her skin lesions and she was discharged from hospital. Several weeks later she had hiisters on the huccal mucous membrane and stopped taking the dapsone and resumed treating herself with prednisone. Since then she has been lost to follow up.
Figure 4. DIF of iesional non-sun-exposed skin, with C id deposited at the BMZ and in the intercellular space of basal and suprdba.sal keratinocytes (x 250).
Discussion Our patient meets the criteria of the ARA'' for SLE with an arthritis, polyserositis. coagulation disorder, thromboeytopenia. with ANF and antibodies against ds-DNA. The patient also had linear IgA disease with no evidence of a gluten enteropathy. The histology of the skin biopsies confirmed the diagnosis of linear IgA disease with a linear and homogeneous deposition of IgA at the dermo-epidermal junction, quite distinct from the IgA
SLE AND LINEAR IGA DISEASE
Figure 9. Human split skin incubated with the patietit's senun diluted 1; Itl. IgA is localized to the epidermal side { x 4001.
pattern occasion ally seen in SIJ;. In addition there was igM deposition as has been previously reported in linear IgA disease/ We found circulating antibodies that reacted to the epidermal portion of the basement membrane zone in split skin as has been reported in other cases of linear IgA disease.'*-^'" These have recently been shown to react only with the epidermal basement zone of split skin and to recognize a 97-kDa antigen in dermal and epidermal extracts." However. we obtained epidermal binding on split-skin substrate with our patient s serum, but could not find any binding to the linear IgA disease antigen. This may be due to a relatively low titre of the antibcxiy." The haplotype of our patient did not demonstrate HLA-BS as found in 8 0 90% of patients with dermatitis herpetiformis and up to 30% of patients with linear IgA disease.'^ Due to our patient's habit of taking prednisone for years without medical control or advice it was difficult to assess how effective dapsone was. It did appear to have some effect in reducing the blistering of her skin although it had liUle or no effect on the lesions of the buccal mucous membrane, which could be attributed to the concurrent SLH. Patients with bullous SLK may respond dramatically to dapsone or sulphapyridine. particularly those with circulating BMZ antibodies.""'*' Those patients without antibodies divide into responders to dapsone'''""^*' and those who do not."''' ~'"^^ Butlous eruptions in SLE are an uncommon manifestation of the disease rather than a co-existing tndepetident bullous dermatosis. There have been four reports of the concurrence of SLE with a bulious dermatosis in the past two decades. !n the light of recent findings a case of epidermolysis bullosa acquisita with a superimposed SlJi where the clinical criteria for both SIM and EBA were met
501
deserves attention'^'*. In that case EBA preceded the onset of SLE by S years. Two reports concerning SLE and concurrent DH have been published.^'•^'' The only publication to date concerning conctirrent SLH and linear IgA disease"' was ofa patient with Sli-^ who later developed a rash compatible with dermatitis herpetiformis and the skin histology was typical for this disorder. However. DIF showed a strong linear deposition of IgA in the normal and involved skin, with faint immunoreactivity for IgG and IgM. This patient had no evidence ofa gluten enteropathy or other circulating antibodies and did not have the Hlu^-BS haplotype. The skin lesions in this patient did not respotid well to corticosteroids but responded dramatically to dapsone. This report is the second since 198 5 ofa patient with concurrent SLH and linear IgA disease. We were also able to demonstrate circulating BMZ antibodies in our patient.
Acknowledgments We are indebted to Dr Leena Bruckner-Tuderman and Y.Seel and for technical assistance to I,Neumann and S.Wentrup. We thank Dv P.Royce for editorial help. .MR was funded by the Swiss National foundation (grant NF 32-27884.89).
References 1 C'hor/elski TF. lablonskii .S. Bcutner V. H. Atiiilt form of lineiir IgA bulluuN dermalosis. In: hnmuno[}allio!ony of the Skin (Beutner VSi. Chor/.elski TP. Bean SI-, edsl. 2nd edn. New York; )ohn Wiley & Sons. 1979: 3Ih. 1 U'oniird IN. HalTenden GP. King NP et at. linear IgA disease In adults. Hr / Dermatol 19K2; 107: JOl-6. } Smith SB. Harrist T|, Murphy CH' cl al Unear IgA bullous ilermatosis v. dermatitis herpetiformis. Arch Dgmtatol 1984: 120: 4 Oirdell II,, Fiilini 11, Hrher WN et al, Immunofn/ymiitic UibcUitig of monoclonal antibodies using imtnuni- complexes of alkalitic phospliatase and monoclonal anti-ulkiiline pho.sphatase (APAAP complexes). / HisttHheni Cytmhem 1984; J2: 219-29. 5 (Gammon WR. Briggaman KA. Inman AOcfftJ. Pifferentiating antilamina lucidit and anti-sublamina densji anti-BMZ antibtxiies by indirect immunolluorescente on I •() M sixliLim chloride-separated skin. / /rtm( Dermatol I9S4: 82: I 39-44. h TanKM.C'ohen AS. l-riL'sircid/. Tbe 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982:25: 1271-7. 7 Leonard IN. Haffenden CP. Unsworth t)l etal [-.vidt-nce that ihe IgA in patients witb linear IgA disease is qualitatively different from tbat of palienls with dermatitis herpetiformis. Br / Dermalol 1984; 110: J 1 5 - 2 I . 8 Mobacken H. Kastrup W. Ljunghall K et al linear IgA dermatosis:
502
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10
II
12
!!
14
15
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a Mudy often adult patlmts. Acta Dermatol Venertot (Sttnkh} 1983; hi: l 2 i - 8 . I'ehHmberger H. Konrad K. Ilolubar K. Circulating IgA antlbasement membrane iintllvHlies in linear detniatitis hfrpfllformis iDuhring): immunofluort-sccticr in linear distribution und immuniH-lectron micntscopU'studies. /Invest l\'nnatol 1477: (»9:490- 1. Yaoiia H. Katz SI. OnuUiting IgA amibasement membrane antlbodtes In dermatitis hcrptrtlfurmis. / Invest tJrnruUol 1977; 69: '558-61. /one II. Taylor TH. Kaduni-c DP. Meyer LJ. IdcniiHcatlon of the i-utaneous basement mrmbnme zone antigen and isolation of antibody in linear imnuituiglobtilln A hullou.s (lermiitdsis, / Clin Invest H 9 n ; 8 S : H12-21I. Uiwley T|. Strober W. Yuoitii H, KatzSI. Small Inlcstinal biopsies and HI_A types in dcrmiitltis herpetifnrmis patients with grunulur and linear IgA skin dcptxsits, / Jnvest l^rrmatol I9S(»: 74: 4 12. ("»immon WR. VVIKHIII-V ixr. Uiie KC. Briggaman R.\. l-Mdcnir (hat anti-basement membrane utne antibodies in bullous eruption of systemic lupus er>ihem*iU«sus ret-ognize epidcrmolyste bullosa antigen. / (nvrst Orrmatol WHS: 84: 472-h. Karton DP. Fine |-l). (lanunon WR. Sams W.M. Bullou!! systemic lupus eiTr'themiitosus: iin unusual clinical course inul detei tublf drill Int ing iiutoiintlliDdifs to Ihc epidennolysis hullosii tic«juisltii anli^cn. I Am Acad Dennutol l^KS: IS: 169-71, KettkT AH, Bt-an Sl\ DulTy |{). tlummon WR. Systemic lupus er>'thematosus presenting as u hullous eruption in a child. Arch Oermatol \*iHK. 124: IDHJ 7. HiippcrsbergcrK.Tsthiich!iTK,TaniM. Wolff K. Biillous disease in s>'stcmic lupusen-thematusus. / Am Aiad Omww/1989; 21: 74552.
17 Camlsa t". Sharma HM. Vesiaitobutlous systemic lupus cry'thematosus. Report of two cases and a review of tlic liteniture. / .Ifri .had Dermatol I9H 1: 9: 924-55. IS Hall RP, 1 jivvlcy l|. Smith HR. KatxSI, Bullouseruption of systemic lupus er>'themalosus. Dramatic rf^ponse to il«pMinc therapy. ,lnn IntXM 19«2:97: Ih5-7O. 19 Pedro SI). Dahl MV. Direct immunoHmircsi-eticc of bullous systemiclupuM-r>ihrmaIosus. .Inh Ormwlt)/ I97J; 1»7: 118-20. 2(t Tani M.Shimbtu K. Ban Metal S>Titemii' lupuscryihematostiswith vesiculobullous lesions. Immunoclcctnm microstopic study. .Arrh Dermatol 1984; 12(1: 1497-iSlM. 21 lacoby RA, Abraham AA. Bullou.s dcmiatlti.s and systemic lupus erylht-miilofnis in ii IS-year-old boy. Arch Dermatol 1979: 115: 1094 7, 22 Olansky A|. Briggaman RA. Gammon WK cl al Bullous systemic lupus er>'thematosus. / Am .Acad Dermalol I9S2: 7: SI I 20. 21 Pcnncys \ S . Wiley HK. Herperifnrm bllsirri In systemic lupus crythnnatwius. -inJi liermatol 1979; 115: 1427 8. 24 Dotson AD. Rnlmrr SS. Purelcy T\'. Tschen J. Systemic lupus erythematosus occurring in a patient with cpidcrmol>'sis bullosa acquisita. Arch Ikrmatol 1981: 117: 422 h. 25 Davies MC>. Marks R. Wiiddington V.. Slnitiliancous systemic lupus erythematosus iind ilcrmalitis hci-pctitomils. Arch tk-rmatol 1976: 112: 1292 4. 26 Arons
ADONIS 0OO7O96391(M)119P
A case report of a patient with features of systemic lupus erythematosus and linear IgA disease M.LAU. I . K A U F M A N N - G R C N Z I N G H K * AND M.RAGHUNATH Division of tmmutioikithologn. Institute of Immunalogif and 'Department of DermatolosH. Vnivenitfi of Heidelberg. Germany Accepted for publication 14 Novt-mbor 1990
Summary
A case is reported of a 5S-yeur-()ld woniiin with a 22-year history of systemic lupus erythemutosus (Sli:) who later developed a blistering eruption compatible with the diagnosis of linear IgA disease.
Since 1979 linear IgA disease has heen differentiated as a distinct entity trom that of dermatitis herpetiformis hy the homogeneous linear deposition of IgA at the basement membrane zone (BMZ) of the epidermis and with a lack ofan associated gluten-dependent enteropathy.'"*
hospital in Heidelberg. She was pyrexial and there were urticated areas of erythema and large tense blisters arranged in a herpetiform manner on her abdomen and left thigh (Fig. 1). There was no involvement of the mucous membranes.
Case report
Utboratorii investigalious
A 3 5-year-oId woman had from the age of 15 a history of intermittent arthralgia. fever and anaemia. She was diagnosed as having systemic lupus erythematosus and in April 19S5 she was seen at the Mannheim Clinic with fever and having had a haemoptysis. A peri myocarditis and pericardial effusion with an alveolitis and hilateral pieural effusions were present, and 5 days after admission she developed an erythematous rash on the trunk and thighs with several blisters on the right thigh. Vesicles later appeared on the biiccal mucous membrane and on the tongue. Oedema of both legs and purpura was associated with a vascuiitis and a thrombotytcjpenia. Anti-ONA antihodies and antinuclear antihodies in a titre of 1 :640 were present. The serological findings were compatible with a diagnosis of systemic lupus erythematosus (SIJi). The patietit was initially treated with 100 mg prednisone daily and with an improvement of the symptoms the dose was reduced to 60 mg daily and she was then discharged from hospital. She remained well over the next 4 years until March 1989. when the dose of prednisone was reduced further to 2- 5 mg daily, hi midJuly 1989 she developed blisters in itchy areas of erythema on the abdomen and thighs. She later had an arthralgia with fever and diarrhoea and was admitted to
Haemoglobin was 11-7 g/dl. WBC 24-9 x 10^1. with 50% neutrophils. ?6% lymphocytes. 6% monocytes, 2% atypical lymphocytes. The platelet count was 4S1.000/ ml and the liSK was 37 mm/h. The serum immunoglobulins were IgG 20-S g/l, IgA 2-14 g/l and IgM la'i g/i. Kchocardiography revealed a small pericardial effusion and there was also a small left-sided pieural diffusion.
Corrcspondemc: l> M.Uu. llnivcrsitHtskHnikum Stcgiilz. Mautklinlk WF 07. HindcnbiirKdamni JU. U-IOOO Berlin 45. Uemiany.
498
A skin biopsy from the medial aspect of the thigh showed a subepidermal hulla. the lumen of which was filled with fibrinoid material and inflammatory cells that consisted mainly of lymphocytes and eosinophils (Fig. 2). There was a marked lymphohistiocytic inliliralion of the upper dermis with numerous eosinophils und a perivascularmlxed infiltrate in the dermis. hnmunopathologii I'our separate skin biopsies were examined by direct immunotluorescence (I)ll") and the findings are shown in Table 1. In all four biopsies there was a homogeneous linear deposition of IgA at the dermo-epidermal junction zone (Fig. 3). This was most intense in the biopsy taken of a fresh lesion from non-sun-exposed skin and showed only subclass IgAl. In the lesional non-sun-exposed skin there was also an additional faint homogeneous deposi-
SLE AND LINEAR IGA DISEASE
499
Figure 1. Vesiculobullous eruptions on the medial aspect of the thtgh.
tion of IgM at the BMZ. This was confirmed using the more sensitive APAAP method.'* The BMZ showed deposits of C4d. C3b. C3d in an unusual pattern with involvement of the epidermal basal layer (Fig. 4). The capillaries in lesional skin from the papillary dermis showed deposits of C4d. C3d (Fig. 4). CS and C9 together with focal deposits of fibrin in the upper dennis away from the BMZ. The serum of the patient was screened for autoantibodies and the only significant finding was the presence of IgA autoantibodies to the BMZ using monkey oesophagus and in a titre of 1 : 5. The presence of circulating
Figure 2. Cryostat section of subepidermal bulla in lesional sun-exposed skin showing a large leucocytic intiltration in the upper dennis consisting largely of eosinophilic granuloeytes (Haematoxylin and eosin. X 100; insert x 4t)0).
IgA antibodies directed to the BMZ was confirmed on cryostat sections of human skin.'' A high titre of 1 :20 was found. The bound IgA was localized in the split skin exclusively to the epidermal side (Fig. 5). An identical pattern was obtained with the serum from another patient with linear IgA disease. The HliA haplotypes of our patient were A23. A24. B35. B57. Cw4 and Cw7. Progress
Our patient was treated with prednisone at a dosage of 50 mg daily for 1 week with some improvement of her
500
M.LAU et al.
Biopsy LNEX
LEX
NHX
Histology No hullii or cleft, multiple dense foci of infiltriiting cells in upper dermis Subepidermal blister. multiplt' den,se perivascular foci of inliltratiny cells in upper dermis Few perivascular foci of jiililiratin^ cells, in upper dermis
IgG
IgM
IgA
BMZ
Few discrete perivascular cellular intiltrations. in upper demiis only
TaWe I. DIF findings in four biopsies from different sites
BMZ r e s of BK. PAPCA
l&c BMZ
pseudolineiir BMZ. ICS of BK
l&d BMZ
l&c BMZ ICSofBK
only NNEX
C3d
l&d BMZ
Ll'.X. lesional, sun-exposed: LNEX, lesional. non sun-exposed; NEX. nonnal. sun-exposed; NNEX. nonnal. mm .sun-exposed: ICS. intercellular Hp«ce: BK. basal keratinocytes; PAPCA. papillary capillaries; l&c. linear and continuous; l&d. linear and discontinuous: linear, sharply defined deposits: pseudo linear, homogeneous, but ill defined deposits along the DE|.
Figure 3. Homogeneous linear deposition oflgA at the BMZ in lesional. non-sun-exposed skin as revealed by DIF ( x 2 SO).
general symptoms and slight improvement of her skin eruptions. On stopping prednisone new blisters occurred and with the tindings from DIF she was then treated with topical eorticosteroids and 100 mg daily of dapsone. Within 1 week there was a marked improvement of her skin lesions and she was discharged from hospital. Several weeks later she had hiisters on the huccal mucous membrane and stopped taking the dapsone and resumed treating herself with prednisone. Since then she has been lost to follow up.
Figure 4. DIF of iesional non-sun-exposed skin, with C id deposited at the BMZ and in the intercellular space of basal and suprdba.sal keratinocytes (x 250).
Discussion Our patient meets the criteria of the ARA'' for SLE with an arthritis, polyserositis. coagulation disorder, thromboeytopenia. with ANF and antibodies against ds-DNA. The patient also had linear IgA disease with no evidence of a gluten enteropathy. The histology of the skin biopsies confirmed the diagnosis of linear IgA disease with a linear and homogeneous deposition of IgA at the dermo-epidermal junction, quite distinct from the IgA
SLE AND LINEAR IGA DISEASE
Figure 9. Human split skin incubated with the patietit's senun diluted 1; Itl. IgA is localized to the epidermal side { x 4001.
pattern occasion ally seen in SIJ;. In addition there was igM deposition as has been previously reported in linear IgA disease/ We found circulating antibodies that reacted to the epidermal portion of the basement membrane zone in split skin as has been reported in other cases of linear IgA disease.'*-^'" These have recently been shown to react only with the epidermal basement zone of split skin and to recognize a 97-kDa antigen in dermal and epidermal extracts." However. we obtained epidermal binding on split-skin substrate with our patient s serum, but could not find any binding to the linear IgA disease antigen. This may be due to a relatively low titre of the antibcxiy." The haplotype of our patient did not demonstrate HLA-BS as found in 8 0 90% of patients with dermatitis herpetiformis and up to 30% of patients with linear IgA disease.'^ Due to our patient's habit of taking prednisone for years without medical control or advice it was difficult to assess how effective dapsone was. It did appear to have some effect in reducing the blistering of her skin although it had liUle or no effect on the lesions of the buccal mucous membrane, which could be attributed to the concurrent SLH. Patients with bullous SLK may respond dramatically to dapsone or sulphapyridine. particularly those with circulating BMZ antibodies.""'*' Those patients without antibodies divide into responders to dapsone'''""^*' and those who do not."''' ~'"^^ Butlous eruptions in SLE are an uncommon manifestation of the disease rather than a co-existing tndepetident bullous dermatosis. There have been four reports of the concurrence of SLE with a bulious dermatosis in the past two decades. !n the light of recent findings a case of epidermolysis bullosa acquisita with a superimposed SlJi where the clinical criteria for both SIM and EBA were met
501
deserves attention'^'*. In that case EBA preceded the onset of SLE by S years. Two reports concerning SLE and concurrent DH have been published.^'•^'' The only publication to date concerning conctirrent SLH and linear IgA disease"' was ofa patient with Sli-^ who later developed a rash compatible with dermatitis herpetiformis and the skin histology was typical for this disorder. However. DIF showed a strong linear deposition of IgA in the normal and involved skin, with faint immunoreactivity for IgG and IgM. This patient had no evidence ofa gluten enteropathy or other circulating antibodies and did not have the Hlu^-BS haplotype. The skin lesions in this patient did not respotid well to corticosteroids but responded dramatically to dapsone. This report is the second since 198 5 ofa patient with concurrent SLH and linear IgA disease. We were also able to demonstrate circulating BMZ antibodies in our patient.
Acknowledgments We are indebted to Dr Leena Bruckner-Tuderman and Y.Seel and for technical assistance to I,Neumann and S.Wentrup. We thank Dv P.Royce for editorial help. .MR was funded by the Swiss National foundation (grant NF 32-27884.89).
References 1 C'hor/elski TF. lablonskii .S. Bcutner V. H. Atiiilt form of lineiir IgA bulluuN dermalosis. In: hnmuno[}allio!ony of the Skin (Beutner VSi. Chor/.elski TP. Bean SI-, edsl. 2nd edn. New York; )ohn Wiley & Sons. 1979: 3Ih. 1 U'oniird IN. HalTenden GP. King NP et at. linear IgA disease In adults. Hr / Dermatol 19K2; 107: JOl-6. } Smith SB. Harrist T|, Murphy CH' cl al Unear IgA bullous ilermatosis v. dermatitis herpetiformis. Arch Dgmtatol 1984: 120: 4 Oirdell II,, Fiilini 11, Hrher WN et al, Immunofn/ymiitic UibcUitig of monoclonal antibodies using imtnuni- complexes of alkalitic phospliatase and monoclonal anti-ulkiiline pho.sphatase (APAAP complexes). / HisttHheni Cytmhem 1984; J2: 219-29. 5 (Gammon WR. Briggaman KA. Inman AOcfftJ. Pifferentiating antilamina lucidit and anti-sublamina densji anti-BMZ antibtxiies by indirect immunolluorescente on I •() M sixliLim chloride-separated skin. / /rtm( Dermatol I9S4: 82: I 39-44. h TanKM.C'ohen AS. l-riL'sircid/. Tbe 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982:25: 1271-7. 7 Leonard IN. Haffenden CP. Unsworth t)l etal [-.vidt-nce that ihe IgA in patients witb linear IgA disease is qualitatively different from tbat of palienls with dermatitis herpetiformis. Br / Dermalol 1984; 110: J 1 5 - 2 I . 8 Mobacken H. Kastrup W. Ljunghall K et al linear IgA dermatosis:
502
4
10
II
12
!!
14
15
Ifi
M.LAU et al.
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