Linear IgA bullous dermatosis

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Abstract


Aim: to present a clinical case of linear IgA bullous dermatosis.

Materials and methods. A 44 years old patient complaining of skin rashes on the trunk and extremities, accompanied by severe itching was examined. We carried out a morphological investigation of biopsy samples derived from the lesion and apparently unaffected skin areas using the method of indirect immunofluorescence.

Results. The patient having rashes in the form of multiple vesicles and small bubbles with a tight cover, which had been grouped into figures resembling pearl necklaces, demonstrated the presence of focal subepidermal cracks (subepidermal bubble in one location), as well as a linear deposition of IgA along the epidermal basement membrane. According to the clinical picture and following the histological and immunofluorescent investigation of skin biopsies, the patient was diagnosed with linear IgA bullous dermatosis. Lesion regression was achieved as a result of systemic therapy with prednisolone at a dose of 50 mg per day.

Conclusion. The diagnosis of linear IgA bullous dermatosis should be made on the basis of skin biopsy investigation by the method of indirect immunofluorescence. Systemic glucocorticosteroids are seen as an effective approach to the treatment of such patients.


About the authors

L. F. Znamenskaya

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Author for correspondence.
Email: fake@neicon.ru

Russian Federation

Dr. Sci. (Med.), Leading Researcher, Department of Dermatology,

Korolenko str., 3, bldg 6, Moscow, 107076

V. V. Chikin

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: platonova1105@gmail.com

Russian Federation

Dr. Sci. (Med.), Senior Researcher, Department of Dermatology,

Korolenko str., 3, bldg 6, Moscow, 107076

M. A. Nefedova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Russian Federation

Junior Researcher, Department of Dermatology,

Korolenko str., 3, bldg 6, Moscow, 107076

References

  1. Egan C. A., Zone J. J. Linear IgA bullous dermatosis. Int J Dermatol. 1999;38:818–827.
  2. Zillikens D., Wever S., Roth A. et al. Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol. 1995;131:957–958.
  3. Bernard P., Vaillant L., Labeille B. et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol. 1995;131:48–52.
  4. Nanda A., Dvorak R., Al-Sabah H., Alsaleh Q. A. Linear IgA bullous disease of childhood: an experience from Kuwait. Pediatr Dermatol. 2006;23:443–447.
  5. Chaudhari S., Mobini N. Linear IgA Bullous Dermatosis: A rare clinicopathologic entity with an unusual presentation. J Clin Aesthet Dermatol. 2015;8(10):43–46.
  6. Lings K., Bygum A. Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark. Acta Derm Venereol. 2015;95:466–471.
  7. Fortuna G., Marinkovich M. P. Linear immunoglobulin A bullous dermatosis. Clin Dermatol. 2012;30:38–50.
  8. Mintz E. M., Morel K. D. Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin. 2011;29(3):459–462.
  9. Wojnarowska F., Marsden R. A., Bhogal B., Black M. M. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol. 1988;19(5 Pt 1):792–805.
  10. Kanwar A. J., Sandhu K., Handa S. Chronic bullous dermatosis of childhood in north India. Pediatr Dermatol. 2004;21:610–612.
  11. Kenani N., Mebazaa A., Denguezli M. et al. Childhood linear IgA bullous dermatosis in Tunisia. Pediatr Dermatol. 2009;26:28–33.
  12. Kharfi M., Khaled A., Karaa A. et al. Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children. Dermatol Online J. 2010;16:2.
  13. Carpenter S., Berg D., Sidhu-Malik N. et al. Vancomycin-associated linear IgA dermatosis. A report of three cases. J Am Acad Dermatol. 1992;26:45–48.
  14. Fortuna G., Salas-Alanis J. C., Guidetti E., Marinkovich M. P. A critical reappraisal of the current data on drug-induced linear immunoglobulin A bullous dermatosis: a real and separate nosological entity? J Am Acad Dermatol. 2012;66(6):988–994.
  15. Ho J. C., Ng P. L., Tan S. H., Giam Y. C. Childhood linear IgA bullous disease triggered by amoxicillin-clavulanic acid. Pediatr Dermatol. 2007;24(5):E40–E43.
  16. Nousari H. C., Costarangos C., Anhalt G. J. Vancomycin-associated linear IgA bullous dermatosis. Ann Intern Med. 1998;129(6):507–508.
  17. Navi D., Michael D. J., Fazel N. Drug-induced linear IgA bullous dermatosis. Dermatol Online J. 2006;12(5):12.
  18. McEvoy M. T., Connolly S. M. Linear IgA dermatosis: association with malignancy. J Am Acad Dermatol. 1990;22:59–63.
  19. Van der Waal R. I., van de Scheur M. R., Pas H. H. et al. Linear IgA bullous dermatosis in a patient with renal cell carcinoma. Br J. Dermatol. 2001;144:870–873.
  20. Shipman A. R., Reddy H., Wojnarowska F. Association between the subepidermal autoimmune blistering diseases linear IgA disease and the pemphigoid group and inflammatory bowel disease: two case reports and literature review. Clin Exp. Dermatol. 2012;37:461–468.
  21. Mavragani C. P., Asvesti K., Moutsopoulos H. M. Linear IgA dermatosis in a patient with primary Sjogren’s syndrome. Rheumatology. 2013;52:403–404.
  22. Gottlieb J., Ingen-Housz-Oro S., Alexandre M. et al. Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case-series of 72 adults. Br J Dermatol. 2017;177 (1):212–222.
  23. Horiguchi Y., Ikoma A., Sakai R. et al. Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan. J Dermatol. 2008;35 (11):737–743.
  24. Taniguchi T., Maejima H., Saito N. et al. Case of linear IgA bullous dermatosis-involved ulcerative colitis. Inflamm Bowel Dis. 2009;15(9):1284– 1285.
  25. Paige D. G., Leonard J. N., Wojnarowska F. et al. Linear IgA disease and ulcerative colitis. Br J Dermatol. 1997;136(5):779–782.
  26. Cooke N., Jenkinson H., Wojnarowska F. et al. Coexistence of psoriasis and linear IgA disease in a patient with recent herpes zoster infection. Clin Exp Dermatol. 2005;30(6):643–645.
  27. Takagi Y., Sawada S.,Yamauchi M. et al. Coexistence of psoriasis and linear IgA bullous dermatosis. Br J Dermatol. 2000;142(3):513–516.
  28. Tobon G. J., Toro C. E., Bravo J. C. et al. Linear IgA bullous dermatosis associated with systemic lupus erythematosus: a case report. Clin Rheumatol. 2008;27(3):391–393.
  29. Collier P. M., Wojnarowska F., Welsh K. et al. Adult linear IgA disease and chronic bullous disease of childhood: the association with human lymphocyte antigens Cw7, B8, DR3 and tumour necrosis factor influences disease expression. Br J Dermatol. 1999;141(5):867–875.
  30. Hendrix J. D., Mangum K. L., Zone J. J., Gammon W. R. Cutaneous IgA deposits in bullous diseases function as ligands to mediate adherence of activated neutrophils. J Invest Dermatol. 1990;94(5):667–672.
  31. Spiegelberg H. L., Lawrence D. A., Henson P. Cytophilic properties of IgA to human neutrophils. Adv Exp Med Biol. 1974;45(0):67–74.
  32. Fanger M. W., Shen L., Pugh J., Bernier G.M. Subpopulations of human peripheral granulocyes and monocytes express receptors for IgA. Proc Natl Acad Sci USA. 1980;77(6):3640–3644.
  33. Fanger M.W., Pugh J., Bernier G.M. The specificity of receptors for IgA on human peripheral polymorphonuclear cells and monocytes. Cell Immunol. 1981;60(2):324–334.
  34. Fanger M. W., Goldstine S. N., Shen L. Cytofluorographic analysis of receptors for IgA on human polymorphonuclear cells and monocytes and the correlation of receptor expression with phagocytosis. Mol Immunol. 1983;20(9):1019–1027.
  35. Fanger M. W., Goldstine S. N., Shen L. The properties and role of receptors for IgA on human leukocytes. Ann N Y Acad Sci. 1983;409:552– 563.
  36. Sibille Y., Delacroix D.L., Merill W.W. et al. IgA-induced chemokinesis of human polymorphonuclear neutrophils: requirement of their Fc-alpha receptor. Mol Immunol. 1987;24(6):551–559.
  37. Weisbart R. H., Kacena A., Schuh A., Golde D. W. GM-CSF induces human neutrophil IgA-mediated phagocytosis by an IgA Fc receptor activation mechanism. Nature. 1988;332(6165):647–648.
  38. Egan C. A., Martineau M. R., Taylor T. B. et al. IgA antibodies recognizing LABD97 are predominantly IgA1 subclass. Acta Derm Venereol. 1999;79(5):343–346.
  39. Zone J. J., Taylor T. B., Kadunce D. P., Meyer L. J. Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis. J Clin Invest. 1990;85:812–820.
  40. Zone J. J., Taylor T. B., Meyer L. J., Petersen M. J. The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2. J Invest Dermatol. 1998;110:207–210.
  41. Marinkovich M. P., Taylor T. B., Keene D. R. et al. LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring filament protein synthesized by epidermal cells. J Invest Dermatol. 1996;106:734–738.
  42. Guide S. V., Marinkovich K. D. Linear IgA bullous dermatosis. Clin Dermatol. 2001;19(6):719–727.
  43. Chorzelski T. P., Jablonska S., Maciejowska E. Linear IgA bullous dermatosis of adults. Clin Dermatol. 1991;9(3):383–392.
  44. Zillikens D. BP180 as the common autoantigen in blistering diseases with different clinical phenotypes. Keio J Med. 2002;51:21–28.
  45. Hirako Y., Nishizawa Y., Sitaru C. et al. The 97-kDa (LABD97) and 120-kDa (LAD-1) fragments of bullous pemphigoid antigen 180/type XVII collagen have different N-termini. J Invest Dermatol. 2003;121:1554–1556.
  46. Zhou S., Ferguson D. J., Allen J., Wojnarowska F. The localization of target antigens and autoantibodies in linear IgA disease is variable: correlation of immunogold electron microscopy and immunoblotting. Br J Dermatol. 1998;139:591–597.
  47. Darling T. N., Cardenas A. A., Beard J. S. et al. A child with antibodies targeting both linear IgA bullous dermatosis and bullous pemphigoid antigens. Arch Dermatol. 1995;131:1438–1442.
  48. Ghohestani R. F., Nicolas J. F., Kanitakis J., Claudy A. Linear IgA bullous dermatosis with IgA antibodies exclusively directed against the 180- or 230-kDa epidermal antigens. J Invest Dermatol. 1997;108:854–858.
  49. Tsuchisaka A., Ohara K., Ishii N. et al. Type VII collagen is the major autoantigen for sublamina densa-type linear IgA bullous dermatosis. J Invest Dermatol. 2015;135:626–629.
  50. Li X., Tsuchisaka A., Qian H. et al. Linear IgA/IgG bullous dermatosis reacts with multiple laminins and integrins. Eur J Dermatol. 2015;25:418–423.
  51. Seta V., Aucouturier F., Bonnefoy J. et al. Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA). J Am Acad Dermatol. 2016;74:1166–1172.
  52. Zillikens D., Herzele K., Georgi M. et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113:947–953.
  53. Allen J., Wojnarowska F. Linear IgA disease: the IgA and IgG response to the epidermal antigens demonstrates that intermolecular epitope spreading is associated with IgA rather than IgG antibodies, and is more common in adults. Br J Dermatol. 2003;149:977–985.
  54. Lally A., Chamberlain A., Allen J. et al. Dermal-binding linear IgA disease: an uncommon subset of a rare immunobullous disease. Clin Exp Dermatol. 2007;32:493–498.
  55. Sakaguchi M., Bito T., Oda Y. et al. Three cases of linear IgA/IgG bullous dermatosis showing IgA and IgG reactivity with multiple antigens, particularly laminin-332. JAMA Dermatol. 2013;149:1308–1313.
  56. Venning V. A. Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. Dermatol Clin. 2011;29:453–458.
  57. Leonard J. N., Haffenden G. P., Ring N. P. et al. Linear IgA disease in adults. Br J Dermatol. 1982;107:301–316.
  58. Yaoita H., Katz S. I. Immunoelectron microscopic localization of IgA in skin of patients with dermatitis herpetiformis. J Invest Dermatol. 1976;67:502–506.
  59. Pehamberger H., Konrad K., Stingl G., Holubar K. Immunoelectron microscopy of linear dermatitis herpetiformis: report of a case. Acta Derm Venereol. 1977;57:462–465.
  60. Dabrowski J., Chorzelski T. P., Jablońska S. et al. The ultrastructural localization of IgA deposits in chronic bullous disease of childhood (CBDC). J Invest Dermatol. 1979;72:291–295.
  61. Dabrowski J., Chorzelski T., Jabłońska S. et al. Immunoelectron microscopic studies in IgA linear dermatosis. Arch Dermatol Res. 1979;265:289–298.
  62. Rantala I., Hietanen J., Soidinmäki H., Reunala T. Immunoelectron microscopic findings in oral mucosa of patients with dermatitis herpetiformis and linear IgA disease. Scand J Dent Res. 1985;93:243–248.
  63. Bhogal B., Wojnarowska F., Marsden R.A. et al. Linear IgA bullous dermatosis of adults and children: an immunoelectron microscopic study. Br J Dermatol. 1987;117:289–296.
  64. Prost C., De Leca A.C., Combemale P. et al. Diagnosis of adult linear IgA dermatosis by immunoelectron microscopy in 16 patients with linear IgA deposits. J Invest Dermatol. 1989;92:39–45.
  65. Zambruno G., Manca V., Kanitakis J. et al. Linear IgA bullous dermatosis with autoantibodies to a 290 kD antigen of anchoring fibrils. J Am Acad Dermatol. 1994;31:884–888.
  66. Chan L.S., Traczyk T., Taylor T.B. et al. Linear IgA bullous dermatosis. Characterization of a subset of patients with concurrent IgA and IgG anti-basement membrane autoantibodies. Arch Dermatol. 1995;131:1432–1437.
  67. Kárpáti S., Stolz W., Meurer M. et al. Ultrastructural immunogold studies in two cases of linear IgA dermatosis. Are there two distinct types of this disease? Br J Dermatol. 1992;127:112–118.
  68. Cauza K., Hinterhuber G., Sterniczky B. et al. Unusual clinical manifestation of linear IgA dermatosis: a report of two cases. J Am Acad Dermatol 2004;51:S112–117.
  69. Yamasaki Y., Hashimoto T., Nishikawa T. Dermatitis herpetiformis with linear IgA deposition: ultrastructural localization of in vivo bound IgA. Acta Derm Venereol. 1982;62:401–405.
  70. Collier P. M., Kelly S. E., Wojnarowska F. Linear IgA disease and pregnancy. J Am Acad Dermatol. 1994;30(3):407–411.
  71. Sobjanek M., Sokolowska-Wojdylo M., Sztaba-Kania M. et al. Clinical and immunopathological heterogeneity of 22 cases of linear IgA bullous dermatosis. J Eur Acad Dermatol Venereol. 2008;22:1131.
  72. Jabłońska S., Chorzelski T. P., Rosinska D., Maciejowska E. Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood). Clin Dermatol. 1991;9:393–401.
  73. Gluth M. B., Witman P. M., Thompson D. M. Upper aerodigestive tract complications in a neonate with linear IgA bullous dermatosis. Int J Pediatr Otorhinolaryngol. 2004;68(7):965–970.
  74. Chanal J., Ingen-Housz-Oro S., Ortonne N. et al. Linear IgA bullous dermatosis: comparison between drug-induced and spontaneous forms. Br J Dermatol. 2013;169:1041–1048.
  75. Torchia D., Caproni M., Del Bianco E. et al. Linear IgA disease presenting as prurigo nodularis. Br J Dermatol. 2006;155(2):479–480.
  76. Waldman M. A., Black D. R., Callen J. P. Vancomycin-induced linear IgA bullous disease presenting as toxic epidermal necrolysis. Clin Exp Dermatol. 2004;29(6):633–636.
  77. Billet S. E., Kortuem K. R., Gibson L. E. et al. A morbilliform variant of vancomycin-induced linear IgA bullous dermatosis. Arch Dermatol. 2008;144(6):774–778.
  78. Armstrong A. W., Fazeli A., Yeh S. W. et al. Vancomycin-induced linear IgA disease manifesting as bullous erythema multiforme. J Cutan Pathol. 2004;31(5):393–397.
  79. Khan I., Hughes R., Curran S. et al. Drug-associated linear IgA disease mimicking toxic epidermal necrolysis. Clin Exp Dermatol. 2009;34(6):715–717.
  80. Collier P. M., Wojnarowska F., Millard P. R. Variation in the deposition of the antibodies at different anatomical sites in linear IgA disease of adults and chronic bullous disease of childhood. Br J Dermatol. 1992;127(5):482–484.
  81. Kersting E., Goebeler M., Hamm H. et al. Subepidermal blasenbildende Autoimmundermatose mit linearen Ablagerungen von IgA und IgG. Hautarzt. 2000;51:340–344.
  82. Watanabe M., Tsunoda T., Tagami H. A subepidermal blistering dermatosis associated with coexistent IgG and IgA anti-dermal basement membrane zone antibodies; demonstration of IgG antibodies reactive against a 200-kDa dermal antigen. Eur J Dermatol. 2002;12:603–606.
  83. Shimizu S., Natsuga K., Shinkuma S. et al. Localized linear IgA/ IgG bullous dermatosis. Acta Derm Venereol. 2010;90:621–624.
  84. Kakugawa T., Tomimura S., Hayashi T. et al. Interstitial pneumonia associated with linear immunoglobulin A/immunoglobulin G bullous dermatosis. Respiration. 2013;86:347–351.
  85. Kromminga A., Scheckenbach C., Georgi M. et al. Patients with bullous pemphigoid and linear IgA disease show a dual IgA and IgG autoimmune response to BP180. J Autoimmun. 2000;15:293–300.
  86. Sandoval M., Farias M.M., Gonzalez S. Linear IgA bullous dermatosis: report of five cases in Chile. Int J Dermatol. 2012;51:1303– 1306.
  87. Ingen-Housz-Oro S., Bernard P., Bedane C. et al. Linear IgA dermatosis. Guidelines for the diagnosis and treatment. Centres de référence des maladies bulleuses auto-immunes. Société Française de Dermatologie. 2011;138(3):267–270.
  88. Franzke C.-W., Tasanen K., Schäcke H. et al. Transmembrane collagen XVII, an epithelial adhesion protein, is shed from the cell surface by ADAMs. EMBO J. 2002;21:5026–5035.

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