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Presentation

History

Some patients note a prolonged period of prodromal itching or transient pruritus or burning before lesions appear. Patients with ocular manifestations may complain of pain, grittiness, or discharge.

Bullae may be chronic, or lesions may appear acutely, as seen in drug-induced disease. Rash latency in vancomycin-induced cases of linear IgA dermatosis ranges from 1-13 days after the first dose. Review of medication exposures and delineation of the drug timeline are crucial in identifying potential inciting agents.

Physical Examination

The classic primary lesions of linear IgA dermatosis are clear and/or hemorrhagic round or oval vesicles or bullae on normal, erythematous, or urticarial skin. Cutaneous manifestations may also include erythematous plaques, blanching macules and papules, fixed drug eruption–like dusky patches with central violaceous changes,^[20]or targetoid erythema multiforme–like lesions. The diagnosis is not dependent on the presence of vesicles and/or bullae and a morbilliform variant has been described.^[21]

Bullae may be discrete or arranged in a herpetiform pattern, often described as the cluster of jewels sign. Alternatively, vesicles and bullae may be seen at the edge of annular or polycyclic lesions, the appearance of which has been described as the string of beads sign (see the images below). Rarely, linear IgA dermatosis may exhibit an isomorphic phenomenon.^[22]

Annular lesions demonstrating the string of beads sign.

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Bullous lesions of the palmar surface in an elderly man with vancomycin-induced linear immunoglobulin A (IgA) dermatosis.

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The distribution of linear IgA dermatosis differs between adults and children. Lesions in children are typically localized to the lower abdomen and anogenital areas, with frequent involvement of the perineum (see the image below). Other sites of involvement include the feet, hands, and face, particularly the perioral area. In adults, the trunk and the limbs are most commonly affected. In adults, involvement of the perineum and the perioral area is less common than in children. Lesions in both children and adults may be distributed symmetrically or asymmetrically. Dermatitis herpetiformis–like involvement of the extensor surfaces of the knees and the elbows is seen infrequently. Crusts, excoriations, erosions, or ulcers may be present.

Bullous lesions on the genital area in a child with linear IgA dermatosis.

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Oral manifestations are common in children and adults with linear IgA dermatosis. Oral lesions include vesicles, ulcerations, erythematous patches, erosions, desquamative gingivitis, or erosive cheilitis, and they may precede skin lesions.^[23]

Both children and adults frequently complain of ocular symptoms, such as grittiness, burning, or discharge. Ophthalmologic findings even in the absence of ocular complaints may include subconjunctival fibrosis, shrinkage of the fornices, symblepharon formation, and cicatricial entropion with trichiasis (see the image below).^[24]

Persons with linear immunoglobulin A (IgA) dermatosis may present with prominent ocular signs and symptoms.

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Differential Diagnoses

Zone JJ, Taylor TB, Meyer LJ, Petersen MJ. 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 Mar. 110(3):207-10. [QxMD MEDLINE Link].
Zone JJ, Pazderka Smith E, Powell D, Taylor TB, Smith JB, Meyer LJ. Antigenic specificity of antibodies from patients with linear basement membrane deposition of IgA. Dermatology. 1994. 189 Suppl 1:64-6. [QxMD MEDLINE Link].
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 Nov. 149(11):1308-13. [QxMD MEDLINE Link].
Zenke Y, Nakano T, Eto H, Koga H, Hashimoto T. A case of vancomycin-associated linear IgA bullous dermatosis and IgA antibodies to the a3 subunit of laminin-332. Br J Dermatol. 2014 Apr. 170(4):965-9. [QxMD MEDLINE Link].
Nousari HC, Kimyai-Asadi A, Caeiro JP, Anhalt GJ. Clinical, demographic, and immunohistologic features of vancomycin-induced linear IgA bullous disease of the skin. Report of 2 cases and review of the literature. Medicine (Baltimore). 1999 Jan. 78(1):1-8. [QxMD MEDLINE Link].
Nartker N, Kudlak N, Crowe D. Linear IgA bullous dermatosis protracted by vancomycin-loaded bone cement. JAAD Case Rep. 2019 Mar. 5 (3):234-236. [QxMD MEDLINE Link].
Riemenschneider K, Diiorio DA, Zic JA, Livingood MR, Fine JD, Powers JG, et al. Drug-induced linear IgA bullous dermatosis in a patient with a vancomycin-impregnated cement spacer. Cutis. 2018 Apr. 101 (4):293-296. [QxMD MEDLINE Link].
Sanke S, Kumar A, Chander R. Linear IgA bullous dermatosis induced by diclofenac sodium. Indian J Dermatol Venereol Leprol. 2018 Jul-Aug. 84 (4):496-497. [QxMD MEDLINE Link].
Hoffmann J, Hadaschik E, Enk A, Stremmel W, Gauss A. Linear IgA Bullous Dermatosis Secondary to Infliximab Therapy in a Patient with Ulcerative Colitis. Dermatology. 2015. 231 (2):112-5. [QxMD MEDLINE Link].
Jonna S, Neiders M, Lakshmanan S, Khan A, DeKlotz T, Lanasa D, et al. Linear IgA Disease of the Gingiva Following Nivolumab Therapy. J Immunother. 2019 Nov/Dec. 42 (9):345-347. [QxMD MEDLINE Link].
Alberta-Wszolek L, Mousette AM, Mahalingam M, Levin NA. Linear IgA bullous dermatosis following influenza vaccination. Dermatol Online J. 2009 Nov 15. 15(11):3. [QxMD MEDLINE Link].
Yhim HY, Kwon DH, Lee NR, Song EK, Yim CY, Kwak JY. Linear IgA bullous dermatosis following autologous PBSC transplantation in a patient with non-Hodgkin's lymphoma. Bone Marrow Transplant. 2010 Apr 12. [QxMD MEDLINE Link].
Godfrey K, Wojnarowska F, Leonard J. Linear IgA disease of adults: association with lymphoproliferative malignancy and possible role of other triggering factors. Br J Dermatol. 1990 Oct. 123(4):447-52. [QxMD MEDLINE Link].
Colmant C, Camboni A, Dekeuleneer V, Marot L, Dachelet C, Baeck M. Linear IgA dermatosis in association with angioimmunoblastic T-cell lymphoma infiltrating the skin: A case report with literature review. J Cutan Pathol. 2020 Mar. 47 (3):251-256. [QxMD MEDLINE Link].
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 Jan. 131(1):48-52. [QxMD MEDLINE Link].
Kharfi M, Khaled A, Karaa A, Zaraa I, Fazaa B, Kamoun MR. Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children. Dermatol Online J. 2010 Jan 15. 16(1):2. [QxMD MEDLINE Link].
Chanal J, Ingen-Housz-Oro S, Ortonne N, et al. Linear IgA bullous dermatosis: comparison between the drug-induced and spontaneous forms. Br J Dermatol. 2013 Nov. 169(5):1041-8. [QxMD MEDLINE Link].
Wojnarowska F, Marsden RA, Bhogal B, Black MM. 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 Nov. 19(5 Pt 1):792-805. [QxMD MEDLINE Link].
Collier PM, Kelly SE, Wojnarowska F. Linear IgA disease and pregnancy. J Am Acad Dermatol. 1994 Mar. 30(3):407-11. [QxMD MEDLINE Link].
Bakke JR, Chantara A, Cash J, Fisher KR. Vancomycin-induced linear IgA bullous dermatosis presenting as generalized fixed drug eruption. J Cutan Pathol. 2019 Dec. 46 (12):979-981. [QxMD MEDLINE Link].
Billet SE, Kortuem KR, Gibson LE, El-Azhary R. A morbilliform variant of vancomycin-induced linear IgA bullous dermatosis. Arch Dermatol. 2008 Jun. 144(6):774-8. [QxMD MEDLINE Link].
Choudhry SZ, Kashat M, Lim HW. Vancomycin-induced linear IgA bullous dermatosis demonstrating the isomorphic phenomenon. Int J Dermatol. 2015 Nov. 54 (11):1211-3. [QxMD MEDLINE Link].
Chan LS, Regezi JA, Cooper KD. Oral manifestations of linear IgA disease. J Am Acad Dermatol. 1990 Feb. 22(2 Pt 2):362-5. [QxMD MEDLINE Link].
Aultbrinker EA, Starr MB, Donnenfeld ED. Linear IgA disease. The ocular manifestations. Ophthalmology. 1988 Mar. 95(3):340-3. [QxMD MEDLINE Link].
El-Domyati M, Abdel-Wahab H, Ahmad H. Immunohistochemical localization of basement membrane laminin 5 and collagen IV in adult linear IgA disease. Int J Dermatol. 2015 Aug. 54 (8):922-8. [QxMD MEDLINE Link].
Tomida E, Kato Y, Ozawa H, Hasegawa H, Ishii N, Hashimoto T, et al. Causative drug detection by drug-induced lymphocyte stimulation test in drug-induced linear IgA bullous dermatosis. Br J Dermatol. 2015 Aug 12. [QxMD MEDLINE Link].
Krejci-Manwaring J, West DA, Tonkovic-Capin V. If at first you don't succeed: a difficult case of Linear IgA. Dermatol Online J. 2009 Sep 15. 15(9):16. [QxMD MEDLINE Link].
Tieppo Francio V, Towery C, Davani S, Allen T, Brown TL. Previously misdiagnosed linear IgA dermatosis resolved with dapsone. BMJ Case Rep. 2018 Apr 25. 2018:[QxMD MEDLINE Link].
Jablonska S, Chorzelski TP, Rosinska D, Maciejowska E. Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood). Clin Dermatol. 1991 Jul-Sep. 9(3):393-401. [QxMD MEDLINE Link].
Yang Z, Liu ZH, Sun CQ, Shen H. Successful treatment of a case of idiopathic linear IgA bullous dermatosis with oral sulfasalazine. Dermatol Ther. 2020 Jan 2. e13210. [QxMD MEDLINE Link].
Maalouf NS, Hanna D. Linear IgA bullous dermatosis successfully treated with omalizumab: A case report. JAAD Case Rep. 2019 Nov. 5 (11):966-969. [QxMD MEDLINE Link].
Shan XF, Zhang FR, Tian HQ, Wang N, Zhou SJ, Wang GJ. A case of linear IgA dermatosis successfully treated with tetracycline and niacinamide. Int J Dermatol. 2015 Dec 23. [QxMD MEDLINE Link].
Lozinski A, Baum S, Sagi L, Volkov A, Trau H, Barzilai A. [Rituximab (Mabthera) for treatment of rare autoimmune bullous skin disorders]. Harefuah. 2012 Oct. 151 (10):562-5, 606. [QxMD MEDLINE Link].
Norris IN, Haeberle MT, Callen JP, Malone JC. Generalized linear IgA dermatosis with palmar involvement. Dermatol Online J. 2015 Sep 17. 21 (9):[QxMD MEDLINE Link].
Kaya İslamoğlu ZG, Akyürek FT. A case of recalcitrant linear IgA bullous dermatosis: Successfully treated with rituximab. Dermatol Ther. 2019 May. 32 (3):e12911. [QxMD MEDLINE Link].
Pinard C, Hebert V, Lecuyer M, Sacre L, Joly P. Linear IgA bullous dermatosis treated with rituximab. JAAD Case Rep. 2019 Feb. 5 (2):124-126. [QxMD MEDLINE Link].
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Media Gallery

Annular lesions demonstrating the string of beads sign.
Bullous lesions on the genital area in a child with linear IgA dermatosis.
Persons with linear immunoglobulin A (IgA) dermatosis may present with prominent ocular signs and symptoms.
Neutrophilic microabscesses in linear immunoglobulin A (IgA) dermatosis.
Bullous lesions of the palmar surface in an elderly man with vancomycin-induced linear immunoglobulin A (IgA) dermatosis.

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Author

Mark Tye Haeberle, MD Assistant Clinical Faculty, Division of Dermatology, University of Louisville School of Medicine

Mark Tye Haeberle, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society of Dermatopathology, International Society of Dermatopathology

Disclosure: Received income in an amount equal to or greater than $250 from: UpToDate.

Coauthor(s)

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Russell Hall, MD J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Federation for Medical Research, American Society for Clinical Investigation, Society for Investigative Dermatology

Disclosure: Received consulting fee from Novan for consulting; Received consulting fee from Stieffel, a GSK company for consulting; Received salary from Society for Investigative Dermatology for board membership.

Acknowledgements

Peter A Klein, MD Associate Professor, Department of Dermatology, University Hospital, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

Linear IgA Dermatosis Clinical Presentation

History

Physical Examination

References

Tables

Contributor Information and Disclosures

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