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Linear IgA Dermatosis Clinical Presentation

  • Author: Mark Tye Haeberle, MD; Chief Editor: William D James, MD  more...
 
Updated: Jun 18, 2015
 

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.

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Physical

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, 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.[10]

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).

Annular lesions demonstrating the string of beads Annular lesions demonstrating the string of beads sign.
Bullous lesions of the palmar surface in an elderl Bullous lesions of the palmar surface in an elderly man with vancomycin-induced linear immunoglobulin A (IgA) dermatosis.

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

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.[11]

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).[12]

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

The list of agents implicated in linear IgA dermatosis continues to grow, especially with regard to medications. Many patients report prodromal events, such as illnesses or ingestion of drugs. In absent large series or a preponderance of case reports, only a subset of cases have identifiable causes. Gluten-sensitive enteropathy is not associated with linear IgA dermatosis. Various causes are discussed as follows:

  • Seventeen case reports have implicated vancomycin in linear IgA dermatosis. Of all reported causative drugs, it is the best-documented agent in the literature. [13] Other potential triggers and include the following: amiodarone, ampicillin sodium, captopril, cefamandole nafate, cyclosporine, depot sulfonamide, diclofenac, glibenclamide, interferon gamma and interleukin 2, iodine contrast agent, lithium carbonate, penicillin sodium, phenytoin sodium, somatostatin, sulfamethoxazole/trimethoprim, sulfisoxazole, topical sodium hypochlorite (1), moxifloxacin, amoxicillin-clavulanate, and vigabatrin.
  • The development of linear IgA bullous dermatosis has been reported following influenza vaccination in a 54-year-old woman. [14]
  • Preceding illnesses, such as typhoid, brucella, tuberculosis, antibiotic-treated tetanus, varicella, herpes zoster, Paecilomyces lung infection, gynecologic infections, and upper respiratory infections, have all been reported in association with linear IgA dermatosis. The significance of these associations is uncertain. Their potential role in stimulation of the IgA mucosal system has yet to be elucidated.
  • Linear IgA dermatosis associated with malignancy has been reported in as many as 5% of cases. Lymphoproliferative malignancies, specifically Hodgkin disease, non-Hodgkin lymphoma (eg, following stem cell transplantation), [15] and chronic lymphocytic leukemia have been described. [16] Linear IgA dermatosis has also been reported with solid tumors, such as bladder carcinoma. Other associated malignancies include polycythemia rubra vera, plasmacytoma, multiple myeloma, ocular melanoma, squamous cell carcinoma of the esophagus, breast carcinoma, uterine carcinoma, eccrine carcinoma, metastatic squamous cell carcinoma, colon carcinoma, thyroid carcinoma, retroperitoneal carcinoma, metastatic hypernephroma, pancreatic carcinoma, and hydatidiform mole. The validity of the association between linear IgA dermatosis and malignancy remains to be proven.
  • Because linear IgA dermatosis is itself an autoimmune disease, an association with other such disorders is interesting despite proven causality. Cases have been described in association with systemic lupus erythematosus, dermatomyositis, rheumatoid arthritis, polymyalgia rheumatica, hypothyroidism, chronic hepatitis, Crohn disease, ulcerative colitis, multiple sclerosis, acquired hemophilia, and IgA nephropathy. Again, these associations may be coincidental.
  • Linear IgA dermatosis in children has been reported in association with human leukocyte antigen B8 (HLA-B8), but the significance of this finding is unknown.
  • Pancreatic lipase deficiency has been reported once in association with linear IgA dermatosis.
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Contributor Information and Disclosures
Author

Mark Tye Haeberle, MD Resident Physician, Department of Medicine, 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 Medical Student Association/Foundation, American Society of Dermatopathology

Disclosure: Nothing to disclose.

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: Eli Lilly; XOMA; Biogen/IDEC; Novartis; Celgene<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received i do not control these accounts, but 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 i inherited these trust accounts; .

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: Nothing to disclose.

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.

References
  1. 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. [Medline].

  2. 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. [Medline].

  3. 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. [Medline].

  4. 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. [Medline].

  5. 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. [Medline].

  6. 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. [Medline].

  7. 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. [Medline].

  8. 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. [Medline].

  9. Collier PM, Kelly SE, Wojnarowska F. Linear IgA disease and pregnancy. J Am Acad Dermatol. 1994 Mar. 30(3):407-11. [Medline].

  10. 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. [Medline].

  11. Chan LS, Regezi JA, Cooper KD. Oral manifestations of linear IgA disease. J Am Acad Dermatol. 1990 Feb. 22(2 Pt 2):362-5. [Medline].

  12. Aultbrinker EA, Starr MB, Donnenfeld ED. Linear IgA disease. The ocular manifestations. Ophthalmology. 1988 Mar. 95(3):340-3. [Medline].

  13. 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. [Medline].

  14. 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. [Medline].

  15. 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. [Medline].

  16. 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. [Medline].

  17. 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. [Medline].

  18. 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. [Medline].

  19. Klein PA, Callen JP. Drug-induced linear IgA bullous dermatosis after vancomycin discontinuance in a patient with renal insufficiency. J Am Acad Dermatol. 2000 Feb. 42(2 Pt 2):316-23. [Medline].

 
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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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