We present a case of burn-induced LAD in a 48-year-old caucasian male presenting with a recent history of blistering eruption on the periphery of a cicatricial area caused by boiling methyl alcohol. [ncbi.nlm.nih.gov]

• Fever of Unknown Origin

  This entity, which is well known to dermatologists but not to internists, should be added to the extensive list of disorders than can provoke the syndrome of fever of unknown origin. [ncbi.nlm.nih.gov]

• Chronic Watery Diarrhea

  The case of a 66-year-old female patient is presented, who suffered from chronic watery diarrhea. In addition, she developed linear IgA dermatosis after oral treatment of a presumed yeast infection with nystatin. [ncbi.nlm.nih.gov]

• Erythema Annulare

  To our knowledge this is the second report of adult linear IgA dermatosis in association with erythema annulare centrifugum lesions. [ncbi.nlm.nih.gov]

• Hemorrhagic Bullae

  We report a case of a previously healthy 77-year-old man who developed a diffuse macular rash and hemorrhagic bullae on the left leg 10 days after placement of a vancomycin-impregnated cement spacer (VICS) during a revision knee arthroplasty and initiation [mdedge.com]

• Koebner Phenomenon

  Drug induced linear IgA disease with unusual features: Koebner phenomenon, local insulin sensitivity and annular blister of the nipples. Acta Dermatol venerol Croat 2008;16:215-7. 8. Patsatsi A. [jomfp.in]

Immunofluorescence studies should be made a mandatory part of the diagnostic workup in the evaluation of chronic vesiculobullous diseases. Acknowledgments We thank Dr. Jon Wagner for his contribution in language editing. [sdsjournal.com]

• Pyuria

  The initial urinalysis showed pyuria and hematuria. This, combined with the patient's symptoms of fever, prompted urine and blood culture. [ncbi.nlm.nih.gov]

• Candida

  The blood culture revealed growth of Candida albicans, which was subsequently treated with oral fluconazole (200 mg/day for 14 days). [ncbi.nlm.nih.gov]

• HLA-A1

  Five of 9 cases carried the haplotype HLA-A1, B8, DR3. In spite of a close similarity between linear IgA dermatosis and DH, differences exist which indicate discrepancies in etiopathogenesis. [ncbi.nlm.nih.gov]

• HLA-DQ2

  Symptoms are noted within one month of drug initiation and withdrawal of the offending agent does cause remission within several weeks. 22 - 24 Significant genetic associations between LABD and human leukocyte antigen (HLA) B8, HLA DR3, HLA DQ2, HLA Cw7 [ncbi.nlm.nih.gov]

One patient cleared after treatment with dapsone. The second patient did not respond to dapsone alone and various immunosuppressive treatment regimens. [ncbi.nlm.nih.gov]

The most well-recognized treatment for LABD is dapsone or, if that fails, sulfapyridine. [jamanetwork.com]

If given too late, or for too long, corticosteroids can lead to sepsis and other complications. 9, 14, 15, 23, 24, Prognosis Prompt improvement and lesions resolve spontaneously without scar after discontinuation of vancomycin. [jaoa.org]

[…] usually remits by age 13. [1] Historical Perspective Classification Pathophysiology Causes Differentiating Linear IgA bullous dermatosis from other Diseases Epidemiology and Demographics Age Gender Race Risk Factors Natural History, Complications and Prognosis [wikidoc.org]

Treatment and prognosis of LABD The diagnosis is based upon the typical clinical features and direct immunofluorescence of the skin biopsy sample to identify the autoantibodies. [news-medical.net]

Prognosis [ 9, 10 ] In children, most idiopathic cases resolve within two years. In adults the disease can be more protracted and in some cases can be refractory. LDA may improve during pregnancy. [patient.info]

[…] deficiency before dapsone is prescribed Other treatments include tetracycline antibiotics, colchicine, sulfamethoxypyridazine, prednisolone, azathioprine, mycophenolate mofetil Mucosal lesions - respond less well to treatment than cutaneous lesions Prognosis [pcds.org.uk]

The etiology of LABD is yet to be fully elucidated, but some have reported the presence of a group of autoantigens that have been found to be targets in the idiopathic form of LABD. [ncbi.nlm.nih.gov]

[…] disorder of pruritic subepidermal bullae or vesicles with linear deposits of IgA along the basement membrane Sites Thighs, buttocks, lower trunk, genital area, scalp, perioral face Mucous membrane involvement is importance because of associated scarring Etiology [pathologyoutlines.com]

[…] vancomycin and insulin, infection and may be associated with malignancy. [3], [4], [5], [7] In the present case, the reported history of hospitalization following viral fever and subsequent undefined medication could have played an etiological role. [jomfp.in]

2015 Number of Print Pages: 4 Number of Figures: 4 Number of Tables: 0 ISSN: 1018-8665 (Print) eISSN: 1421-9832 (Online) For additional information: Abstract Linear IgA bullous disease (LABD) is a rare vesiculobullous autoimmune skin disorder whose etiology [karger.com]

The etiology, cellular and molecular mechanisms, therapeutic strategies, and pathophysiology of diseases such as psoriasis, dermatitis, cutaneous lupus erythematosus, vitiligo, and scleroderma are discussed by individuals in dermatology, genetics, immunology [books.google.es]

[…] acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13. [1] Historical Perspective Classification Pathophysiology Causes Differentiating Linear IgA bullous dermatosis from other Diseases Epidemiology [wikidoc.org]

Pemphigoid gestationis (if pregnant) Pemphigus Stevens-Johnson syndrome Toxic epidermal necrolysis Monitoring Epithelial basement membrane zone IgA antibody testing or epithelial skin antibodies to monitor disease activity and response to therapy Background Epidemiology [arupconsult.com]

Epidemiology [ 2 ] This is a rare condition with an incidence in Western Europe of 0.5 per million. There are no figures for the incidence in children. Some studies have reported a slight preponderance of women. [patient.info]

Epidemiologically, there is an unestablished predominance of race or sex. 1, 2 LABD has two clinical variants. [ncbi.nlm.nih.gov]

Epidemiology Adult linear IgA disease is either idiopathic or drug-induced, with most patients are 60 years. [unboundmedicine.com]

Pathophysiology of LABD The exact mechanism involved in the loss of self-tolerance to target antigens in the basement membrane is unknown. [news-medical.net]

Childhood linear IgA disease (also known as "Chronic bullous disease of childhood") is an acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13. [1] Historical Perspective Classification Pathophysiology [wikidoc.org]

[…] colitis, Crohn disease, gastric hypochlorhydria, thyrotoxicosis, systemic lupus erythematous, dermatomyositis, rheumatoid arthritis Malignancies Lymphoma or chronic lymphocytic leukemia (rare in both) Carcinoma of the bladder, thyroid, or esophagus Pathophysiology [arupconsult.com]

The etiology, cellular and molecular mechanisms, therapeutic strategies, and pathophysiology of diseases such as psoriasis, dermatitis, cutaneous lupus erythematosus, vitiligo, and scleroderma are discussed by individuals in dermatology, genetics, immunology [books.google.es]

Risk of linear IgA disease is increased in patients who have inflammatory bowel disease (possibly with a related pathophysiology that involves a generation of autoantibodies) or lymphoproliferative cancers (in adults) but not other autoimmune disorders [merckmanuals.com]

Gender Race Risk Factors Natural History, Complications and Prognosis Diagnosis Diagnostic Criteria Symptoms Physical Examination Skin Trunk And Extremities Laboratory Findings Imaging Findings Other Diagnostic Studies Treatment Medical Therapy Surgery Prevention [wikidoc.org]

Via CS, Shustov A, Rus V, Lang T, Nguyen P, Finkelman FD: In vivo neutralization of TNF-alpha promotes humoral autoimmunity by preventing the induction of CTL. J Immunol 2001;167:6821-6826. [karger.com]

She was advised not to use vancomycin again in the future to prevent further recurrences. DISCUSSION Although drug-induced LABD is uncommon, its incidence has been steadily increasing in frequency in recent years. [ncbi.nlm.nih.gov]

[…] mycophenolic acid, which was investigated in the 1970s as an oral agent for psoriasis. 17 - 22 The drug was subsequently rediscovered in its new form, the prodrug of mycophenolic acid, and approved by the Food and Drug Administration in 1997 for the prevention [jamanetwork.com]

Some bullous diseases have serious sequelae, necessitating early treatment and intervention to prevent further morbidity or mortality. [aafp.org]