Presentation
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]
Entire Body System
- Cushingoid
In 1997, an eight and a half years old male child weighing 30 kg, diagnosed with LABD, on treatment with 20 mg prednisolone for a year, presented with active disease and Cushingoid features [Figure 1]. [ijdvl.com]
- 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]
Gastrointestinal
- 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]
Skin
- 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]
Musculoskeletal
- Leg Pain
After a one-year complete disease-free period, he developed intractable leg pain which was unresponsive to non-steroidal anti-inflammatory agents. On discontinuing, his pains disappeared and reappeared on starting thalidomide again. [ijdvl.com]
Workup
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]
Urine
- 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]
Microbiology
- 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 Type
- 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]
Treatment
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]
Ig in patients with LABD resistant to conventional treatment or having experienced complications as a result of conventional treatment has a reasonable chance of producing remission. [nature.com]
Prognosis
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]
What is the prognosis? DISCUSSION Linear IgA bullous dermatosis (LABD) is a mucocutaneous autoimmune disease that may be drug-induced or idiopathic. [ncbi.nlm.nih.gov]
Etiology
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]
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]
Etiology and Pathophysiology The underlying mechanism is not known. Like other autoimmune blistering disorders, it is characterized by autoantibody formation against structural proteins in the epidermis or BMZ. [unboundmedicine.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]
Epidemiology
[…] 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]
Epidemiology Adult linear IgA disease is either idiopathic or drug-induced, with most patients are >60 years. [unboundmedicine.com]
Epidemiologically, there is an unestablished predominance of race or sex. 1, 2 LABD has two clinical variants. [ncbi.nlm.nih.gov]
Pathophysiology
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]
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]
[…] 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]
Prevention
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]