Bullae are elevated, fluid-filled blisters ≥ 10 mm in diameter.
Linear IgA disease has 2 main clinical variants—bullous disease of childhood and adult linear IgA disease. Although they vary clinically in minor ways, their immunofluorescence patterns are identical. The IgA autoantibodies target several antigens within the dermal–epidermal junction.
Infections and penicillins trigger many childhood and adult cases (1 General reference Linear immunoglobulin A (IgA) disease is an uncommon bullous disease distinguished from bullous pemphigoid and dermatitis herpetiformis by linear deposits of IgA in the basement membrane zone... read more ). Vancomycin, diclofenac, nonsteroidal anti-inflammatory drugs (NSAIDs), captopril, and lithium also have been suggested as causes. Risk of linear IgA disease is increased in patients who have inflammatory bowel disease Overview of Inflammatory Bowel Disease Inflammatory bowel disease (IBD), which includes Crohn disease and ulcerative colitis, is a relapsing and remitting condition characterized by chronic inflammation at various sites in the gastrointestinal... read more (possibly with a related pathophysiology that involves a generation of autoantibodies) or lymphoproliferative cancers (in adults) but not other autoimmune disorders.
General reference
1. Khan M, Park L, Skopit S: Management options for linear immunoglobulin A (IgA) bullous dermatosis: A literature review. Cureus 15(3):e36481, 2023. doi: 10.7759/cureus.36481
Symptoms and Signs of Linear IgA Disease
In linear IgA disease, vesicular or bullous skin lesions occur frequently in a clustered (herpetiform) arrangement. In younger children, the face and perineum are often involved, and spread to the limbs, trunk, hands, feet, and scalp is common. In adults, the trunk is almost always involved, and the scalp, face, and limbs are often involved. Lesions are often pruritic and may burn. Mucosal involvement is common in both age groups; milia (superficial epidermal inclusion cysts) are not characteristic.
Diagnosis of Linear IgA Disease
Skin biopsy and direct immunofluorescence
Diagnosis of linear immunoglobulin A disease is by skin biopsy and direct immunofluorescence. The histologic features are not specific, but direct immunofluorescence shows IgA deposited along the basement membrane zone in a linear fashion.
Treatment of Linear IgA Disease
Withdrawal of causative medications
For mild disease, topical corticosteroids
For children, penicillin or erythromycin
Drug-induced disease may be treated solely with withdrawal of the causative medication.
Mild disease can be treated with topical corticosteroids. Oral penicillin (or erythromycin) can be used in children (1 Treatment reference Linear immunoglobulin A (IgA) disease is an uncommon bullous disease distinguished from bullous pemphigoid and dermatitis herpetiformis by linear deposits of IgA in the basement membrane zone... read more ). Dapsone and sulfonamides (using doses and precautions similar to those for dermatitis herpetiformis Treatment Dermatitis herpetiformis is an intensely pruritic, chronic, autoimmune, papulovesicular cutaneous eruption strongly associated with celiac disease. Typical findings are clusters of intensely... read more ) and colchicine are alternatives for all ages. Often the cutaneous lesions respond before the mucosal lesions. Spontaneous remission occurs in most patients after 3 to 6 years.
Treatment reference
1. Khan M, Park L, Skopit S: Management options for linear immunoglobulin A (IgA) bullous dermatosis: A literature review. Cureus 15(3):e36481, 2023. doi: 10.7759/cureus.36481
Key Points
Linear immunoglobulin A (IgA) disease is an uncommon bullous disease with manifestations similar to those of bullous pemphigoid Bullous Pemphigoid Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in older adults. Mucous membrane involvement is rare. Diagnosis is by skin biopsy... read more and dermatitis herpetiformis Dermatitis Herpetiformis Dermatitis herpetiformis is an intensely pruritic, chronic, autoimmune, papulovesicular cutaneous eruption strongly associated with celiac disease. Typical findings are clusters of intensely... read more .
The trigger is often an infection or medication.
Skin biopsy and direct immunofluorescence can help confirm the diagnosis.
Treat by withdrawing any causative medication and sometimes using topical corticosteroids and, in children, erythromycin.
Drugs Mentioned In This Article
Drug Name | Select Trade |
---|---|
vancomycin |
FIRVANQ, Vancocin, Vancocin Powder, VANCOSOL |
diclofenac |
Aspercreme Arthritis Pain Reliever, Cambia, Cataflam, Diclofono, DICLOPREP, DICLOVIX, Dyloject, Flector, INFLAMMA-K , Licart, Lofena, Motrin Arthritis Pain, PENNSAID, PROFINAC, Solaravix, Solaraze, ValcoPrep-100, VennGel One, Voltaren Arthritis, Voltaren Delayed-Release, Voltaren Gel, Voltaren Ophthalmic, Voltaren-XR, VOPAC MDS, Xrylix II, Zipsor, Zorvolex |
captopril |
Capoten |
lithium |
Eskalith, Eskalith CR, Lithobid |
erythromycin |
A/T/S, Akne-mycin, E.E.S., Emcin Clear , EMGEL, E-Mycin, ERYC, Erycette, Eryderm , Erygel, Erymax, EryPed, Ery-Tab, Erythra Derm , Erythrocin, Erythrocin Lactobionate, Erythrocin Stearate, Ilosone, Ilotycin, My-E, PCE, PCE Dispertab , Romycin, Staticin, T-Stat |
dapsone |
Aczone |
colchicine |
ColciGel, Colcrys , GLOPERBA, LODOCO, MITIGARE |