Basement Membrane Zone Antibody-Associated Diseases
Pemphigoid
- Bullous pemphigoid (BP)
- Mucous membrane pemphigoid (MMP)
- Cicatricial pemphigoid
- Ocular cicatricial pemphigoid (OCP)
- Brunsting-Perry pemphigoid
- Anti-laminin-332 pemphigoid
- (formerly anti-epiligrin pemphigoid and anti-laminin 5 pemphigoid)
- Anti-p200 (laminin gamma-1) pemphigoid
- Pemphigoid gestationis (formerly herpes gestationis)
- Lichen planus pemphigoides
- Other pemphigoid variants
- Infantile pemphigoid
- Localized pemphigoid
- Vegetating pemphigoid
- Vulvar, prepubertal pemphigoid
- Pemphigoid herpetiformis
- Pemphigoid nodularis
- Dyshidrosiform pemphigoid
- Drug-induced pemphigoid
- Urticarial pemphigoid
- Eczematous pemphigoid
- Generalized pruritus pemphigoid
- Epidermolysis bullosa acquisita (EBA)
- Linear IgA disease (LAD)
- Linear IgA bullous dermatosis (LABD)
- Chronic bullous disease of childhood
- Drug-induced linear IgA disease
- Linear IgA / IgG bullous dermatosis
Recommended testing
- Biopsy (perilesional) direct immunofluorescence (DIF) for diagnosis
- Serum indirect immunofluorescence and ELISAs for diagnosis and disease monitoring
- Biopsy (lesional, newly developed, intact skin / mucosa) DIF for diagnosis
- Biopsy (lesional, newly developed, intact skin / mucosa) for eosinophil granule major basic protein 1 (eMBP1)
Cell Surface / Intercellular Substance (ICS) Antibody-Associated Diseases
Pemphigus, IgG variants
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Pemphigus vulgaris
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Pemphigus vegetans
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Pemphigus foliaceus
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Endemic pemphigus
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Fogo selvagem
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Pemphigus erythematosus, alias Senear-Usher syndrome
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Pemphigus herpetiformis
IgA pemphigus
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Subcorneal pustular dermatosis (SPD)
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Intraepidermal neutrophilic IgA dermatosis (IEN)
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IgA pemphigus vulgaris
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IgA pemphigus vegetans
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Unclassified IgA pemphigus
Intercellular IgG / IgA dermatosis
Paraneoplastic pemphigus (PNP), alias paraneoplastic autoimmune multiorgan syndrome (PAMS)
Mixed, Overlapping, and Atypical Epithelial Antibody-Associated Diseases
- Drug reactions including with immune-modulating agents/immune-checkpoint inhibitors1,2 (consider 3 if nonblistering)
- Concurrent disease presentations with co-dominant autoantibody expression1,2
- Incidental cross-over antibodies with dominant features of one immunobullous disease1,2
- Autoimmune diseases in patients who are multiple autoantibody producers3
- Nonspecific expression of one or more of the antibodies
- Associated with paraneoplastic conditions/malignancy including paraneoplastic pemphigus and others1,2
Miscellaneous Dermatologic Diseases with Immunopathologic Test Findings
Dermatitis herpetiformis, aliases Duhring disease, Brocq-Duhring Disease1,2
Celiac disease, aliases celiac sprue, gluten-sensitive enteropathy, with and without associated dermatitis herpetiformis
Lichen planus3
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Lichen planus pemphigoides1,2
Lichenoid reactions, other3
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Drug reactions
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Erythema multiforme
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Steven’s Johnson/toxic epidermal necrolysis
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Connective tissue diseases including lupus erythematosus and dermatomyositis
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Graft versus host disease
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Paraneoplastic pemphigus1,2
Lupus erythematosus3
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Bullous lupus erythematosus
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Cutaneous lupus erythematosus
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Subacute cutaneous lupus erythematosus
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Neonatal lupus erythematosus
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Drug-induced lupus erythematosus
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Other disorders with lupus band
Mixed, overlap, and undifferentiated connective tissue disease3
Vasculitis3
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Immune complex, small-vessel, leukocytoclastic, necrotizing
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IgA vasculitis (Henoch–Schönlein purpura)
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Lupus vasculitis
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Drug-induced vasculitis
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Cryoglobulinemic
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Hypocomplementemic urticarial vasculitis
Porphyria3 (consider 1,2 to distinguish from pemphigoid)
Pseudoporphyria3 (consider 1,2 to distinguish from pemphigoid)
Eosinophil-related diseases3,4
Board Certified
John J. Zone, M.D., Kristin M. Leiferman, M.D., and Melanie K. Kuechle, M.D. are American Board of Dermatology Diplomats in Dermatology and Dermatological Immunology / Diagnostic and Laboratory Immunology. Mazdak A. Khalighi, M.D. and Margaret M. Cocks, Ph.D., M.D. are American Board of Pathology Diplomats in Anatomic Pathology. Dr. Khalighi additionally has fellowship training and faculty experience in Renal Pathology/Immunopathology. Dr. Cocks additionally is an American Board of Pathology Diplomat in Clinical Pathology and is an American Board of Dermatology Diplomat in Dermatology and Dermatopathology. Drs. Khalighi and Cocks have had specialized, fellowship-equivalent training in laboratory immunodermatology by Drs. Zone and Leiferman.
Contact Us
Immunodermatology Laboratory,
Department of Dermatology
University of Utah Health
417 South Wakara Way, Suite 2151
Salt Lake City, UT 84108