Immunofluorescence testing in the diagnosis of autoimmune blistering
               diseases: overview of 10-year experience

Arbache, Samia Trigo; Nogueira, Tarsila Gasparotto; Delgado, Lívia; Miyamoto, Denise; Aoki, Valéria

doi:10.1590/abd1806-4841.20143221

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Sumário

Investigation • An. bras. dermatol. 89 (6) • Nov-Dec 2014 • https://doi.org/10.1590/abd1806-4841.20143221 copy

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience^* * Work performed at the Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP) - São Paulo (SP), Brasil.

Authorship SCIMAGO INSTITUTIONS RANKINGS

BACKGROUND:

Immunofluorescence testing is an important tool for diagnosing blistering diseases.

OBJECTIVE:

To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases.

METHODS:

We retrospectively analyzed immunofluorescence results encompassing a 10-year period.

RESULTS:

421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate.

CONCLUSION:

Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect immunofluorescence when Salt-split skin technique is performed.

Autoimmune diseases; Epidermolysis bullosa acquisita; Linear IgA bullous dermatosis; Pemphigoid, benign mucous membrane; Pemphigoid, bullous; Pemphigoid gestationis; Skin Diseases; Skin diseases, vesiculobullous

Bullous disease (group 1)	n (patients)	n (positive DIF)	DIF: intercellular deposits (conjugate=n)	DIF: BMZ deposits (conjugate=n)	DIF: cytoid bodies (conjugate=n)	Positive IIF/Performed IIF
PF	117	115	IgG=110	IgM=14	IgM=6	104/115
			C3=85
PV	142	135	IgG=130;	IgG-4;C3-8	IgM-4	109/115
			C3=113
			IgM=11
PNP	3	2	IgG=2	C3=1	-	2/3
			C3=1
IgA	8	8	IgG=1	IgM=1	-	1/8
			IgA=8
Hailey-Hailey disease	7	1	IgG=1	IgG=1	-	0/5

Bullous disease (group 2)	n (patients)	n (positive DIF)	DIF: intercellular deposits (conjugate=n)	DIF: cytoid bodies (conjugate=n)	Positive IIF/Performed IIF	Salt-Split Skin Technique (conjugate=n/total)
BP	90	88	IgG=35 C3=82	IgM=5	33/72	Epidermal:
			IgA=10			IgG=25/33;
			IgM=6			IgG/IgA=2/33
						Both sides:
						IgG=1/33, IgA-1/33
						Dermal side:
						IgG/C3=1/33
EBA	19	19	IgG=15	IgM=1	9/18	Dermal side:
			C3=17			IgG=5/13,
			IgM=4			IgA/IgG=3/13
			IgA=9
MMP	5	4	IgG=4	NA	0/2	NA
			C3=4
			IgM=4
			IgA=4
IgA linear dermatosis	15	15	IgG=2	IgM=1	2/13	Epidermal: IgA=4/5
			C3=2
			IgM=1
			IgA=15
DH	13	12	IgG=1	NA	0/1	NA
			C3=2
			IgA=12
LPP	2	2	IgG=1	IgM=2	1/2	IgG-epidermal=2/2;
			C3=2			focal epidermal
						IgA=1/2

Sociedade Brasileira de Dermatologia Av. Rio Branco, 39 18. and., 20090-003 Rio de Janeiro RJ, Tel./Fax: +55 21 2253-6747 - Rio de Janeiro - RJ - Brazil
E-mail: revista@sbd.org.br

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[1] MAILING ADDRESS: Lívia Delgado, Rua Dr. Ovídio Pires de Campos, 225, Cerqueira César, 05403-010 - São Paulo - SP. Brazil. E-mail: dermahc.labimuno@gmail.com

DERMATOSIS	DIF FINDINGS	IIF FINDINGS
Pemphigus foliaceus/vulgaris	Intercellular IgG and C3.	Intercellular IgG
Paraneoplastic pemphigus	Weak intercellular IgG and linear C3; granulous BMZ deposits.	Intercellular IgG +/- linear BMZ. Epithelial surface staining on rat bladder
IgA pemphigus	Intercellular IgA	Intercellular IgA
Hailey-Hailey disease	Negative	Negative
Bullous pemphigoid	Linear IgG and C3 at BMZ.	Linear IgG at BMZ. SS: epidermal
Epidermolysis Bullosa Acquisita	Linear IgG and C3 at BMZ +/-IgA , IgM	Linear IgG. SS: dermal
Mucous membrane pemphigoid	Linear IgG, C3 and IgA at BMZ.	Linear IgG at BMZ
IgA linear dermatosis	Linear IgA at BMZ.	Linear IgA at BMZ. SS: epidermal, dermal (both)
Dermatitis herpetiformis	Granular IgA deposits in the dermal papillae +/- C3	IgA class endomysial antibody
Lichen planus pemphigoid	Linear IgG and C3 at BMZ. Cytoid bodies.	Linear IgG at BMZ. SS: epidermal

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Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience* * Work performed at the Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP) - São Paulo (SP), Brasil.

BACKGROUND:

OBJECTIVE:

METHODS:

RESULTS:

CONCLUSION:

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience^* * Work performed at the Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP) - São Paulo (SP), Brasil.