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Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid.
JAMA Dermatol. 2016 08 01; 152(8):897-904.JD

Abstract

IMPORTANCE

Anti-p200 pemphigoid is a rare subepidermal autoimmune blistering disease characterized by autoantibodies against a 200-kDa protein in the basement membrane zone. Anti-p200 pemphigoid is probably often misdiagnosed because of low availability of diagnostic assays and expertise and classified as bullous pemphigoid or epidermolysis bullosa acquisita.

OBJECTIVE

To clinically characterize patients with anti-p200 pemphigoid, identified by using indirect immunofluorescence microscopy on skin substrates deficient in type VII collagen and laminin-332 (knockout analysis), to validate this technique by immunoblot with dermal extract, and to incorporate direct immunofluorescence serration pattern analysis in the diagnostic algorithm.

DESIGN, SETTING, AND PARTICIPANTS

This was a retrospective study performed from January 2014 to June 2015 with biobank patient materials and clinical data for the period 1998 to 2015 from the single national referral center on autoimmune bullous diseases. Patients were selected based on a dermal side binding on 1-mol/L salt (sodium chloride)-split human skin substrate by indirect immunofluorescence microscopy, not diagnosed epidermolysis bullosa acquisita or anti-laminin-332 mucous membrane pemphigoid.

MAIN OUTCOMES AND MEASURES

Indirect immunofluorescence microscopy knockout analysis was performed and diagnosis of anti-p200 confirmed by immunoblot with dermal extract. Clinical, histological, and immunological findings were registered. Autoantibodies against laminin γ1 were determined by immunoblot.

RESULTS

Twelve patients with anti-p200 pemphigoid (7 male and 5 female; mean age, 66.6 years) were identified using the indirect immunofluorescence microscopy knockout analysis. Direct immunofluorescence microscopy showed a linear n-serrated IgG deposition pattern along the basement membrane zone in 9 of 11 patients. The diagnosis was confirmed by immunoblot showing autoantibodies against 200-kDa protein in dermal extract in 12 of 12 patients. Autoantibodies against recombinant laminin γ1 were detected by immunoblot in 8 of 12 patients. Remarkable similarities were seen in clinical features with predominantly tense blisters on hands and feet, resembling dyshidrosiform pemphigoid. Mucosal involvement was seen in 6 (50%) of the patients.

CONCLUSIONS AND RELEVANCE

Predominance of blisters on hands and feet may be a clinical clue to the diagnosis of anti-p200 pemphigoid. Direct immunofluorescence microscopy serration pattern analysis and indirect immunofluorescence microscopy knockout analysis are valuable additional techniques to facilitate the diagnosis of anti-p200 pemphigoid.

Authors+Show Affiliations

Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.Department of Dermatology, University of Lübeck, Lübeck, Germany.Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

Pub Type(s)

Journal Article
Validation Study

Language

eng

PubMed ID

27167149

Citation

Meijer, Joost M., et al. "Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid." JAMA Dermatology, vol. 152, no. 8, 2016, pp. 897-904.
Meijer JM, Diercks GF, Schmidt E, et al. Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid. JAMA Dermatol. 2016;152(8):897-904.
Meijer, J. M., Diercks, G. F., Schmidt, E., Pas, H. H., & Jonkman, M. F. (2016). Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid. JAMA Dermatology, 152(8), 897-904. https://doi.org/10.1001/jamadermatol.2016.1099
Meijer JM, et al. Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid. JAMA Dermatol. 2016 08 1;152(8):897-904. PubMed PMID: 27167149.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid. AU - Meijer,Joost M, AU - Diercks,Gilles F H, AU - Schmidt,Enno, AU - Pas,Hendri H, AU - Jonkman,Marcel F, PY - 2016/5/12/entrez PY - 2016/5/12/pubmed PY - 2017/6/28/medline SP - 897 EP - 904 JF - JAMA dermatology JO - JAMA Dermatol VL - 152 IS - 8 N2 - IMPORTANCE: Anti-p200 pemphigoid is a rare subepidermal autoimmune blistering disease characterized by autoantibodies against a 200-kDa protein in the basement membrane zone. Anti-p200 pemphigoid is probably often misdiagnosed because of low availability of diagnostic assays and expertise and classified as bullous pemphigoid or epidermolysis bullosa acquisita. OBJECTIVE: To clinically characterize patients with anti-p200 pemphigoid, identified by using indirect immunofluorescence microscopy on skin substrates deficient in type VII collagen and laminin-332 (knockout analysis), to validate this technique by immunoblot with dermal extract, and to incorporate direct immunofluorescence serration pattern analysis in the diagnostic algorithm. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective study performed from January 2014 to June 2015 with biobank patient materials and clinical data for the period 1998 to 2015 from the single national referral center on autoimmune bullous diseases. Patients were selected based on a dermal side binding on 1-mol/L salt (sodium chloride)-split human skin substrate by indirect immunofluorescence microscopy, not diagnosed epidermolysis bullosa acquisita or anti-laminin-332 mucous membrane pemphigoid. MAIN OUTCOMES AND MEASURES: Indirect immunofluorescence microscopy knockout analysis was performed and diagnosis of anti-p200 confirmed by immunoblot with dermal extract. Clinical, histological, and immunological findings were registered. Autoantibodies against laminin γ1 were determined by immunoblot. RESULTS: Twelve patients with anti-p200 pemphigoid (7 male and 5 female; mean age, 66.6 years) were identified using the indirect immunofluorescence microscopy knockout analysis. Direct immunofluorescence microscopy showed a linear n-serrated IgG deposition pattern along the basement membrane zone in 9 of 11 patients. The diagnosis was confirmed by immunoblot showing autoantibodies against 200-kDa protein in dermal extract in 12 of 12 patients. Autoantibodies against recombinant laminin γ1 were detected by immunoblot in 8 of 12 patients. Remarkable similarities were seen in clinical features with predominantly tense blisters on hands and feet, resembling dyshidrosiform pemphigoid. Mucosal involvement was seen in 6 (50%) of the patients. CONCLUSIONS AND RELEVANCE: Predominance of blisters on hands and feet may be a clinical clue to the diagnosis of anti-p200 pemphigoid. Direct immunofluorescence microscopy serration pattern analysis and indirect immunofluorescence microscopy knockout analysis are valuable additional techniques to facilitate the diagnosis of anti-p200 pemphigoid. SN - 2168-6084 UR - https://www.unboundmedicine.com/medline/citation/27167149/Laboratory_Diagnosis_and_Clinical_Profile_of_Anti_p200_Pemphigoid_ L2 - https://jamanetwork.com/journals/jamadermatology/fullarticle/10.1001/jamadermatol.2016.1099 DB - PRIME DP - Unbound Medicine ER -