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Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence.
J Cutan Med Surg. 2018 Jan/Feb; 22(1):22-24.JC

Abstract

BACKGROUND

It has been postulated that periodic acid-Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions.

OBJECTIVE

In this study, we aimed to confirm these observations.

METHODS

We reviewed 13 cases of direct immunofluorescence-confirmed epidermolysis bullosa acquisita and 19 cases of direct immunofluorescence-confirmed bullous pemphigoid, all with a subepidermal blister in the routinely processed specimen. The gold standard for diagnosis of epidermolysis bullosa acquisita vs bullous pemphigoid was taken to be identification of immune deposits on the dermal side ('floor' for epidermolysis bullosa acquisita) or the epidermal side ('roof' for bullous pemphigoid) of the salt-split direct immunofluorescence specimen. Our tests to distinguish epidermolysis bullosa acquisita from bullous pemphigoid on the routinely processed biopsy included periodic acid-Schiff basement membrane on the blister roof, neutrophilic infiltrate, lack of eosinophilic infiltrate, and absence of keratinocyte fraying.

RESULTS

Sensitivity and specificity for each test were as follows: periodic acid-Schiff staining of roof (sensitivity 25%, specificity 95%), neutrophilic infiltrate (sensitivity 54%, specificity 74%), lack of eosinophilic infiltrate (sensitivity 92%, specificity 68%), and absence of keratinocyte fraying (sensitivity 62%, specificity 58%).

CONCLUSIONS

Features in the routinely processed biopsy were unable to reliably distinguish between epidermolysis bullosa acquisita and bullous pemphigoid. Direct immunofluorescence on salt-split skin remains the standard for differentiation.

Authors+Show Affiliations

1 Departments of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada.1 Departments of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada. 2 Departments of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28719980

Citation

Gardner, Kerry M., and Richard I. Crawford. "Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence." Journal of Cutaneous Medicine and Surgery, vol. 22, no. 1, 2018, pp. 22-24.
Gardner KM, Crawford RI. Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence. J Cutan Med Surg. 2018;22(1):22-24.
Gardner, K. M., & Crawford, R. I. (2018). Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence. Journal of Cutaneous Medicine and Surgery, 22(1), 22-24. https://doi.org/10.1177/1203475417722734
Gardner KM, Crawford RI. Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence. J Cutan Med Surg. 2018 Jan/Feb;22(1):22-24. PubMed PMID: 28719980.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence. AU - Gardner,Kerry M, AU - Crawford,Richard I, Y1 - 2017/07/18/ PY - 2017/7/20/pubmed PY - 2018/8/11/medline PY - 2017/7/20/entrez KW - bullous pemphigoid KW - epidermolysis bullosa acquisita SP - 22 EP - 24 JF - Journal of cutaneous medicine and surgery JO - J Cutan Med Surg VL - 22 IS - 1 N2 - BACKGROUND: It has been postulated that periodic acid-Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions. OBJECTIVE: In this study, we aimed to confirm these observations. METHODS: We reviewed 13 cases of direct immunofluorescence-confirmed epidermolysis bullosa acquisita and 19 cases of direct immunofluorescence-confirmed bullous pemphigoid, all with a subepidermal blister in the routinely processed specimen. The gold standard for diagnosis of epidermolysis bullosa acquisita vs bullous pemphigoid was taken to be identification of immune deposits on the dermal side ('floor' for epidermolysis bullosa acquisita) or the epidermal side ('roof' for bullous pemphigoid) of the salt-split direct immunofluorescence specimen. Our tests to distinguish epidermolysis bullosa acquisita from bullous pemphigoid on the routinely processed biopsy included periodic acid-Schiff basement membrane on the blister roof, neutrophilic infiltrate, lack of eosinophilic infiltrate, and absence of keratinocyte fraying. RESULTS: Sensitivity and specificity for each test were as follows: periodic acid-Schiff staining of roof (sensitivity 25%, specificity 95%), neutrophilic infiltrate (sensitivity 54%, specificity 74%), lack of eosinophilic infiltrate (sensitivity 92%, specificity 68%), and absence of keratinocyte fraying (sensitivity 62%, specificity 58%). CONCLUSIONS: Features in the routinely processed biopsy were unable to reliably distinguish between epidermolysis bullosa acquisita and bullous pemphigoid. Direct immunofluorescence on salt-split skin remains the standard for differentiation. SN - 1615-7109 UR - https://www.unboundmedicine.com/medline/citation/28719980/Distinguishing_Epidermolysis_Bullosa_Acquisita_From_Bullous_Pemphigoid_Without_Direct_Immunofluorescence_ L2 - https://journals.sagepub.com/doi/10.1177/1203475417722734?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -