An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines
Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-02-01
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| Series: | Dermatopathology |
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| Online Access: | https://www.mdpi.com/2296-3529/10/1/9 |
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| author | Jason R. McFadden Advaita S. Chaudhari Debra Birenbaum Lynette Margesson Jorge Gonzalez Aravindhan Sriharan |
| author_facet | Jason R. McFadden Advaita S. Chaudhari Debra Birenbaum Lynette Margesson Jorge Gonzalez Aravindhan Sriharan |
| author_sort | Jason R. McFadden |
| collection | DOAJ |
| description | Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and nonspecific presentation. We present the case of a 69-year-old female in which MMP of the vulva was not initially suspected. The first biopsy, from lesional tissue for routine histology, revealed fibrosis, late-stage granulation tissue, and nonspecific findings. A second biopsy, from perilesional tissue for direct immunofluorescence (DIF), revealed DIF findings typical of MMP. Scrutiny of both the first and second biopsies revealed a subtle but telling histologic feature: subepithelial clefts along adnexae in the context of a scarring process with neutrophils and eosinophils, which can be an important clue to MMP. This histologic clue has been previously described; reinforcing its importance may prove useful for future cases, especially those for which DIF is not feasible. Our case demonstrates the protean presentations of MMP, the need for persistence in sampling unusual cases, and the relevance of inconspicuous histologic features. The report highlights this underrecognized yet potentially decisive histologic clue to MMP, reviews current biopsy guidelines when MMP is suspected, and delineates the clinical and morphological features of vulvar MMP. |
| first_indexed | 2024-03-11T06:41:33Z |
| format | Article |
| id | doaj.art-25f1945741af4e438b42eb61b7345a21 |
| institution | Directory Open Access Journal |
| issn | 2296-3529 |
| language | English |
| last_indexed | 2024-03-11T06:41:33Z |
| publishDate | 2023-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Dermatopathology |
| spelling | doaj.art-25f1945741af4e438b42eb61b7345a212023-11-17T10:32:44ZengMDPI AGDermatopathology2296-35292023-02-01101636910.3390/dermatopathology10010009An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic GuidelinesJason R. McFadden0Advaita S. Chaudhari1Debra Birenbaum2Lynette Margesson3Jorge Gonzalez4Aravindhan Sriharan5Department of Biological Sciences, Dartmouth College, Hanover, NH 03755, USADepartment of Biological Sciences, Dartmouth College, Hanover, NH 03755, USADepartment of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USADepartment of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USADepartment of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USADepartment of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USAMucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and nonspecific presentation. We present the case of a 69-year-old female in which MMP of the vulva was not initially suspected. The first biopsy, from lesional tissue for routine histology, revealed fibrosis, late-stage granulation tissue, and nonspecific findings. A second biopsy, from perilesional tissue for direct immunofluorescence (DIF), revealed DIF findings typical of MMP. Scrutiny of both the first and second biopsies revealed a subtle but telling histologic feature: subepithelial clefts along adnexae in the context of a scarring process with neutrophils and eosinophils, which can be an important clue to MMP. This histologic clue has been previously described; reinforcing its importance may prove useful for future cases, especially those for which DIF is not feasible. Our case demonstrates the protean presentations of MMP, the need for persistence in sampling unusual cases, and the relevance of inconspicuous histologic features. The report highlights this underrecognized yet potentially decisive histologic clue to MMP, reviews current biopsy guidelines when MMP is suspected, and delineates the clinical and morphological features of vulvar MMP.https://www.mdpi.com/2296-3529/10/1/9mucous membrane pemphigoidsubepithelial clefts along adnexaegynecological pathologycicatricial pemphigoid |
| spellingShingle | Jason R. McFadden Advaita S. Chaudhari Debra Birenbaum Lynette Margesson Jorge Gonzalez Aravindhan Sriharan An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines Dermatopathology mucous membrane pemphigoid subepithelial clefts along adnexae gynecological pathology cicatricial pemphigoid |
| title | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
| title_full | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
| title_fullStr | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
| title_full_unstemmed | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
| title_short | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
| title_sort | underrecognized histologic clue to the diagnosis of mucous membrane pemphigoid a case report and review of diagnostic guidelines |
| topic | mucous membrane pemphigoid subepithelial clefts along adnexae gynecological pathology cicatricial pemphigoid |
| url | https://www.mdpi.com/2296-3529/10/1/9 |
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