Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report
Polymorphous skin lesions have classically been described in paraneoplastic pemphigus (PNP), but it can present as toxic epidermal necrolysis (TEN) though this type of presentation is extremely rare. We report a case of PNP presenting as TEN in a young male patient. Patient had history of fever and...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)
2018-03-01
|
| Series: | Nepal Journal of Dermatology, Venereology & Leprology |
| Subjects: | |
| Online Access: | https://nepjol.info/index.php/NJDVL/article/view/19416 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849226713310953472 |
|---|---|
| author | Jignaben K Padhiyar Nayankumar H Patel Kishan Ninama Freny E Bilimoria Rashmi Mahajan Trusha Gajjar Mansi Buch |
| author_facet | Jignaben K Padhiyar Nayankumar H Patel Kishan Ninama Freny E Bilimoria Rashmi Mahajan Trusha Gajjar Mansi Buch |
| author_sort | Jignaben K Padhiyar |
| collection | DOAJ |
| description |
Polymorphous skin lesions have classically been described in paraneoplastic pemphigus (PNP), but it can present as toxic epidermal necrolysis (TEN) though this type of presentation is extremely rare. We report a case of PNP presenting as TEN in a young male patient. Patient had history of fever and diarrhoea six weeks before starting of lesions in oral cavity, for which he was treated with injectable medicines. Then patient developed generalized necrosis and peeling of skin with involvement of conjunctiva, oropharynx and genital mucosa. For this, the patient was given intravenous dexamethasone considering it as TEN, but after transient improvement initially skin lesions recurred when dose of dexamethasone was reduced. On seventh day, patient developed few circular deep ulcers over arms and back. Nikolsky sign was positive with tzanck smear showing acantholytic cells. Hence, we added PNP as one of the differential diagnosis. On further investigations patient was found to have B cell lymphoma in mediastinum and skin biopsy and direct immunofluorescence were confirmative of PNP. Unfortunately, patient then succumbed to death due to multiorgan failure and electrolyte imbalance. The onset of PNP can be as acute as TEN and clinical picture being initially undistinguishable, high index of suspicion is required in diagnosis.
|
| format | Article |
| id | doaj-art-45504768280b43b486d57554e4e5439c |
| institution | Kabale University |
| issn | 2091-0231 2091-167X |
| language | English |
| publishDate | 2018-03-01 |
| publisher | Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON) |
| record_format | Article |
| series | Nepal Journal of Dermatology, Venereology & Leprology |
| spelling | doaj-art-45504768280b43b486d57554e4e5439c2025-08-24T10:06:22ZengSociety of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)Nepal Journal of Dermatology, Venereology & Leprology2091-02312091-167X2018-03-01161Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case ReportJignaben K Padhiyar0Nayankumar H Patel1Kishan Ninama2Freny E Bilimoria3Rashmi Mahajan4Trusha Gajjar5Mansi Buch6Gujarat Cancer Society Medical College, Hospital & Research Centre, Ahmedabad, GujaratGujarat Cancer Society Medical College, Hospital & Research Centre, Ahmedabad, GujaratSmt. B K Shah Medical Institute and Research Centre, Vadoddara, GujaratSmt. B K Shah Medical Institute and Research Centre, Vadoddara, GujaratSmt. B K Shah Medical Institute and Research Centre, Vadoddara, GujaratGujarat Cancer Society Medical College, Hospital & Research Centre, Ahmedabad, GujaratGujarat Cancer Society Medical College, Hospital & Research Centre, Ahmedabad, Gujarat Polymorphous skin lesions have classically been described in paraneoplastic pemphigus (PNP), but it can present as toxic epidermal necrolysis (TEN) though this type of presentation is extremely rare. We report a case of PNP presenting as TEN in a young male patient. Patient had history of fever and diarrhoea six weeks before starting of lesions in oral cavity, for which he was treated with injectable medicines. Then patient developed generalized necrosis and peeling of skin with involvement of conjunctiva, oropharynx and genital mucosa. For this, the patient was given intravenous dexamethasone considering it as TEN, but after transient improvement initially skin lesions recurred when dose of dexamethasone was reduced. On seventh day, patient developed few circular deep ulcers over arms and back. Nikolsky sign was positive with tzanck smear showing acantholytic cells. Hence, we added PNP as one of the differential diagnosis. On further investigations patient was found to have B cell lymphoma in mediastinum and skin biopsy and direct immunofluorescence were confirmative of PNP. Unfortunately, patient then succumbed to death due to multiorgan failure and electrolyte imbalance. The onset of PNP can be as acute as TEN and clinical picture being initially undistinguishable, high index of suspicion is required in diagnosis. https://nepjol.info/index.php/NJDVL/article/view/19416Acantholysisfluorescent antibody techniquedirectpemphigusstevens-johnson syndrome |
| spellingShingle | Jignaben K Padhiyar Nayankumar H Patel Kishan Ninama Freny E Bilimoria Rashmi Mahajan Trusha Gajjar Mansi Buch Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report Nepal Journal of Dermatology, Venereology & Leprology Acantholysis fluorescent antibody technique direct pemphigus stevens-johnson syndrome |
| title | Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report |
| title_full | Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report |
| title_fullStr | Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report |
| title_full_unstemmed | Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report |
| title_short | Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report |
| title_sort | paraneoplastic pemphigus presenting as toxic epidermal necrolysis a case report |
| topic | Acantholysis fluorescent antibody technique direct pemphigus stevens-johnson syndrome |
| url | https://nepjol.info/index.php/NJDVL/article/view/19416 |
| work_keys_str_mv | AT jignabenkpadhiyar paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport AT nayankumarhpatel paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport AT kishanninama paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport AT frenyebilimoria paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport AT rashmimahajan paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport AT trushagajjar paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport AT mansibuch paraneoplasticpemphiguspresentingastoxicepidermalnecrolysisacasereport |