A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report
The febrile ulceronecrotic Mucha-Habermann disease is a rare and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). It is characterized by a sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Herein, we report a 23-year-old...
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| Format: | Article |
| Language: | English |
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Karger Publishers
2023-01-01
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| Series: | Case Reports in Dermatology |
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| Online Access: | https://www.karger.com/Article/FullText/528500 |
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| author | Waseem Alhawsawi Khalid Al Hawsawi Bashaer Almahdi Shahad Alkidaiwi Khlood Alzubaidy Reema Alhuthayli Abdulmohsin Algethami Alshareef Alshareef |
| author_facet | Waseem Alhawsawi Khalid Al Hawsawi Bashaer Almahdi Shahad Alkidaiwi Khlood Alzubaidy Reema Alhuthayli Abdulmohsin Algethami Alshareef Alshareef |
| author_sort | Waseem Alhawsawi |
| collection | DOAJ |
| description | The febrile ulceronecrotic Mucha-Habermann disease is a rare and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). It is characterized by a sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Herein, we report a 23-year-old male, not known to have any medical illnesses, presented with a month-long history of persistent fever of unknown origin associated with a sudden onset of progressive diffuse necrotic ulcers and widespread papulosquamous lesions. Pan CT showed enlarged lymph nodes in the cervix, chest, and abdomen. Unfortunately, a skin biopsy was done late, showing features consistent with PLEVA. Few days after admission, despite being on intravenous methylprednisolone, our patient rapidly deteriorated by showing severe acute respiratory symptoms and consequently died. In spite of the continuous addition of new case reports to the literature, no definite diagnostic criteria have been established, leading to late or missed cases, and an optimum treatment is still waiting. |
| first_indexed | 2024-04-10T09:54:36Z |
| format | Article |
| id | doaj.art-b726519e809f4aa088f724c1b7c4d40a |
| institution | Directory Open Access Journal |
| issn | 1662-6567 |
| language | English |
| last_indexed | 2024-04-10T09:54:36Z |
| publishDate | 2023-01-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Dermatology |
| spelling | doaj.art-b726519e809f4aa088f724c1b7c4d40a2023-02-16T12:58:35ZengKarger PublishersCase Reports in Dermatology1662-65672023-01-01151172110.1159/000528500528500A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case ReportWaseem Alhawsawi0Khalid Al Hawsawi1Bashaer Almahdi2Shahad Alkidaiwi3Khlood Alzubaidy4Reema Alhuthayli5Abdulmohsin Algethami6Alshareef Alshareef7King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi ArabiaDermatology Consultant, King Abdulaziz Hospital, Makkah, Saudi ArabiaKing Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi ArabiaMedical Intern, Umm Al-Qura University, Makkah, Saudi ArabiaMedical Intern, Ibn Sina University, Jeddah, Saudi ArabiaMedical Intern, King Abdulaziz University, Jeddah, Saudi ArabiaKing Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi ArabiaKing Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi ArabiaThe febrile ulceronecrotic Mucha-Habermann disease is a rare and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). It is characterized by a sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Herein, we report a 23-year-old male, not known to have any medical illnesses, presented with a month-long history of persistent fever of unknown origin associated with a sudden onset of progressive diffuse necrotic ulcers and widespread papulosquamous lesions. Pan CT showed enlarged lymph nodes in the cervix, chest, and abdomen. Unfortunately, a skin biopsy was done late, showing features consistent with PLEVA. Few days after admission, despite being on intravenous methylprednisolone, our patient rapidly deteriorated by showing severe acute respiratory symptoms and consequently died. In spite of the continuous addition of new case reports to the literature, no definite diagnostic criteria have been established, leading to late or missed cases, and an optimum treatment is still waiting.https://www.karger.com/Article/FullText/528500febrile ulceronecrotic mucha-habermann diseasepityriasis lichenoides et varioliformis acuta |
| spellingShingle | Waseem Alhawsawi Khalid Al Hawsawi Bashaer Almahdi Shahad Alkidaiwi Khlood Alzubaidy Reema Alhuthayli Abdulmohsin Algethami Alshareef Alshareef A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report Case Reports in Dermatology febrile ulceronecrotic mucha-habermann disease pityriasis lichenoides et varioliformis acuta |
| title | A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report |
| title_full | A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report |
| title_fullStr | A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report |
| title_full_unstemmed | A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report |
| title_short | A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report |
| title_sort | diagnostically challenging case of de novo febrile ulceronecrotic mucha habermann disease with fatal pulmonary involvement a case report |
| topic | febrile ulceronecrotic mucha-habermann disease pityriasis lichenoides et varioliformis acuta |
| url | https://www.karger.com/Article/FullText/528500 |
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