Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood
Kikuchi–Fujimoto disease (KFD) is a rare condition characterized by necrotizing lymphadenitis and fever, often associated with immune dysregulation. Histologically, it features necrotic foci with abundant histiocytes and plasmacytoid dendritic cells but notably lacks neutrophils and eosinophils. Rec...
Saved in:
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2025-02-01
|
| Series: | Dermatopathology |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2296-3529/12/1/7 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849342550821830656 |
|---|---|
| author | Alberto Soto-Moreno Francisco Vílchez-Márquez María Narváez-Simón Julia Castro-Martín Francisco Manuel Ramos-Pleguezuelos Agustín Soto-Díaz Jesús Tercedor-Sánchez Salvador Arias-Santiago |
| author_facet | Alberto Soto-Moreno Francisco Vílchez-Márquez María Narváez-Simón Julia Castro-Martín Francisco Manuel Ramos-Pleguezuelos Agustín Soto-Díaz Jesús Tercedor-Sánchez Salvador Arias-Santiago |
| author_sort | Alberto Soto-Moreno |
| collection | DOAJ |
| description | Kikuchi–Fujimoto disease (KFD) is a rare condition characterized by necrotizing lymphadenitis and fever, often associated with immune dysregulation. Histologically, it features necrotic foci with abundant histiocytes and plasmacytoid dendritic cells but notably lacks neutrophils and eosinophils. Recent evidence reveals a notable prevalence among pediatric patients, who may exhibit distinct features compared to adults. We reported the case of an 11-year-old girl presenting with persistent fever, cervical adenopathy, and a malar rash, leading to a diagnosis of KFD following lymph node biopsy, which revealed non-suppurative necrosis and histiocytic infiltration. Empirical treatment with antivirals and antibiotics was ineffective, but corticosteroid therapy achieved symptom remission. A literature review identified 48 relevant studies involving 386 pediatric cases, with histopathological findings consistent with classical descriptions of KFD. Cutaneous involvement was reported in 11.14% of cases, ranging from maculopapular rashes to lupus-like eruptions. Notable complications included the development of systemic lupus erythematous, Sjögren syndrome, and rare instances of hemophagocytic syndrome or central nervous system involvement. Kikuchi–Fujimoto disease should be considered in the differential diagnosis of pediatric patients presenting with fever and lymphadenopathy, taking into account a higher frequency of cutaneous manifestations in pediatric cases. A skin biopsy may be helpful in diagnosing KFD and provide valuable information regarding the potential risk of developing systemic lupus erythematosus in the future. |
| format | Article |
| id | doaj-art-c46d031f16d6440a9a20f70caed13129 |
| institution | Kabale University |
| issn | 2296-3529 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Dermatopathology |
| spelling | doaj-art-c46d031f16d6440a9a20f70caed131292025-08-20T03:43:21ZengMDPI AGDermatopathology2296-35292025-02-01121710.3390/dermatopathology12010007Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in ChildhoodAlberto Soto-Moreno0Francisco Vílchez-Márquez1María Narváez-Simón2Julia Castro-Martín3Francisco Manuel Ramos-Pleguezuelos4Agustín Soto-Díaz5Jesús Tercedor-Sánchez6Salvador Arias-Santiago7Dermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainDermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainPathology Derpartment, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainDermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainPathology Derpartment, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainDermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainDermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainDermatology Department, Hospital Universitario Virgen de Las Nieves, 18012 Granada, SpainKikuchi–Fujimoto disease (KFD) is a rare condition characterized by necrotizing lymphadenitis and fever, often associated with immune dysregulation. Histologically, it features necrotic foci with abundant histiocytes and plasmacytoid dendritic cells but notably lacks neutrophils and eosinophils. Recent evidence reveals a notable prevalence among pediatric patients, who may exhibit distinct features compared to adults. We reported the case of an 11-year-old girl presenting with persistent fever, cervical adenopathy, and a malar rash, leading to a diagnosis of KFD following lymph node biopsy, which revealed non-suppurative necrosis and histiocytic infiltration. Empirical treatment with antivirals and antibiotics was ineffective, but corticosteroid therapy achieved symptom remission. A literature review identified 48 relevant studies involving 386 pediatric cases, with histopathological findings consistent with classical descriptions of KFD. Cutaneous involvement was reported in 11.14% of cases, ranging from maculopapular rashes to lupus-like eruptions. Notable complications included the development of systemic lupus erythematous, Sjögren syndrome, and rare instances of hemophagocytic syndrome or central nervous system involvement. Kikuchi–Fujimoto disease should be considered in the differential diagnosis of pediatric patients presenting with fever and lymphadenopathy, taking into account a higher frequency of cutaneous manifestations in pediatric cases. A skin biopsy may be helpful in diagnosing KFD and provide valuable information regarding the potential risk of developing systemic lupus erythematosus in the future.https://www.mdpi.com/2296-3529/12/1/7Kikuchi–Fujimoto diseasechildrenhistiocytic necrotizing lymphadenitislupus erythematosusskin |
| spellingShingle | Alberto Soto-Moreno Francisco Vílchez-Márquez María Narváez-Simón Julia Castro-Martín Francisco Manuel Ramos-Pleguezuelos Agustín Soto-Díaz Jesús Tercedor-Sánchez Salvador Arias-Santiago Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood Dermatopathology Kikuchi–Fujimoto disease children histiocytic necrotizing lymphadenitis lupus erythematosus skin |
| title | Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood |
| title_full | Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood |
| title_fullStr | Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood |
| title_full_unstemmed | Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood |
| title_short | Pediatric Kikuchi–Fujimoto Disease: Case Report and Review of Cutaneous and Histopathologic Features in Childhood |
| title_sort | pediatric kikuchi fujimoto disease case report and review of cutaneous and histopathologic features in childhood |
| topic | Kikuchi–Fujimoto disease children histiocytic necrotizing lymphadenitis lupus erythematosus skin |
| url | https://www.mdpi.com/2296-3529/12/1/7 |
| work_keys_str_mv | AT albertosotomoreno pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT franciscovilchezmarquez pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT marianarvaezsimon pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT juliacastromartin pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT franciscomanuelramospleguezuelos pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT agustinsotodiaz pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT jesustercedorsanchez pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood AT salvadorariassantiago pediatrickikuchifujimotodiseasecasereportandreviewofcutaneousandhistopathologicfeaturesinchildhood |