Journal article
A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review
The American journal of dermatopathology, Vol.43(12), pp.e267-e272
12/01/2021
DOI: 10.1097/DAD.0000000000002075
PMID: 34797807
Abstract
Cutaneous eruptions associated with hemophagocytic lymphohistiocytosis (HLH) have been reported in 6%-63% of patients. Clinical findings of these skin lesions vary widely and include maculopapular rashes, ulcers, and violaceous nodules. Corresponding histologic findings are also variable and are considered nonspecific. We report the case of a 4-year-old boy who initially developed a widespread popular-pustular rash 2 weeks after his 12-month measles, mumps, and rubella vaccinations. These resolved with scarring then recurred following his 24-month vaccinations. Multiple skin biopsies were negative for infectious organisms and showed a granulomatous infiltrate with perforation and necrobiosis. The differential diagnosis included perforating granuloma annulare, infection, or rheumatoid nodules. At the age of 4, he developed fever, hepatosplenomegaly, pancytopenia and other laboratory abnormalities, requiring hospitalization. A number of studies were performed including biopsies of bone marrow and liver. Molecular testing revealed 2 mutations in UNC13D known to be associated with familial HLH. His prior cutaneous lesions were likely caused by immune dysregulation exacerbated by immunizations because of underlying familial HLH. This case illustrates the importance of recognizing an unusual cutaneous manifestation of a rare disease to arrive at an earlier diagnosis in a pediatric patient. Although cutaneous eruptions usually develop concurrently with other systemic symptoms of HLH, preceding unusual skin lesions may be the first indication of this rare disease.
Details
- Title: Subtitle
- A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review
- Creators
- Hatice B Zengin - Departments of Pathology and Laboratory Medicine, and.Cynthia Reyes-Barron - University of Rochester Medical CenterElizabeth Cusick - University of Rochester Medical CenterMaria Cordisco - University of Rochester Medical CenterPhilip J Katzman - Departments of Pathology and Laboratory Medicine, and.W Richard Burack - Departments of Pathology and Laboratory Medicine, and.Glynis Scott - University of Rochester Medical Center
- Resource Type
- Journal article
- Publication Details
- The American journal of dermatopathology, Vol.43(12), pp.e267-e272
- DOI
- 10.1097/DAD.0000000000002075
- PMID
- 34797807
- ISSN
- 0193-1091
- eISSN
- 1533-0311
- Language
- English
- Date published
- 12/01/2021
- Academic Unit
- Dermatology
- Record Identifier
- 9984296310602771
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