Farrah Bakr1, Naomi Webber2, Hiva Fassihi2, Victoria Swale3, Fiona Lewis2, Ed Rytina4, Galia Tamar Ben-Zvi5, Paul Norris5, Olivia Espinosa6, Sunanda Dhar6, Paul Craig7, Alistair Robson2. 1. St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom. Electronic address: farrahbakr@doctors.org.uk. 2. St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom. 3. Department of Dermatology, Royal Free Hospital, London, United Kingdom. 4. Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom. 5. Department of Dermatology, Addenbrooke's Hospital, Cambridge, United Kingdom. 6. Nuffield Department of Pathology, John Radcliffe Hospital, Oxford, United Kingdom. 7. Gloucestershire Hospitals NHS Foundation Trust, Gloucestershire Cellular Pathology Laboratory, Cheltenham General Hospital, Cheltenham, United Kingdom.
Abstract
BACKGROUND: Intralymphatic histiocytosis (IH) is a rare condition often associated with systemic disease. A benign condition, clinical presentations can vary greatly and its cause is largely unknown. Histologically, there are macrophages within distended lymphatic vessels, although this can be an incidental finding or the primary abnormality. OBJECTIVE: We present a series of 7 cases of IH with and without disease associations, and a review of the literature. We propose IH as either primary (without associated conditions) or secondary (associated with systemic disease). METHODS: This was a retrospective collection of patients whose skin biopsy specimens revealed intralymphatic collections of histiocytes. We reviewed their clinical presentation, disease associations, and staining of slides with CD68 in all cases, D2-40 in 5 cases, and HLA-DR in 4 cases. RESULTS: Clinical features were highly variable, and not all cases were associated with systemic disease. One case had admixed reactive angioendotheliomatosis. All 4 cases stained for HLA-DR showed strong expression by the intralymphatic macrophages. LIMITATIONS: Retrospective analysis and limited numbers are limitations. CONCLUSION: IH is not always associated with systemic disease although macrophage activation nevertheless implies immune activation.
BACKGROUND: Intralymphatic histiocytosis (IH) is a rare condition often associated with systemic disease. A benign condition, clinical presentations can vary greatly and its cause is largely unknown. Histologically, there are macrophages within distended lymphatic vessels, although this can be an incidental finding or the primary abnormality. OBJECTIVE: We present a series of 7 cases of IH with and without disease associations, and a review of the literature. We propose IH as either primary (without associated conditions) or secondary (associated with systemic disease). METHODS: This was a retrospective collection of patients whose skin biopsy specimens revealed intralymphatic collections of histiocytes. We reviewed their clinical presentation, disease associations, and staining of slides with CD68 in all cases, D2-40 in 5 cases, and HLA-DR in 4 cases. RESULTS: Clinical features were highly variable, and not all cases were associated with systemic disease. One case had admixed reactive angioendotheliomatosis. All 4 cases stained for HLA-DR showed strong expression by the intralymphatic macrophages. LIMITATIONS: Retrospective analysis and limited numbers are limitations. CONCLUSION: IH is not always associated with systemic disease although macrophage activation nevertheless implies immune activation.