Skin nonmelanocytic tumor
Vascular tumors
Hemangioma variants
Angiolymphoid hyperplasia with eosinophilia
Author: Jayalakshmi Balakrishna, M.D.
Last author update: 1 August 2017
Last staff update: 9 February 2023 (update in progress)
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Angiolymphoid hyperplasia with eosinophilia skin
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Laboratory | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Positive stains | Negative stains | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1Cite this page: Balakrishna J. Angiolymphoid hyperplasia with eosinophilia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodesangiolymphoidhyperplasiawitheosinophili.html. Accessed April 2nd, 2023.
Definition / general
- Low grade vascular tumor characterized by proliferation of blood vessels lined by plump endothelial cells
Essential features
- Vascular proliferation
- Histiocytoid endothelial cells with hobnail appearance
- Mixed inflammatory infiltrate consisting of lymphocytes, plasma cells and eosinophils
Terminology
- Angiolymphoid hyperplasia with eosinophilia
- Epithelioid hemangioma
- Obsolete terms
- Histiocytoid hemangioma
- Angiomatous nodule
- Pseudopyogenic granuloma
- Inflammatory angiomatous nodule
ICD coding
- L98.8 (ILDS L98.812)
Epidemiology
- Young to middle aged adults
- Some reports show female preponderance and some show no clear gender predilection
- Most patients are of Asian origin or Caucasian
Sites
- Predominantly head and neck
- Other sites reported: trunk, extremities, hands, penis, oral mucosa, colon
Pathophysiology
- Accompanying inflammatory infiltrate is thought to play a role in the pathogenesis of vascular proliferation
- Inflammatory cells generate a proliferative stimulus to which the endothelial cells respond and cause vascular proliferation
- Other possible contributing factors are
- Arteriovenous shunting
- Local trauma
- Elevated serum estrogen levels
Etiology
- Not known currently
- Theories include: a reactive process, infectious etiology, immunologic process or neoplastic process
Clinical features
- Lesions occur as
- Single or multiple
- Nondescript
- Flesh to plum colored papules or nodules
- Ranging in size from a few to several centimeters
- No associated symptoms are present in most cases
- Some patients may experience tenderness, pulsation, pruritus, bleeding, either spontaneously or after minor trauma
- Peripheral blood eosinophilia and regional lymphadenopathy are also reported
Diagnosis
- Biopsy and histopathologic examination
- Ancillary studies to rule out other causes of vascular proliferation and eosinophilia
Laboratory
- Eosinophilia reported in majority of the cases
Prognostic factors
- Benign lesion
- Does not regress without intervention
- Incomplete surgical excision may lead to recurrence
Case reports
- 29 year old man with gradually increasing painless swelling on the right parotid region (Arch Int Surg 2015;5:217)
- 32 year old man presenting with multiple nodules on the cheeks, preauricular region and the scalp (Head Face Med 2006;2:32)
- 52 year old woman with pruriginous papules on her scalp (Case Rep Dermatol 2015;7:113)
- 63 year old man with severe gastrointestinal hemorrhage (J Clin Pathol 1997;50:611)
- 64 year old Caucasian woman with a 2 year history of recurrent painful nodules on her occipital scalp (Proc (Bayl Univ Med Cent) 2014;27:336)
Treatment
- Total surgical excision is the current treatment of choice
Clinical images
Images hosted on other servers:
Erythematous nodule on right cheek
Erythematous nodules in occipital region
Coalescing nodules
Gross description
- Single or multiple, dome shaped, light pink to red brown papules or subcutaneous masses with no specific distinguishing surface features
- There might be erosion or crust formation of the surface
Microscopic (histologic) description
- Predominantly in the dermis and subcutaneous tissue
- Proliferation of vascular channels with accompanying mixed inflammatory infiltrate seen as nests and cords of endothelial cell proliferations with admixed lymphocytes, plasma cells and eosinophils, accompanied by hemorrhage and proliferation of thick and thin walled blood vessels
- Endothelial cells show large vesicular nuclei with acidophilic and sometimes vacuolated cytoplasm, imparting a hobnail appearance
- Mitoses can be seen but lack atypical features and anaplasia
Positive stains
- Vascular / endothelial markers
Negative stains
Differential diagnosis
Additional references
Board review style question #1
Which of the following histopathological findings helps most to differentiate angiolymphoid hyperplasia with eosinophilia from Kimura disease?
- Eosinophilia
- Lymphoid follicles
- Prominent histiocytoid endothelial cells
- Vascular proliferation
Board review style answer #1
C. Prominent histiocytoid endothelial cells; the presence of these cells with a hobnail appearance is the characteristic histologic finding of angiolymphoid hyperplasia with eosinophilia.
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Reference: Angiolymphoid hyperplasia with eosinophilia
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