Angiolymphoid hyperplasia with eosinophilia: a case report
Abstract
Background
Angiolymphoid hyperplasia with eosinophilia is a benign neoplasm that includes blood vessel proliferation and a dense eosinophilic inflammatory infiltrate. Mostly, it affects middle-aged adults manifesting as flesh/plum-colored pruritic nodules and papules, most commonly affecting the ear and the periauricular area.
Case presentation
In this case, we report a 13-year-old Caucasian girl with bilateral, huge, protruding, and yellowish nostril masses which were peculiar in location and of gross appearance. At first, the disease proved to be a diagnostic dilemma. After making a diagnosis of angiolymphoid hyperplasia with eosinophilia, the disease also proved to be a therapeutic dilemma. It did not respond to oral prednisolone or to oral indomethacin, and it proved to be resistant to topical steroids. Although surgery is the standard therapeutic approach, it recurred despite multiple surgical attempts. However, the only regimen that seemed to partially control the lesion was intralesional steroids combined with topical tacrolimus ointment.
Conclusions
Angiolymphoid hyperplasia with eosinophilia proves a therapeutic dilemma, because there is a large variety of proposed treatments, yet there is not enough data on most of them. Although the disease is not deadly by itself, it usually presents with disfiguring lesions that grimly affect the patient’s quality of life. This warrants further research and efforts to find an effective cure and a unified therapeutic approach.
Keywords
Angiolymphoid hyperplasia with eosinophilia Therapeutic approach Pediatric disorders Peculiar presentationAbbreviations
- ALHE
Angiolymphoid hyperplasia with eosinophilia
- ALT
Alanine transferase
- ANA
Antinuclear antibodies
- ANCA c
Antineutrophil cytoplasmic antibodies cytoplasmic
- ANCA p
antineutrophil cytoplasmic antibodies perinuclear
- AST
Aspartate transferase
- CRP
C-reactive protein
- EH
Epithelioid hemangioma
- ESR
Erythrocytes sedimentation rate
- FNA
Fine-needle aspiration
- HHV8
Human herpes virus 8
- IgE
Immunoglobulin E
- IgG
Immunoglobulin G
- IgM
Immunoglobulin M
- PCR
Polymerase chain reaction
- TCR
T-cell receptor gene
Background
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign neoplasm of debated etiology. Most commonly, it affects the face and the periauricular area especially, where it usually manifests as papules or nodules. We describe here a distinctive case with regard to the location and gross appearance of the tumor.
Case presentation
Presurgical photograph. The mass appears as a congested and yellowish tumor, protruding outside the nostrils. a caudal view of the lesion. b. superior view of the lesion
Laboratory values throughout the disease course
Lab results at the other institution | Lab results at time of diagnosis | 4 months after surgery | Units | Reference range | |
---|---|---|---|---|---|
Leukocytes | 11,700 | 12,000 | 8000 | C/uL | 3500–10,000 |
Neutrophils | 72 | 68 | 60 | % | 34–71 |
Eosinophils | 15.2 | 16 | 5 | % | 0.7–6 |
Lymphocytes | 8 | 10 | 25 | % | 19–51 |
Erythrocytes | 4.80 | 4.70 | 5 | ×103/mm3 | M 4.5–6.2 F 4–5.4 |
Hemoglobin | 14 | 14 | 15 | g/dL | M 13–18 F 12–16 |
CRP | 3.2 | 4 | 1.5 | mg/L | 0.5–5 |
ESR | 35 | 45 | 14 | mm/hL | Up to 15 |
Tuberculin test | Negative | Negative | |||
ANCA c | 2.5 Negative | U | Positive more than 18 Negative less than 18 | ||
ANCA p | 2.2 Negative | U | Positive more than 18 Less than 18 | ||
HHV8: | |||||
IgG | 8.1 Positive | U | Positive more than 1.1 | ||
IgM | 0.2 Negative | U | Negative less than 0.9 | ||
IgE | 1997 | 1200 | 250 | IU/mL | U to 120 |
Creatinine | 0.6 | 0.7 | 0.6 | mg/dL | 0.5–1.2 |
Urea | 25 | 23 | 20 | mg/dL | 5–45 |
ALT | 6 | U/L | 5–40 | ||
AST | 18 | U/L | 0–40 | ||
Urine protein | Negative | Negative | Negative | ||
Urine casts | 0 | 0 | Number/field | 0 | |
Blood film | Within normal |
Histopathologic study and description. a Skin biopsy, nose - pseudoepitheliomatous hyperplasia with ulceration and necrosis. Diffuse lymphohistiocytic infiltrate with numerous eosinophils in a fibrous stroma (hematoxylin and eosin, × 40). b Skin biopsy, nose - vascular proliferation with prominent (plump) endothelial cells and occasional cytoplasmic vacuoles (hematoxylin and eosin, × 60). c Skin biopsy, nose - vascular proliferation with intense eosinophilic infiltrate (hematoxylin and eosin, × 100)
The results of postsurgical treatment with intralesional steroids and tacrolimus cream
Treatment of angiolymphoid hyperplasia with eosinophilia
Our patient’s parents reported her full adherence to treatment. The patient herself reported decreased quality of life and impaired social interactions due to the disfiguring lesions. She also reported marked fear and distress because of the ineffectiveness of multiple therapeutic regimens and surgeries. The patient’s family reported severe financial burden due to the high costs of the treatments.
Discussion
The term angiolymphoid hyperplasia with eosinophilia (ALHE) was first created (in 1969) by Wells and Whimster [1], who used it to describe a distinguished neoplasm. The tumor is characterized by a florid proliferation of blood vessels lined by plump endothelial cells and admixed with a dense inflammatory infiltrate of lymphocytes, eosinophils, and mast cells. They reported nine cases in which the neoplasm remained benign although they reported recurrence after excision in some cases. Later, Weiss and Enzinger argued the nature of that entity, for they wanted to evidently differentiate the lesion from the malignant vascular tumor, epithelioid hemangioendothelioma. For this, they introduced the term epithelioid hemangioma (EH) in 1982 [1]. Since then, many hypotheses have been proposed to explain the etiology of the tumor, including a reactive process, a neoplastic process, and infectious mechanisms with possible association with HIV [1]. However, the argument that ALHE/EH may represent a monoclonal T-cell process is supported in some cases [2]. Interestingly, peripheral T-cell lymphoma has been reported to develop in a patient with ALHE/EH. Additionally, a certain association between ALHE/EH and follicular mucinosis has been reported. There are also cases of ALHE/EH in which T-cell receptor gene (TCR) rearrangement and monoclonality have been detected [1].
In attempt to define the histogenesis of this disorder, a team analyzed both the phenotype and the genotype of the inflammatory infiltrate using immunohistochemistry and TCR gene rearrangement by polymerase chain reaction (PCR) and other methods. The results showed five out of seven patients with ALHE displaying a clonal T-cell population and proliferative T-cell activity in ALHE tissue; most of these cases were recurrent following a prolonged and therapy-resistant course. Unfortunately, these tests were not available for our case because of war restrictions [2].
In 2015, a statistical analysis yielded no sex predominance among 908 patients [3]. Over half of the patients presented with a single lesion, and most common locations were the ear, and periauricular area; face; and scalp. Furthermore, the analysis revealed a consequential association between the existence of multiple lesions and pruritus along with bleeding. Considering age, statistics has shown a wide prevalence range (0.7 months to 91 years) and the mean age of presentation was 37.6 years. According to the literature [3], cases of ALHE with earlier age of onset, longer duration, and multiple lesions were linked to higher recurrence rates after excision, which exactly fits the course of our case. Although the ear is the most common location, in our case the lesion was located in the nostrils. To the best of our knowledge, only one similar case of ALHE in the nostrils has been reported in the medical literature [4]. While ALHE usually present as flesh- to plum-colored papules or nodules, our patient had bilateral soft tissue masses of congestive nature and a distinctive yellowish color. Kharkwal et al. have reported a similar unusual gross appearance [5].
ALHE is commonly associated with neither an elevated level of immunoglobulin E (IgE) nor an eosinophilia; in contrast, these two signs are common findings in Kiruma’s disease [1]. Eosinophilia is evident in only 20% of ALHE cases and elevated IgE levels is quite a rare finding [2]. Unexpectedly, our patient had both signs (remarkably high IgE level and eosinophilia). While Kimura’s disease surmounts the differential diagnosis list, it also includes angiomatous lymphoid hamartoma, hemangioma, pyogenic granuloma, Kaposi sarcoma, lymphoma, and epithelioid hemangioendothelioma [1].
A list of the available treatment options for ALHE in addition to hydrogen pyroxide which was effective in our case
Conclusions
What is peculiar about our case is the gross appearance and unconventional location of the intranasal lesions and the high levels of IgE and eosinophilia, which is rather uncommon in ALHE. Taking into account the highly debilitating and disfiguring nature of the disease, more efforts should be aimed towards creating a standardized and effective therapeutic approach that could help physicians treat such a recurring disease.
Notes
Acknowledgements
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Funding
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Authors’ contributions
AY: Drafted the article and collected the patient data. ARH: Participated in drafting the article and critically revising it. YY: Participated in drafting the article and critically revising it. LA: participated in data collection and drafting the article. YE: Critically revised the work for intellectual content and assisted with the diagnosis of the case. ZA: Performed the histologic examination of the biopsy and critically revised the article. All authors read and approved the final manuscript.
Authors’ information
AY is a medical student. ARH is a medical student. YY is a professor of otolaryngology. LS is a dermatologist. ZA is a professor of pathology. YE is a professor of pathology/oncology.
Ethics approval and consent to participate
Not applicable.
Consent for publication
Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
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