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Angiolymphoid hyperplasia with eosinophilia in a hand treated with a reverse digital island flap and artificial skin: a case report
© The Author(s). 2019
- Received: 8 August 2018
- Accepted: 18 February 2019
- Published: 27 March 2019
Angiolymphoid hyperplasia with eosinophilia is a rare nodular skin tumor characterized by eosinophilic invasion and vascular proliferation. Previous reports suggested that irritation and inflammation are the causative factors of this disease. Most cases of angiolymphoid hyperplasia with eosinophilia occur around the auricle, forehead, and scalp; the hand is rarely affected. Moreover, the tumor seldom presents as multiple nodules.
A 67-year-old Japanese woman presented with a complaint of skin masses on her left thumb and index finger, which had gradually grown in size over the past few months. A biopsy was performed confirming a diagnosis of angiolymphoid hyperplasia with eosinophilia. The aponeurosis on her index finger was resected and tissue was reconstructed using a reverse palmar digital island flap harvested from the base of her index finger. The thumb lesion was also resected and covered with collagen-based artificial skin. Gradual progression of skin epithelialization followed by healing was noted 2 months after the surgery.
Angiolymphoid hyperplasia with eosinophilia is a rare tumor; it is seldom seen in the hands. It is generally treated by surgical resection. It is important to resect a sufficiently large area of the tissue due to the possibility of relapse in some cases. Furthermore, appropriate reconstruction is mandatory after wide margin tumor resection.
- Angiolymphoid hyperplasia
- Reverse palmar digital island flap
- Collagen-based artificial skin
Angiolymphoid hyperplasia with eosinophilia (ALHE) was first reported by Wells and Whimster in 1969 . It is a rare skin tumor that occurs around the auricle, forehead, scalp, shoulder, chest, oral mucosa, and scrotum; the hand is rarely affected by this disease. ALHE is a benign tumor that presents with mild symptoms such as itching during the early stages. However, as the tumor grows, severe symptoms such as bleeding or pain may appear. Several studies indicated that the first choice of treatment of ALHE is surgical resection. However, the recurrence rate of this disease is reported to be as high as 41% , thus, necessitating the resection of a sufficiently large area in the affected region to ensure surgical success. Unfortunately, wide resection margins also result in large skin deformities, thereby reducing the daily living activities of the patient. Therefore, reconstruction is an additional important component of ALHE treatment.
Here we present a rare case of ALHE of the hand in a woman.
She was under medication (hypoglycemic agents, antihypertensive drugs, and antihistamine drugs) for diabetes mellitus, hypertension, and allergic rhinitis, respectively. She had a history of smoking 10 cigarettes a day over the past 20 years. She did not drink alcohol, and there was no history of ALHE or other allergic diseases in her family. She was a housewife and was not involved in any specific occupation.
Reported instances of angiolymphoid hyperplasia with eosinophilia in the hand
Number of lesions
James M. Swinehart et al., 1979 
Arnold M et al., 1999 
left arm and hand subcutaneous
no local reccurence, but another site
B. D. Krapohl et al., 2003 
Rt.palm and ring finger subcutaneous
no recurrence in 3 months
C Conill et al., 2004 
Rt.index and middle fingers subcutaneous and bone
deformation of the nail, pain
no recurrence in 9 years
A. Satpathy, 2005 
Rt.dorsum of hand subcutaneous
no recurrence in 12 months
H Ozcanli et al., 2007 
Rt.palm, middle and ring fingers subcutaneous
itching, pain, disturbance of sensation
resection, laser treatment
no recurrence in 2 years
Nick Pappas et al., 2010 
no recurrence in 1 year
Mohammad M. Al-qattan et al., 2017 
no recurrence in 1 year
On clinical examination, it is important to differentiate between ALHE and Kimura’s disease, using both laboratory and histological tests. Similar to AHLE, Kimura’s disease is classified as a type of eosinophilic dermatitis. Although eosinophilia was observed in the blood of the patient in the current report, systemic eosinophilia is generally rare. In addition, ALHE exhibits a slow growth rate and rarely presents with lymphadenopathy (5–20% of cases) . Moreover, unlike in Kimura’s disease, the itching sensation is strong in AHLE. In many cases, immunoglobulin levels, such as that of immunoglobulin E, are normal. ALHE often involves neoangiogenesis and, accordingly, presents with plump, epithelioid endothelial cells and arteriovenous shunts . Often, ALHE lesions are located in the dermis. Kimura’s disease is probably an allergic or autoimmune response, whereas ALHE is the result of a benign neoplasm of endothelial cells caused by inflammation or stimulation . The cause of ALHE is unknown . Some clinicians speculate that it develops in response to various infections, such as herpes virus 8 or human papillomavirus-6, or injury, such as excessive contact with the rim of eye glasses [7, 8]. Meanwhile, tumor growth is reported to occur in response to changes in hormone levels during pregnancy; however, there are no clear differences between men and women [9–12]. In the present case report, our patient had numerous skin injuries on her fingers other than that affected by the lesion. She reported that she frequently fed wild cats near her home and had received several scratches from the animals in the past, which may be related to the onset of ALHE in this case.
Is it necessary to treat benign ALHE tumors?
ALHE does not develop complications during conservative therapy, and no cases of malignant transformation have been reported so far . Yet, ALHE can be exacerbated by surgical stress; hence, refraining from excision without careful consideration is critical. Some cases of ALHE also recognize genetic mutation in T cell. According to Kempf et al., ALHE is not a vascular lesion, but a type of CD4-positive T cell lymphoma [13–15]. Accordingly, careful observation is necessary, even in cases not involving surgical procedures. Early symptoms include itching and a throbbing sensation, but bleeding and pain are only observed as the tumor grows in size. Surgical resection may be indicated in the presence of more serious symptoms, such as pain, and if the patient wishes to undergo resection.
Is there a cure for ALHE other than surgical resection?
In some cases the outcomes of administration of local and systemic corticosteroids to patients with AHLE were unclear. Furthermore, laser treatment has also been provided with unsatisfactory results [16, 17]. These treatments may be effective in elderly people and in situations where surgical procedures are contraindicated (such as in individuals using anticoagulants). The primary treatment choice for ALHE is surgical resection. However, the recurrence rate following surgical excision is high.
What is critical for success in surgical resection?
Pathologically, ALHE involves vascular damage characterized by the presence of an unstable basement membrane and arteriovenous shunts [18, 19]. The removal of these abnormal vessels may be important during surgical resection. Furthermore, difficulties in identifying the margins of these lesions are believed to contribute to their high recurrence rate. Abnormal blood vessels related to ALHE often exist in the dermis; therefore, it is advisable to use natural anatomic barriers, such as the aponeurosis and fascia, as the resection margin. Several clinicians believe that ALHE resection should mirror the resection margin of malignant soft tissue tumors. It is important to examine the preoperative image before surgical treatment. In the present case study, both abnormal vessels and aponeurosis at the bottom of the tumor were excised. Recurrence is less likely when a wide resection with margins involving the dermis is performed. Reconstruction using a skin flap or artificial epithelium is recommended in cases where the deformity is large.
ALHE is a rare, benign skin tumor characterized by the presence of vascular proliferation and eosinophil invasion, and is often treated conservatively. However, when accompanied by symptoms such as pain, surgical resection of the tumor is required. In such cases, removal of a sufficiently large area of resection is important to ensure a favorable outcome. In addition, adequate reconstruction is necessary to guarantee post-surgical functions.
Special thanks to the orthopedic surgery team who assisted in the treatment of this patient.
Availability of data and materials
All data and materials related to this report are accessible at any time upon request.
NO, SN, HT, KH, and RK were involved in patient care, review of literature, and writing of the manuscript. NO, RK, and MA prepared the manuscript. All authors read and approved the final manuscript.
Ethics approval and consent to participate
This case report does not involve any active intervention on patients; therefore, ethics approval is waived.
Consent for publication
Written informed consent was obtained from the patient 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.
The authors declare that they have no competing interests.
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