Angiolymphoid hyperplasia with eosinophilia

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Angiolymphoid hyperplasia with eosinophilia

William Y-M Tang and Loi-yuen Chan

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Angiolymphoid hyperplasia with eosinophilia (ALHE) was first described by Wells and Whimster in 1969. It is a benign vascular proliferation of unknown etiology with a characteristic component of epithelioid endothelial cells. It is an uncommon disease, so data on its natural course and treatment response are based on a small number of patients.

ALHE usually affects women in their third decade and presents as cutaneous papules or subcutaneous nodules, sometimes with inflammatory features, on the head, neck, and periauricular region. Involvement elsewhere is rare. Approximately 20% of patients have blood eosinophilia. Malignant transformation has not been observed. Although benign in nature, there may be disfigurement, bleeding, and pain. The etiology of ALHE is unknown, but neoplastic proliferation of vascular tissue, or reactive hyperplasia of vascular tissue secondary to trauma, infection, renin, or hyperestrogenic states have been proposed as causal factors.

The previous alleged overlap with Kimura’s disease is incorrect: ALHE and Kimura’s disease are separate clinicopathological entities. Kimura’s disease is a chronic inflammatory condition of unknown etiology often affecting young male Orientals, and typically presents as cervical lymphadenopathy and subcutaneous nodules in the head and neck region. It is often associated with blood and tissue eosinophilia, and raised serum IgE.

Specific investigations

Microscopically, ALHE is characterized by a proliferation of capillaries and small vessels with plump, round, oval, or cuboidal endothelial cells that protrude into the lumen, creating a cobblestone appearance. There is also a perivascular lymphocytic and eosinophilic infiltrate.

The location and extent of underlying vascular anomalies may be assessed by angiography, angiomagnetic resonance imaging, and angio-computed tomography.

Owing to its predominant occurrence in females, hyper-estrogenic states may have a causative role in ALHE. However, successful treatment of ALHE using hormone therapy has not yet been reported.

Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature.

Zarrin-Khameh N, Spoden JE, Tran RM. Arch Pathol Lab Med 2005; 129: 1168–71.

The authors report a 33-year-old woman who developed ALHE in her right ear during the second trimester of pregnancy. The lesion was completely excised. The authors also reviewed a total of five other ALHE cases associated with pregnancy. Lesions in some of these patients increased in size during pregnancy. One patient improved with cessation of oral contraceptive pills while another patient had her lesions reduced in size by half during the postpartum period. Two patients had skin biopsy which showed significant amounts of estrogen and progesterone receptors but not detected in the uninvolved skin.

First-line therapies

image Surgery D
image Laser therapy D
image Corticosteroid, topical or intralesional E
image Cryotherapy E

Angiolymphoid hyperplasia with eosinophilia treated with vascular laser.

Alcántara González J, Boixeda P, Truchuelo Díez MT, Pérez García B, Jaén Olasolo P. Lasers Med Sci 2011; 26: 285–90.

The sequential laser combines 595 nm PDL with 1064 nm Nd : YAG and targets structures at different dermal levels using two different wavelengths. Greater effectiveness and lower recurrence rate are possible with the Nd : YAG laser which allows the destruction of deeper vessels than PDL. Three patients with ALHE were treated with this sequential laser system. Complete resolution was presented in two of them 3 years after their last treatment, and the other one showed a marked improvement.

Treatment using PDL is based on the principle of selective photothermolysis, causing destruction of blood vessels in the lesions. PDL should be a first-line treatment for ALHE due to its reasonably good results with low incidence of side effects. Combination with Nd : YAG laser produces better results. Carbon dioxide and argon laser have also been used with success in treating ALHE, but their use is limited because of the greater chance of post-treatment scarring.

Second-line therapies

image Imiquimod E
image Tacrolimus E
image Isotretinoin E
image Radiofrequency ablation and sclerotherapy E

Third-line therapies

image Interferon-α2b E
image Anti-IL-5 antibody E
image Suplatast tosilate E
image Photodynamic therapy E
image Radiotherapy E

Angiolymphoid hyperplasia with eosinophilia: successful treatment with the antiallergic agent suplatast tosilate.

Harada K, Kambe Y, Takeda H, Nakano H, Hanada K. Dermatology 2004; 208: 176–7.

Suplatast tosilate is an anti-allergic agent on the market in Japan. It was reported to exert an inhibitory effect of IL-4 and -5 production in helper T cells, resulting in reduced eosinophil infiltration and suppression of IgE production in B cells. A 32-year-old man with ALHE not responding to topical steroid, cryotherapy, indomethacin farnesil, and oral betamethasone received suplatast tosilate 300 mg/day. A reduction of pruritus was noted after 1 week and a reduction of nodule size after 1 month of treatment. The skin lesions almost disappeared after 5 months of treatment and the dose was reduced to 200 mg/day.

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Angiolymphoid hyperplasia with eosinophilia

William Y-M Tang and Loi-yuen Chan

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Angiolymphoid hyperplasia with eosinophilia (ALHE) was first described by Wells and Whimster in 1969. It is a benign vascular proliferation of unknown etiology with a characteristic component of epithelioid endothelial cells. It is an uncommon disease, so data on its natural course and treatment response are based on a small number of patients.

ALHE usually affects women in their third decade and presents as cutaneous papules or subcutaneous nodules, sometimes with inflammatory features, on the head, neck, and periauricular region. Involvement elsewhere is rare. Approximately 20% of patients have blood eosinophilia. Malignant transformation has not been observed. Although benign in nature, there may be disfigurement, bleeding, and pain. The etiology of ALHE is unknown, but neoplastic proliferation of vascular tissue, or reactive hyperplasia of vascular tissue secondary to trauma, infection, renin, or hyperestrogenic states have been proposed as causal factors.

The previous alleged overlap with Kimura’s disease is incorrect: ALHE and Kimura’s disease are separate clinicopathological entities. Kimura’s disease is a chronic inflammatory condition of unknown etiology often affecting young male Orientals, and typically presents as cervical lymphadenopathy and subcutaneous nodules in the head and neck region. It is often associated with blood and tissue eosinophilia, and raised serum IgE.

Specific investigations

Microscopically, ALHE is characterized by a proliferation of capillaries and small vessels with plump, round, oval, or cuboidal endothelial cells that protrude into the lumen, creating a cobblestone appearance. There is also a perivascular lymphocytic and eosinophilic infiltrate.

The location and extent of underlying vascular anomalies may be assessed by angiography, angiomagnetic resonance imaging, and angio-computed tomography.

Owing to its predominant occurrence in females, hyper-estrogenic states may have a causative role in ALHE. However, successful treatment of ALHE using hormone therapy has not yet been reported.

Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature.

Zarrin-Khameh N, Spoden JE, Tran RM. Arch Pathol Lab Med 2005; 129: 1168–71.

The authors report a 33-year-old woman who developed ALHE in her right ear during the second trimester of pregnancy. The lesion was completely excised. The authors also reviewed a total of five other ALHE cases associated with pregnancy. Lesions in some of these patients increased in size during pregnancy. One patient improved with cessation of oral contraceptive pills while another patient had her lesions reduced in size by half during the postpartum period. Two patients had skin biopsy which showed significant amounts of estrogen and progesterone receptors but not detected in the uninvolved skin.

First-line therapies

image Surgery D
image Laser therapy D
image Corticosteroid, topical or intralesional E
image Cryotherapy E