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Angiolymphoid hyperplasia with eosinophilia
See also in: Hair and Scalp
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Angiolymphoid hyperplasia with eosinophilia

See also in: Hair and Scalp
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Contributors: Gabriela Cobos MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Angiolymphoid hyperplasia with eosinophilia (ALHE), also known as epithelioid hemangioma, is an uncommon, benign vascular proliferation typically seen in young to middle-aged adults. There is no sex predilection.

ALHE typically presents with flesh-colored, pink, dull red, or brown papules or nodules on the head and neck. Approximately 50% of patients have multiple lesions that tend to be clustered particularly around the ears, forehead, and scalp. Other less common locations include the genitals (vulva or penis), trunk, and distal extremities. Most lesions are located dermally but ALHE can involve the subcutis and deeper soft tissues.

ALHE may also involve extracutaneous sites such as bone, colon, parotid gland, mouth, and orbit. Peripheral eosinophilia may be present in a subset of patients.

ALHE may present with pain, pruritus, or spontaneous bleeding, or it may be asymptomatic.

The underlying pathogenesis is unknown. There is no consensus as to whether the condition is reactive, possibly in response to trauma, or neoplastic.

Of note, ALHE and Kimura disease, once thought to be disease variants, are now recognized to be separate, distinct diseases with specific clinical and histologic features.

Codes

ICD10CM:
D18.01 – Hemangioma of skin and subcutaneous tissue

SNOMEDCT:
125574005 – Angiolymphoid hyperplasia with eosinophilia

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Sarcoidosis – Papules and plaques, often with a reddish-brown coloration, favor the face, lips, neck, trunk, and upper extremities. They tend to be fairly symmetric. Lesions usually have an "apple jelly" yellow-brown color upon diascopy. 
  • Cylindromas – Early lesions may present as erythematous papules on the head and neck; they usually grow and can coalesce into the "turban tumor."
  • Scleromyxedema – 2-3 mm waxy firm papules on head and neck, dorsal hands, and forearms. Distribution is symmetric, with papules often in linear array. 
  • Granuloma faciale – Classically larger reddish-brown facial plaques.
  • Leiomyomas – Painful; typically occur on the trunk.
  • Kimura disease

Best Tests

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Therapy

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References

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Last Reviewed: 10/09/2017
Last Updated: 10/01/2019
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Angiolymphoid hyperplasia with eosinophilia
See also in: Hair and Scalp
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Angiolymphoid hyperplasia with eosinophilia : Ear, Parietal scalp, Smooth papules, EOS increased
Clinical image of Angiolymphoid hyperplasia with eosinophilia
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