Eccrine angiomatous hamartoma
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Synopsis
Eccrine angiomatous hamartoma (EAH) is a rare benign tumor characterized by the proliferation of eccrine and vascular elements in the dermis.
EAH typically presents as a red, violaceous, or brownish nodule or plaque on an extremity. Papules or macules are less frequently seen. The face, neck, and trunk may also be primary sites of involvement. Patients report variable levels of pain. This is thought to occur as a result of small nerves that are seen on electron microscopy in close proximity to the eccrine and vascular structures. Hypertrichosis of the tumor is encountered in most cases. Hyperhidrosis is an additional diagnostic feature that is seen in under half of reported cases.
EAH is classically solitary; however, multiple tumors in a Blaschkoid or segmental distribution have been described. EAH grows proportionately with the affected individual.
Females and males are equally affected.
EAH typically presents as a red, violaceous, or brownish nodule or plaque on an extremity. Papules or macules are less frequently seen. The face, neck, and trunk may also be primary sites of involvement. Patients report variable levels of pain. This is thought to occur as a result of small nerves that are seen on electron microscopy in close proximity to the eccrine and vascular structures. Hypertrichosis of the tumor is encountered in most cases. Hyperhidrosis is an additional diagnostic feature that is seen in under half of reported cases.
EAH is classically solitary; however, multiple tumors in a Blaschkoid or segmental distribution have been described. EAH grows proportionately with the affected individual.
Females and males are equally affected.
Codes
ICD10CM:
Q85.81 – PTEN hamartoma tumor syndrome
SNOMEDCT:
239115005 – Eccrine angiomatous hamartoma
Q85.81 – PTEN hamartoma tumor syndrome
SNOMEDCT:
239115005 – Eccrine angiomatous hamartoma
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Last Updated:07/13/2016