Eccrine angiomatous hamartoma

Eccrine Angiomatous Hamartoma (EAH) is a rare, benign, cutaneous condition characterized by an abnormal proliferation of eccrine sweat glands and blood vessels. It is a type of hamartoma, which is a benign, focal malformation that resembles a neoplasm in the tissue of its origin. EAH typically presents at birth or during early childhood, but cases of late onset have also been reported.

Etiology and Pathogenesis

The exact cause of Eccrine Angiomatous Hamartoma remains unknown. It is considered a developmental anomaly rather than a true neoplasm. The lesion is characterized by an increased number of normal or slightly dilated eccrine sweat glands, accompanied by an abnormal proliferation of blood vessels. Some theories suggest a genetic component, although no specific genetic markers have been identified.

Clinical Features

EAH usually presents as a solitary, well-demarcated, flesh-colored to slightly bluish nodule or plaque. It is most commonly located on the extremities, particularly the lower legs, although it can occur anywhere on the body. The size of the lesion can vary significantly. Some patients may experience localized hyperhidrosis (excessive sweating) or pain in the affected area. Rarely, hair and fat may also be components of the hamartoma, leading to a more complex clinical presentation.

Diagnosis

The diagnosis of Eccrine Angiomatous Hamartoma is primarily clinical, supported by histopathological examination. A biopsy of the lesion will show an increased number of eccrine sweat glands and capillary proliferation within the dermis. Special stains, such as PAS (Periodic Acid-Schiff), can help highlight the eccrine structures. Imaging studies are not typically required but can be used to assess the extent of the lesion in complex cases.

Treatment

Treatment of EAH is not always necessary, as the condition is benign and asymptomatic in many cases. For symptomatic lesions, or if cosmetic concerns warrant, options include surgical excision, laser therapy, or cryotherapy. Complete excision is curative but may not be feasible for large or deeply situated lesions. Recurrence after treatment is rare.

Prognosis

The prognosis for Eccrine Angiomatous Hamartoma is excellent, as it is a benign condition. However, the presence of symptoms such as pain or excessive sweating can impact the quality of life and may require intervention.

Epidemiology

EAH is a rare condition, with limited epidemiological data available. It affects both males and females and has been reported in various ethnic groups. The lesion is usually congenital or appears in early childhood, but cases with onset in adulthood have also been documented.

See Also

• Hamartoma
• Eccrine sweat glands
• Hyperhidrosis
• Benign skin neoplasms