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Adult onset eccrine angiomatous hamartoma

  • Author(s): Mehta, Vandana
  • Balachandran, C
  • Rao, Laxmi
  • et al.
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Adult onset eccrine angiomatous hamartoma
Vandana Mehta MD DNB1, C Balachandran MD1, Laxmi Rao MD2
Dermatology Online Journal 15 (10): 18

1. From the Dept of Skin & STD, Kasturba Medical College, Manipal, Karnataka, India. vandanamht@yahoo.com
2. From the Dept of Pathology, Kasturba Medical College, Manipal, Karnataka, India


Abstract

Eccrine angiomatous hamartoma (EAH) is a benign hamartoma containing eccrine glands and blood vessels. It may be present at birth or may develop during childhood. We report a patient with onset in adulthood.



Introduction

Eccrine angiomatous hamartoma (EAH) is a rare benign hamartoma of the eccrine glands and blood vessels in the dermis. It was first described by Beier in 1895, but, the term EAH was coined by Heyman et al. in 1968 [1]. Eccrine angiomatous hamartoma may be congenital or appear later in childhood. We report a rare case of adult onset eccrine angiomatous hamartoma in a young male.


Figure 1Figure 2

A 28-year-old male presented with a solitary hyperpigmented plaque on his right leg of 2 months duration that had gradually increased to the present state. There was no history of trauma prior to onset. However, the patient reported pain and associated hyperhidrosis over the plaque, which was provoked by physical exercise and emotional stress. Cutaneous examination revealed a solitary 2 x 2 cm hyperpigmented plaque on the right leg with beads of perspiration (Fig. 1). A biopsy for histopathology revealed eccrine gland hyperplasia interspersed with numerous thick walled vascular channels and periadnexal myxoid degeneration in the dermis suggestive of eccrine angiomatous hamartoma (Figs. 2-4).


Figure 3Figure 4

Eccrine angiomatous hamartoma typically occurs as a solitary angiomatous nodule or plaque on the lower extremities at birth or during early childhood. The lesions may be painful and demonstrate hyperhidrosis, especially when they are manipulated. The hyperhidrosis is presumably an expression of its eccrine component [2]. Although usually solitary, several cases with multiple lesions have been described [3]. On histopathology, EAH is characterized by a dermal proliferation of well-differentiated eccrine secretory and ductal elements closely associated with thin walled angiomatous channels. In addition hyperplasia of other dermal constituents such as fat, nerve fibers, pilar structures, and mucin has also been reported, as was seen in our case [4].

The exact etiology of EAH is not known. The involvement of multiple structures (eccrine glands, blood vessels, and mucin) indicates the complex hamartomatous nature of this skin lesion, the origin of which has been attributed to an abnormal induction of epithelial adnexal and mesenchymal differentiation by several authors [5].

The clinical differential diagnosis of EAH includes nevus flammeus, tufted angioma, glomus tumor, blue rubber bleb nevus syndrome, and angiokeratoma, each of which can be readily differentiated by histopathology. Sudariparous angioma has been considered by some authors to be synonymous with EAH in view of the angiomatous component in both, but published reports state that they differ in the size of blood vessels. Also, eccrine gland hyperplasia is essentially a component of EAH and is not seen in sudariparous angioma. The presence of pain or tenderness to palpation and lesional hyperhidrosis may also assist in the diagnosis, and are seen in 34 percent and 42 percent of EAH cases respectively [6].

Worldwide, 46 cases of EAH have been reported of which we could find only 2 cases in which the onset was later in adulthood, as in our case [5]. Considering the benign nature of EAH, aggressive treatment is not indicated. However, surgical excision may be needed for cases with pain or severe hyperhidrosis.

References

1. Heyman AB, Harris H, Brownstein MH. Eccrine angiomatous hamartoma. NY State J Med 1968;68:2803-06.

2. Pelle MT, Pride HB, Tyler WB. Eccrine angiomatous hamartoma. J Am Acad Dermatol 2002;47:429-35. [PubMed]

3. Garcia-Arpa M, Rodriguez-Vazquez M, Cortina-de la Calle P, Romero- Aguilera G, Lopez Parez R. Multiple and familial eccrine angiomatous hamartoma.Acta Derm Venereol 2005;85:355-57. [PubMed]

4. Galan A, Mc Niff JM. Eccrine angiomatous hamartoma with features resembling verrucous hemangioma. J Cutan Pathol 2007;34:68-70 [PubMed]

5. Sugimoto Y, Araki E, Inoguchi N, Matsumura Y, Kore-eda S, Horiguchi Y, Miyachi Y, Utani A. Adult onset multiple eccrine angiomatous hamartoma in enlarging hairy plaques. Acta Derm Venereol 2007;87:180-1. [PubMed]

6. Diaz-Landacta L, Kerdel FA. Hyperhidrotic painful lesion. Arch Dermatol 1993;129:107-110. [PubMed]

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