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Painful Purpuric Digit - Robert M. Law, M.D.

History: 

A 60 year old diabetic male spontaneously developed a painful, pupuric 5th toe. The patient underwent evaluation by a vascular surgeon for peripheral vascular disease, which was negative. An evaluation by a cardiologist failed to reveal evidence of shower emboli as well. The patient denies constitutional symptoms of infection, and states his diabetes is under excellent control. He wears well fitting shoes, and denies any history of trauma to the area. 

 

 

Figure 1. Clinical photo of affected digit. 

 

Laboratory data:

Cultures from the area were negative for both fungi and bacteria. Complete blood count, erythrocyte sedimentation rate and metabolic panel were unremarkable.

A biopsy of the site was performed, and delayed healing was noted at two weeks followup.

 

 

Figure 2. Biopsy of ulcerated, purpuric area. 

 

What is the most likely diagnosis?
a) Glomus tumor
b) Blue rubber bleb nevus
c) Hemangioma
d) Angioleiomyoma
e) Eccrine angiomatous hamartoma
f) Tufted angioma

 

Diagnosis:
Eccrine angiomatous hamartoma (EAH)

 

Discussion:
The histologic sections show superficial ulceration with subjacent dermal fibrosis, a minimal inflammatory cell infiltrate, and an expansile proliferation at the base of the biopsy. On closer examination, this focus is comprised of both thin and thick-walled vessels surrounding a proliferation of eccrine coils within the deep dermis.

This entity was first described in 1859 as an angioma-like lesion on the face of a child. Clinically, they may be red, brown, or flesh-colored, and the most common location is the extremities. They present with pain and hyperhidrosis or even hypertrichosis. Although rare, ulceration may occur, as was seen in this case. The median age affected is 10 years old, although patients as old as 73 years have been reported.

EAH is a benign lesion with no report of associated malignancy. Complete excision is recommended for symptomatic relief. In some lesions, there is infiltration of the eccrine coil/vascular units with cutaneous nerves, which may explain the intense pain often associated with this entity. The clinical differential diagnosis would include vascular malformations, tufted angioma, glomus tumor, angioleiomyoma, and blue rubber bleb nevus. The characteristic histology enables a definitive diagnosis.

 

Acknowlegments:
Special thanks to Joel Brooks, DPM of Southwest Podiatry Associates for contributing this interesting case.

 

References:
1. Eccrine angiomatous hamartoma. Pelle, M., Pride, H., Tyler, W. J Am Acad Dermatol. Sept. 2002 47(3):429-435.

2. Eccrine angiomatous hamartoma: Report of a case and literature review. Nakatsui, T, et al. J Am Acad Dermatol. July 1999 41(1):109-111