Eccrine Angiomatous Hamartoma: Late Onset Facial Presentation

Abstract

Eccrine angiomatous hamartoma (EAH) is a very rare benign neoplastic condition characterized by hamartomatous proliferation of eccrine glands and accompanying blood vessels and lymphatics. These lesions are more often present at birth or appear during early infancy and childhood and present as solitary nodule or plaque with occasional pain and sweating. They are generally present on the extremities, mostly the palms and soles. We report here a case of EAH in a 20-year-old female who presented with a solitary angiomatous plaque close to the lower eyelid of the right eye with occasional pain and sweating for its rarity.

Introduction

What was known?

Eccrine angiomatous hamartoma, a very rare benign hamartoma, usually presents at birth or during early infancy as solitary nodule or plaque generally over extremities.

Eccrine angiomatous hamartoma (EAH) is characterized by benign proliferation of eccrine glands associated with capillary in close approximation with surrounding dilated capillaries. In most of the cases they are solitary and appear at birth or during childhood.[1] They may present as angiomatous nodules to erythematous-purpuric plaques with hypertrichosis, localized hyperhidrosis or occasional pain.[2] Eighty percent of the cases are reported over the extremities,[1] with a predilection for palms and soles. We report a rare case of an EAH on the face below the right lower eyelid.

Case Report

A 20-year-old female patient presented to us with complaints of solitary small red swelling on the face below the right lower eyelid, gradually increasing in size over the past 8 years. Occasionally, she noticed throbbing pain and sweating over the lesion, which aggravated during physical exertion or emotional stress. She denied the use of medications. She did not give any history of birth injury or trauma or any other symptoms suggestive of systemic involvement. There was no family history of similar illness. Her general physical and systemic examinations were normal. Cutaneous examination revealed solitary, irregular, erythematous, soft, slightly elevated plaque of about 3 × 2 cm in size having a smooth, moist surface just below the right lower eyelid [Figures 1 and 2]. On palpation, the lesion was mildly tender. Beaded drops of sweat were observed on the surface of the lesion on mild stroking and physical exertion. No pulsation or bruit was felt over the lesion. Based on the history and clinical features, a possibility of EAH was considered. This was confirmed histopathologically by the presence of circumscribed foci of thin walled capillary channels surrounding large coils of increased eccrine structures in the dermis [Figures 3 and 4]. Color Doppler study of the lesion, in addition, revealed a mild to moderate intensity vascularization in the deep dermis. Above clinicopathological features supported our diagnosis of EAH.

Figure 1.

Solitary, irregular, erythematous plaque with beaded drops of sweat below the right lower eyelid

Figure 2.

Distant view of the same

Figure 3.

Increased number of eccrine coils with surrounding dilated thin walled capillaries in the dermis (H and E, ×100)

Figure 4.

Dilated thin walled capillaries in close approximation with eccrine coils (H and E, ×400)

Discussion

EAH usually appears at birth or during childhood,[1] although in our case it appeared during adolescence. EAH presenting with a blue-colored, ill-defined swelling over the dorsum of left hand in a 26-year-old female has recently been reported.[3] There are a few recent reports of EAH cases presenting in adulthood.[4,5] Generally, they enlarge very slowly; more rapid growth has been described during pregnancy and adolescence.[6] It is usually sporadic in nature but familial occurrence has also been reported.[7] The bluish-red or frankly angiomatous smooth nodules or plaques are usually solitary, although multiple lesions have also been described.[1,7] An unusual presentation of a verrucous lesion over the gluteal region in an elderly person has been reported.[8] Lesions mostly favor sites of increased number of eccrine glands like extremities, but there are case reports of occurrence over face[9] and trunk. The infraorbital presentation in our case was very unusual. The lesions are mostly asymptomatic although pain spontaneous or on pressure, may be encountered and might be due to involvement of nerve fibers. They often have overlying hyperhidrosis, as in our case, probably due to stimulation of eccrine glands by the local temperature generated within the angioma.[10] This helped us to differentiate the lesion from tufted angioma and other vascular malformations. Lesional hypertrichosis and the association of EAH with Cowden's syndrome,[11] neurofibromatosis,[12] etc., have all been reported.

Histopathologically, the large secretory eccrine coils are surrounded by thin walled blood vessels and lymphatic channels in the deep dermis with occasional invasion of adipose tissue.[13] The presence of less number of capillary channels in our case helped us to differentiate it from sudoriparous angioma where numerous such vessels are seen. When there are nonspecific clinical findings, common differentials like nevus flammeus, glomus tumor, smooth muscle hamartoma, blue rubber bleb nevus syndrome, angiokeratoma, and macular telangiectatic mastocytosis can readily be differentiated by histopathology. Color Doppler study, as was done in our case, is not diagnostic but along with magnetic resonance imaging (MRI) can delineate the extent of the lesion. Immunohistochemical studies to demonstrate S-100 protein, carcinoembryonic antigen (CEA) and antifactor VIII-related antigens can be helpful,[10] but were not done for institutional nonavailability.

Although botulinum toxin, lasers, and sclerosants are now being used as treatment options, we referred the patient to plastic surgery outpatient department (OPD) for excision and repair.

The unusual location of EAH in our case and the paucity of its mention in the literature prompted us to report this case.

What is new?

EAH can unusually present late, during adolescence over unusual site like face.