Pediatric Dermatology Consult

Teen boy’s knee lesion has changed

A 14-year-old male was referred to our pediatric dermatology clinic for evaluation of a lesion on the left knee that appeared at 1 year of age. The lesion has been growing with him and was not symptomatic until 6 months prior to the consultation, when it started bleeding and feeling wet.


He has a history of attention-deficit/hyperactivity disorder managed with dextroamphetamine-amphetamine. The changes noted on the knee lesion seem to occur at the same time that his ADHD medication was started.
On physical exam he had a violaceous circular plaque on the left knee.
On dermoscopy the lesion showed multiple dilated red and violaceous lacunae and whitish blue hue.

The diagnosis is:

Hemangioma

Vascular malformation

Sudoriparous angioma

Eccrine angiomatous hamartoma

Angiokeratoma

A biopsy of the lesion was performed which showed an increased number of eccrine glands and blood vessels within the dermis. Some areas showed an increase in adipocytes and smooth muscle bundles. The changes were consistent with eccrine angiomatous hamartoma (EAH).

On dermatoscopy the lesion showed multiple dilated red and violaceous lacunae and whitish-blue hue.

On dermatoscopy the lesion showed multiple dilated red and violaceous lacunae and whitish-blue hue.

The boy was referred to vascular laser therapy for treatment of the lesion.

EAH is a rare benign vascular growth characterized by an increased number of mature eccrine glands and blood vessels in the dermis and subcutis. The lesions are mostly present on the extremities, but cases of diffuse congenital lesions and lesions on the face and trunk have also been described. The lesions can be seen at birth or during the first years of life in about half of the cases, and the others tend to occur later in puberty and rarely in adulthood.1

Clinically, EAH lesions present as red, yellow to brown papules and plaques. Different dermoscopic patterns have been described which include the popcorn pattern that presents as yellow, confluent nodules with popcornlike shapes over a background of erythema, and linear arborizing vessels. The spitzoid pattern are brown globules on a background of erythema and pseudoreticular pigmentation around the globules. The verrucous hemangiomalike pattern has a bluish-white hue, reddish-blue or bluish lacunae, as seen in our patient.2-4

Most of the lesions are asymptomatic, but in some patients, they can be associated with pain, hyperhidrosis, and sometimes bleeding. Hyperhidrosis has been reported early in the presentation or during puberty or pregnancy. Our patient had started on amphetamines when hyperhidrosis occurred. Hyperhidrosis is a knowns side effect of this type of medication and may have had a role in the increased sweating noted on the hamartoma.

EAH can clinically look like verrucous hemangiomas, angiokeratomas, and vascular malformations, and histopathology may be needed to differentiate between them. Eccrine nevi and EAH can be similar. Hyperhidrosis is an early and predominant component of eccrine nevi, compared with one-third of EAH.

The exact etiology of this lesion is not known. It is thought to be caused by an abnormal differentiation of the epithelium, adnexal structure, and the mesenchyme during organogenesis.3 No other associated conditions have been described with EAH.

EAH are benign lesions that rarely require treatment. If the lesions are symptomatic or because of cosmetic reasons, they can be removed surgically. There are some reports of successful treatment with pulse dual-wavelength sequential 595- and 1064-nm lasers.5 Botulinum toxin has also been used in cases of symptomatic hyperhidrosis.

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She has no conflicts. Email her at [email protected].

References

1. Smith SD et al. Pediatr Dermatol. 2019 Nov;36(6):909-12.

2. Patterson AT et al. Am J Dermatopathol. 2016;38:413-7.

3. Garcıa-Garcıa SC et al. JAAD Case Rep. 2018;4(2):165-7.

4. Awatef Kelati et al. JAAD Case Rep. 2018;4(8)835-6.

5. Felgueiras J et al. Dermatol Surg. 2015 Mar;41(3):428-30.

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