Clinical Challenge: Red plaque with ulceration - MPR

Clinical Challenge: Red plaque with ulceration

Slideshow

  • Infantile hemangioma 1_0312 Derm Dx

  • Infantile hemangioma 2_0312 Derm Dx

A 3-month-old infant presents with a lesion on the scalp that her parents are concerned about. At birth, there was only a very small bruise-like lesion on the scalp that the girl’s pediatrician thought may have been due to trauma from delivery.

At three weeks of age, the “bruise” developed into a pink patch and grew into a firm, rubbery, red and slightly blanchable nodule. A week prior to visiting the dermatology clinic, the lesion developed an ulceration that seemed to be causing the infant pain. The ulceration is now crusted over.

On physical exam the baby appears well. Complete physical and neurologic exam is significant only for the red, ulcerated nodule described above. What’s your diagnosis?

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Infantile hemangiomas are common childhood soft tissue tumors. At age 1 year, 10% to 12% of white infants are affected. Risk factors for developing infantile hemangiomas include female sex, prematurity, advanced maternal age, white race, multiple gestation pregnancies, and placenta...

Submit your diagnosis to see full explanation.

Infantile hemangiomas are common childhood soft tissue tumors. At age 1 year, 10% to 12% of white infants are affected. Risk factors for developing infantile hemangiomas include female sex, prematurity, advanced maternal age, white race, multiple gestation pregnancies, and placenta previa and preeclampsia. 

Hemangiomas have three presentations:

  • Deep hemangiomas are subcutaneous, compressible tumors, often with an overlying blue tint and superficial telangectasias
  • Superficial hemangiomas appear as bright red, rubbery nodules, papules or plaques 
  • Mixed hemangiomas combines features of both deep and superficial hemagniomas 

Infantile hemangiomas are not present at birth, but become evident at 2 to 3 weeks of age; however, as many as 50% of hemangiomas develop from precursor lesions, which may be present at birth. Precursor lesions have variable appearances ranging from an area of pale skin to bruise-like to telangectasias to ulcerations.

After the hemangioma becomes evident at 2 to 3 weeks of age, it enters a proliferative growth phase, which peaks at five months. The hemangioma subsequently enters an involution phase, during which it flattens, shrinks in size, become less red and ultimately disappears. The rate of complete involution is estimated to be 10% per year, with approximately 40% of hemangiomas completely involuted after four years, 80% after eight years and so on.

In some case, involution will result in completely normal skin, but in others atrophy, scarring, fibrofatty masses or telangectasias may result. The most common complication of infantile hemangiomas is ulceration, which occurs in 16% of patients and may result in pain, bleeding, scarring and/or infection. 

Hemangiomas involving large segmental portions of the face may be associated with the “PHACES” syndrome, which stands for:

  • Posterior fossa malformations
  • Hemangioma
  • Arterial anomalies
  • Cardiac anomalies and aortic coarctation
  • Eye abnormalities
  • Sternal clefting and supraumbilical abdominal raphe

When the beard area of the face is involved, there is risk of hemangiomas involving the upper airway, which can lead to airway obstruction.

Other particularly problematic infantile hemangiomas are those involving the nasal tip, which can lead to permanent disfigurement, and those that involve the periorbital skin and eyelids, which can obstruct visual access                   

Diagnosis                       

Hemangioma diagnosis is made using classic physical exam findings and history. Biopsy is rarely indicated. 

Histologically, infantile hemangiomas stain for GLUT-1 (erythrocyte-type glucose transporter); all other forms of hemangioma are negative for GLUT-1. 

Congenital hemangiomas are fully formed at birth and may or may not involute. Diffuse neonatal hemangiomatosis describes patients with multiple cutaneous hemangiomas in addition to visceral involvement, with the most commonly involved organ being the liver.  A glomus tumor is a benign vascular lesion that most commonly presents as a painful blue papule in the nail bed. Angiolymphoid hyperplasia with eosinophilia is most common in young women and presents as subcutaneous nodules around the ears.  

Treatment and prognosis           

Most hemangiomas do not require active treatment, as the lesions will involute spontaneously. Parental education and guidance is critical during this period. 

In our practice, we recommend no therapy until the patient is school-aged in most cases.  By the time the patient enters school, the bulk of the hemangioma will have regressed spontaneously, and at this point we can offer vascular laser therapy to improve the appearance of any residual red color. 

Ulcerated hemangiomas are managed using appropriate wound care measures, as well as antibiotics and analgesics, as indicated. Pulse-dye laser may also be useful.

When hemangiomas involve the nasal tip, interfere with visual axis, involve the airway, obstruct the ear canal or have the potential to cause any morbidity or mortality, options include intralesional or oral corticosteroids, oral propranolol, vincristine or surgical excision. In our practice, we are finding oral propranolol to be a very safe and effective medication for manageming problematic hemangiomas. 

Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine in Houston.

References

1. Paller A, Mancini AJ, Hurwitz S. “Chapter 12 – Vascular Disorders of Infancy and Childhood.” Hurwitz Clinical Pediatric Dermatology. 2011; Elsevier Saunder: Edinburgh.