October 10, 2016
4 min read

6-week-old girl presents with a rapidly enlarging red plaque on forehead

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Scarlett Boulos

Marissa J. Perman

A 6-week-old girl presented for evaluation of a red plaque on her forehead that started as a red-purple patch when she was aged 2 weeks, then quickly became larger and more raised over several weeks. The lesion was asymptomatic and did not bleed, but had developed some central crusting. Birth history was notable for a spontaneous vaginal delivery at term complicated by preeclampsia; the patient’s mother was 37 years old at the time of birth. The patient met all of her developmental milestones and had no other significant past medical or family history.

On exam, the patient had a 5.2 cm x 3.5 cm well-defined, brightly erythematous, soft plaque with minimal central hemorrhagic crust on the right lateral forehead in a segmental distribution. No other significant findings were noted on physical exam.

Patient presents with a brightly erythematous, soft plaque with minimal central hemorrhagic crust in a segmental distribution on the right lateral forehead.

Image: Boulos S

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Case Discussion

Infantile hemangioma (B) are the most common vascular neoplasms of infancy. They are characterized by a benign proliferation of endothelial cells during an early, rapid proliferative phase, followed by a plateau phase, and finally, an involutional phase. Superficial infantile hemangioma (IH) typically present at 2 to 3 weeks of age or at birth with an ill-defined red patch with overlying fine telangiectasia or discrete telangiectasias with a background of pallor.

During the proliferative phase, an IH can develop a more distinct superficial brightly erythematous plaque and/or a deeper violaceous-blue nodular component. Although the majority of growth occurs in the first few months, IH can continue to grow at a slower rate until about 12 months of age. The beginning of involution is marked by a change in the color from bright red to purple-grey, usually starting in the center of the lesion. Complete involution for most IH typically occurs by age 5 years.

Some IH may regress with little or no residual skin changes while others may result in atrophy, fibro-fatty tissue, overlying telangiectasia, discoloration, and other changes that may be of cosmetic or functional concern. Risk factors for developing an IH include female gender, Caucasian ethnicity, advanced maternal age, preeclampsia, placenta previa, multiple gestation pregnancy, low birth weight and prematurity. The exact pathogenesis of IH is unknown.

IH most commonly presents on the head and neck, although it can arise in any location. Larger facial hemangiomas can develop in segmental patterns corresponding to known developmental subunits, including frontotemporal (S1), maxillary (S2), mandibular (S3), and frontonasal (S4) patterns. Segmental hemangiomas are important to identify, as they have a higher risk for complications, and are more likely to be associated with extracutaneous manifestations described in PHACE(S) syndrome (Posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, sternal defects and/or supraumbilical raphe) and LUMBAR syndrome (lower body hemangioma, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies).

The patient presented in this case had a facial segmental IH in the frontotemporal (S1) distribution. Given its location and size of greater than 5 cm, it warranted further evaluation and screening for PHACE(S) syndrome with an echocardiogram, brain MRI with and without contrast, magnetic resonance angiography of the head and neck, and ophthalmology consultation. The patient had no other suspicious symptoms and no evidence of other ventral midline defects. Her workup was negative for PHACE(S) syndrome. After obtaining a normal baseline EKG, she was treated with propranolol. While most IH do not require treatment, there is a subset of high risk hemangiomas that do require intervention due to their increased risk for disfigurement, impairment of vital functions, ulceration, and possible association with visceral hemangiomas or other systemic abnormalities.

The differential includes other vascular and soft-tissue anomalies appearing in infancy such as capillary malformations (port-wine stain), congenital hemangioma subtypes NICH and RICH, and infantile myofibromatosis.

A port-wine stain (PWS) presents at birth with a pink-red, segmental, irregular patch that becomes darker red and purple with age. PWS do not spontaneously resolve and have the potential to thicken with time leading to a rubbery or firm quality. Depending on the location, PWS can be associated with underlying abnormalities and syndromes such as Sturge-Weber syndrome.

Congenital hemangioma are vascular neoplasms that are completely formed at birth with their proliferative phase occurring in utero. NICH and RICH are distinguished by their clinical course. As implied by its name, NICH does not spontaneously regress, while RICH starts its involution phase in the first weeks of life with regression completed by about 14 months of age. Congenital hemangiomas can be differentiated from IH immunohistochemically since they lack the placental markers found in IH including GLUT-1, merosin, Lewis Y antigen, and FcyRII.

Infantile myofibromatosis commonly presents on the head and neck like IH and can resemble a vascular neoplasm. It is distinguished clinically by its firm, rubbery consistency, lack of marked proliferation in the first few months, and histologically, as a fibrohistiocytic proliferation. The solitary form has an excellent prognosis with spontaneous regression, while the multicentric form can have visceral involvement and a poor prognosis. Practitioners should be aware of how to distinguish IH from other vascular neoplasms. In particular, recognizing a segmental IH and completing additional work-up can be lifesaving.

Disclosures: Boulos and Perman report no relevant financial disclosures.