Abortive segmental perineal hemangioma
Published Web Locationhttps://doi.org/10.5070/D37b37m8dj
Abortive segmental perineal hemangiomaDepartment of Dermatology, New York University, New York, New York
Brook E Tlougan MD, Mercedes E Gonzalez MD, Seth J Orlow MD PhD
Dermatology Online Journal 17 (10): 8
A six-week-old girl presented with a segmental, focally atrophic, vascular patch in the diaper area, present since birth. It had undergone minimal proliferation, but had ulcerated. Evaluation to rule out LUMBAR (Lower body hemangioma/Lipoma or other cutaneous anomalies, Urogenital anomalies, Myelopathy, Bony deformities, Anorectal/Arterial anomalies, and Renal anomalies) syndrome, which included ultrasound and Doppler examination of the abdomen, spine, and pelvis, was negative. We report a unique case of an ulcerated, segmental abortive hemangioma of the anogenital area with excellent clinical response to topical timolol gel.
A six-week-old girl presented to New York University Dermatologic Associates in August, 2010, for evaluation of a vascular lesion in the diaper area, which had been present since birth. When she was born, her parents had noted a pink discoloration of the skin in the affected area, which became red over weeks. Pink and white papules appeared within the lesion. The patient was otherwise healthy, born at full-term, and was asymptomatic at the time of presentation. The patient’s father had a horseshoe kidney.
Clobetasol cream was prescribed in an effort to inhibit the lesion’s growth. Nonetheless, the hemangioma began to ulcerate and become painful. Subsequent treatments of the ulcerated areas included zinc oxide and becaplermin gel, which were ineffective. While a cardiology evaluation was underway for planned initiation of oral beta-blocker therapy, treatment with twice-daily topical timolol gel was started. With the use of timolol gel alone, there was rapid healing of several of the ulcerated areas. Ultimately, the hemangioma stabilized and required only topical therapy with timolol gel plus lidocaine jelly for relief of pain from the few remaining ulcerated areas.
In a segmental distribution on the left inguinal fold, left labium majus, and extending onto the left perianal and sacral skin, there was a slightly atrophic, geographic, red vascular plaque. Within the plaque there were several foci of pink and white papules, areas of grayish discoloration, and a few scattered, small, superficial erosions. There was no evidence of other cutaneous defects, including lipomas, skin tags, or hair. No foot deformity or discrepancy in leg length was present. The anus was patent.
Both ultrasound and Doppler examinations of the spine, abdomen, and pelvis were normal.
Abortive hemangiomas are a subtype of infantile hemangioma (IH) that are present at birth and are GLUT-1 positive. Also referred to as infantile hemangiomas with minimal or arrested growth (IH-MAG), they are most often found on the lower limbs and appear clinically as slightly atrophic plaques that are studded with pink or pale papules and telangiectases [1, 2]. This type of hemangioma often begins as a vascular patch (and is hence sometimes referred to as pseudocapillary malformation) with fine telangiectases on the surface, within which papules appear. However, they often do not undergo the proliferation phase of classic infantile hemangiomas and thus have been referred to as abortive . They have been defined as hemangiomas in which the proliferative component constitutes < 25 percent of the surface area of the lesion. Ulceration is uncommon in IH-MAGs, but, when it does arise, it is predominantly within anogenital lesions, as was observed in our case .
Although hemangiomas represent the most common neoplasm in infants, there are particular morphologies of IH, particularly segmental hemangiomas, which, although less common, are associated with a higher risk of associated complications . For example, segmental hemangiomas of the face may be associated with PHACE syndrome, which includes developmental defects of the cerebral and cardiac vessels as well as eye and chest wall abnormalities . Additionally, segmental hemangiomas that involve the pelvis or lower body also may also be associated with other extracutaneous abnormalities. Two acronyms (PELVIS and SACRAL) were proposed to describe these associated findings, but neither fully encompasses the anomalies that may be observed in these patients. Subsequent collaboration and unification of the features have led to a more inclusive and comprehensive acronym, LUMBAR, which stands for Lower body hemangioma/Lipoma or other cutaneous anomalies, Urogenital anomalies, Myelopathy, Bony deformities, Anorectal/Arterial anomalies, and Renal anomalies [4, 6, 7].
The recommended imaging guidelines of a segmental hemangioma of the lower body/pelvis vary slightly and depend upon the exact area involved. For patients younger than three months, appropriate evaluation includes ultrasound and Doppler examination of the spine, abdomen, and pelvis. For patients over three months old, any lumbar hemangioma or hemangioma in either the sacral area and/or on the perineum/genitals in a patient with myelopathy, an magnetic resonance imaging study (MRI) of the spine, abdomen, and pelvis is recommended. In patients who have sacral or genital segmental lesions without myelopathy, an MRI of only the abdomen and pelvis is appropriate. In the case of segmental hemangiomas of the lower extremities in patients over three months, a magnetic resonance angiography (MRA)/ magnetic resonance venography (MRV) of the abdomen, pelvis, and the affected limb should be performed along with standard radiographs of the lower extremities . Infantile hemangiomas that were associated with regional congenital anomalies in the recent case series that helped to elucidate the acronym LUMBAR were most often both segmental and of minimal growth or abortive; this morphology is similar to that of our patient, whose evaluation was negative. Many of the lesions in that series also tended to be extensive and involved the entire lower limb. Underlying anomalies tended to be regional and myelopathies were the most common category of associated anomaly.
The emergence of propanolol to successfully treat extensive or function-threatening hemangiomas (via decreased VEGF/bFGF expression via downregulation of the RAF/MAP kinase pathway) now provides a safer, effective alternative to systemic glucocorticoids [8, 9]. Preliminary work shows that beta blockers in topical form, such as timolol maleate gel, can successfully treat certain hemangiomas. As exemplified by the case presented here, topical timolol maleate 0.5 percent gel may be effective for ulcerated, segmental, abortive hemangiomas .
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