A 19-year-old patient presents complaining of ‘blisters” on her abdomen that intermittently leak clear fluid and occasionally bleed. These have been present since she was a little girl.
Lymphangioma circumscriptum is a type of congenital malformation of lymphatic vessels. It can occur anywhere on the skin or in the mouth (including the lips), but favors the proximal limbs and chest. Physical exam reveals clear or hemorrhagic vesicles resembling...
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Lymphangioma circumscriptum is a type of congenital malformation of lymphatic vessels. It can occur anywhere on the skin or in the mouth (including the lips), but favors the proximal limbs and chest. Physical exam reveals clear or hemorrhagic vesicles resembling frogspawn. These vesicles sometimes develop a warty appearance.
Additional clinical findings are intermittent swelling, hemorrhage and leakage of lymph from superficial vesicles. Lesions are often much more extensive than clinically expected from the number of vesicles.
Treatment & Prognosis
Treatment of lymphangioma circumscriptum is challenging. Surgical excision has a high recurrence rate because lesions typically have a deeper component. Other therapies include laser surgery, radiofrequency ablation, cryoablation and sclerotherapy.
Patients with lymphangioma circumscription have a normal life expectancy. Affected patients usually seek medical intervention for cosmetic reasons.
The other answer choices are primary lymphedemas that results from defects in the lymphatic circulation and are categorized according to the age of onset as congenital (Milroy disease), praecox (Meige disease) or (later in life) tarda.
The edema is usually painless and initially pitting. Over time, the skin overlying the edema becomes non-pitting, hyperpigmented, warty and even painful.
Milroy syndrome is a rare lymphedema disorder. Affected individuals typically have lymphedema in their lower legs and feet at birth or develop it in infancy.
Lymphedema praecox is the most common form of primary lymphedema. It develops between puberty and age 35 years. It affects four times as many females than males, and a common characteristic is that only one leg is affected.
Meige disease is an autosomal dominant familial form of lymphedema praecox with peripubertal onset. The swelling typically occurs in the lower legs and feet.
Lymphedema tarda begins after the age of 35 years and often presents with lower extremities involvement.
Nelly Song, BS, is a medical student at Baylor College of Medicine.
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 also in Houston.
- Bolognia J, Jorizzo J, Rapini R. Dermatology. St. Louis, Mo.: Mosby/Elsevier, 2008. pp 1723.
- Connell F et al. “A new classification system for primary lymphatic dysplasias based on phenotype.” Clin Genet. 2010; 77(5):438-52.
- Brice G et al. “Milroy disease and the VEGFR-3 mutation phenotype.” J Med Genet. 2005; 42(2): 98-102.