Dermatology

Vascular Skin Lesions

Vascular skin lesions are very common, occurring in about 40% of all children. 20 % of hemangiomas are present at birth and most begin in the first month of life.

 

Because of their frequent occurrence, it is important to have a good understanding of the present terminology of vascular skin lesion, to be able to differentiate the lesions, know when to follow them yourself and distinguish lesions that may need consultation with a pediatric dermatologist.

Hemangiomas

These are benign lesions that are composed of proliferating endothelial tissue.  The lesions are more common in females (5:1). They usually appear within a few weeks of birth, grow rapidly and then stabilize and involute by fibrosis and diminished vascularization. Usually after involuting, there is minimal cosmetic defect and <2% require active therapy. A third are gone by 3 years of age, 70% by 7 years of age, and 90% by 9 years of age.

  1. Strawberry hemangiomas – raised pink lesions that lie in the upper dermis, composed of small, closely packed blood vessels
  2. Cavernous hemangiomas – subcutaneous spongy mass with blue color
  3. Mixed - combination of cavernous and strawberry

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https://en.wikipedia.org/wiki/Hemangioma

Complications

  1. Local complications include ulceration, bleeding, and infection. Most bleeding can be controlled by simple pressure. Superficial infection can be treated with topical antibiotic ointment and non-adhering dressings. Lesions in the anogenital area are often difficult to treat and laser therapy may be indicated. 
  2. Hemangiomas in vital areas may need special treatment, especially those around the eyes that may affect vision and lead to amblyopia. There may be lesions in the airway that cause respiratory compromise and others may interfere with defecation and feeding.
  3. Kasbach-Merritt syndrome is characterized by rapid growth of hemangiomas associated with thrombocytopenia and bleeding secondary to platelet trapping and consumption coagulopathy.
  4. Disseminated neonatal hemangiomatosis - multiple skin lesions and also may have visceral hemangiomas that can lead to high output cardiac failure and bleeding
  5. Residua post regression - may have hypopigmentation, telangiectasia, scarring, and redundant skin

Treatment

  1. Reassure - Explain the natural course of the hemangiomas.
  2. Encourage families to take pictures to document either progression or involution of lesions. Comparison films help in deciding whether treatment is necessary or has been effective.
  3. If lesions are very large, growing very rapidly, interfering with vital functions, in areas that are constantly irritated, or associated with bleeding disorder or heart failure, referral to a pediatric dermatologist is recommended.
  4. Specific modes of therapy
    1. Intralesional (injected) or oral steroids
    2. Laser treatments
    3. Cryotherapy
    4. Surgical removal
    5. Cyclophosphamide
    6. Interferon

Salmon Patches (“stork bites”)

Flat vascular malformations commonly seen in newborns on the eyelids, glabella, nasal-labial areas and nape of the neck. Most fade within the first year of life, although ones on the nape are more likely to stay but are usually covered by hair and are cosmetically insignificant. They are usually bilateral. Some erythema may persist and be accentuated by exertion or crying.


Salmon patch on the left upper eyelid of a neonate
https://en.wikipedia.org/wiki/Midline_nevus_flammeus

Port Wine Stains

Flat hemangiomas composed of dilated capillaries. They are present at birth and increase in size as the child grows and do not involute by themselves. Their size may vary greatly.  These occur most commonly on the face, where they may cause considerable cosmetic disfigurement and significant emotional problems. Presently, laser therapy is helpful starting at an early age and these children should be referred to centers with experience in treating PWS. Some lesions may be effectively covered with special makeup products. 

PortWineStainHand.jpg
The back of a left hand with a prominent port-wine stain
https://en.wikipedia.org/wiki/Port-wine_stain

Sturge-Weber Syndrome

Occurs in 5-8% of patients with PWS that involve the upper eyelid or forehead. It is classified as PWS with distribution of the trigeminal nerve and includes vascular malformations in the CNS. The syndrome is associated with glaucoma, seizures, hemiplegia, and intellectual disability.

Sturge-Weber CT.jpg
CT scan of Sturge-Weber syndrome
https://en.wikipedia.org/wiki/Sturge%E2%80%93Weber_syndrome

Klippel-Trenaunay and Parkes Weber Syndromes

Vascular malformations (port wine stains and varicose veins) of a limb associated with soft tissue and bone overgrowth. 


https://en.wikipedia.org/wiki/Klippel%E2%80%93Tr%C3%A9naunay_syndrome

 Pyogenic Granulomas

Characterized by bright red, raised, often pedunculated lesions composed of endothelial elements. These may arise at the site of previous trauma. They have a tendency to bleed and should be removed by cauterization or surgery.

Pyogenic granuloma on a finger-1.jpg
https://en.wikipedia.org/wiki/Pyogenic_granuloma

Spider angioma

(nevus araneus, spider nevus) Composed of a central feeder vessel with radiating vessels that blanch with pressure. They have a predilection for upper extremities and face, but usually regress spontaneously in children.

Spider nevus.jpg
https://en.wikipedia.org/wiki/Spider_angioma

References

  1. Berman K and Zieve D.  Birthmarks. A.D.A.M. 3 October 2008.
  2. Case Records of the Massachusettes General Hospital. A Newborn Girl with a Large Cutaneous Lesion, Thrombocytopenia, and Anemia. NEJM. April 22, 2004
  3. Haggstrom A, et al. Prospective Study of Infantile Hemangiomas: Clinical Characteristics Predicting Complications and Treatment.  Pediatrics. Sept 2006
  4. Jasmin L and Zieve D. Sturge-Weber syndrome. A.D.A.M.  14 October 2009
  5. Lehrer M and Zieve D. Pyogenic granuloma. A.D.A.M. 28 October 2008
  6. MacFie C, et. al. Diagnosis of Vascular Skin Lesions in Children: An Audit and Review. Pediatric Dermatology. 2008; 25(1):7-12
  7. Chang L. et al. Growth Characteristics of Infantile Hemangiomas: Implications for Management.  Pediatrics August 2008
  8. TollefsonMM and Frieden I.  Early Growth of Infantile Hemangiomas: What Parents' Photographs Tell Us.  Pediatrics August 2012
  9. Chen,TS et al.  Infantile Hemangioms.  An U//d3siu84079czqs.cloudfront.net/sites/pedclerk/files/migrated/henab.pdfpdate on Pathogenesis and treatme nt.  Pediatrics January 2013