Guidelines Summary

For patients without Kasabach-Merritt syndrome with small tumors, surgical excision is first-line management. For nonresectable tumors, patients should be given 5 mg/kg/day of aspirin as the treatment of choice. In patients who are asymptomatic, careful observation for regression is an option.

For patients with Kasabach-Merritt syndrome, surgical excision is the treatment of choice if the tumor is small and localized. However, large or nonresectable tumors should be treated with intravenous vincristine plus systemic corticosteroids or oral sirolimus. Hemostasis support with platelet infusion, fresh frozen plasma, recombinant human factor VIIa, and cryoprecipitate should only be administered to patients who are actively bleeding or being prepared for surgery.

Medication

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Media Gallery

This lesion is a tufted angioma in a 1-year-old girl. Present since birth, this bluish-red hemangioma-like plaque on the patient's right upper leg slowly enlarged and became tender during the month prior to presentation. The child was otherwise well. A skin biopsy specimen revealed dilated congested capillaries in the lower dermis and subcutis. Clusters of capillaries with hyperplastic endothelial cells were also scattered in the mid and upper parts of the dermis. The epidermis revealed a slight basket-weave orthokeratosis, with minimal acanthosis, and papillomatosis. The patient was treated conservatively with only observation. The lesion remained stable and did not progress since presentation. Courtesy of National Skin Centre, Singapore.

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Author

Alexandra R Vaughn Drexel University College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Amanda T Moon, MD Clinical Assistant Professor of Dermatology, Children's Hospital of Philadelphia

Amanda T Moon, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

John G Albertini, MD Private Practice, The Skin Surgery Center; Clinical Associate Professor (Volunteer), Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine; Past President, American College of Mohs Surgery

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: QualDerm Partners; Novascan<br/>Have a 5% or greater equity interest in: QualDerm Partners - North Carolina.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mitchel P Goldman, MD Voluntary Clinical Professor of Dermatology, University of California, San Diego, Medical Center; Dermatologist, West Dermatology and Cosmetic Laser Dermatology

Mitchel P Goldman, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Cosmetic Surgery, American Academy of Dermatology, American College of Phlebology, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, American Society of Lipo-Suction Surgery, California Medical Association, Dermatology Foundation, Phi Beta Kappa, San Diego County Medical Society

Disclosure: Nothing to disclose.

Leyda E Bowes, MD Cosmetic Surgery Fellow, Department of Dermatology, Cosmetic Laser Associates of San Diego County

Leyda E Bowes, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Florida Medical Association

Disclosure: Nothing to disclose.

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Tufted Angioma Guidelines

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