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Laboratory Data

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1. The most important question is that how can we help this baby? I’m not optimistic about laser in hemangiomas after seeing failures in a few done by my colleagues and after reading a paper about its uselessness.
2. Is there any association between ACC and hemangioma?
3. What about the maternal thyroid problem and ACC?
4. How can we reduce the pharmacists’ mistakes? Is it our responsibility to double check the medications we prescribe?

1. Frieden IJ. Aplasia cutis congenital: a clinical review and proposal of classification. J Am Acad Dermatol 1986;14:646-60
2. Fryburg JS, Greer KE. Epidermal nevus and bullous aplasia cutis congenital in a neonate. J Med Genet 1993;30:962-3
3. Batta K, Goodyear HM, Moss C, Williams HC, Hiller L, Waters R. Randomised controlled study of early pulsed dye laser treatment of uncomplicated childhood haemangiomas: results of a 1-year analysis. Lancet. 2002 Aug 17;360(9332):521-7.

Haitham Al-Qari MD, New York, NY, USA, on Jun 14, 2005

Hi, this is a fascinating case. Aplasia cutis congenita in association with hemangioma is rarely reported. Variable expression of the Adams Oliver syndrome has been reported in the literature. Extra cranial alopecia cutis worth investigating for other systemic abnormalities (Echo, parvovirus B19 antibodies….). Antithyroid drugs such as Methimazole and Carbimazole reported to cause (Aplasia Cutis Congenita).(1) I think prednisolone is excellent choice to start with, plus close monitoring for side effects. Laser therapy may be worth trying in the later stage not in the proliferative stage in which prednisolone will work better due to antiinflammatory and antiproliferative effects.

Ref: Drug effects on the fetus and breast-fed infant. Clin Obstet Gynecol. 2002 Mar;45(1):6-21.

Gangaram Hemandas FRCP, Senior Consultant Dermatologist, Department of Dermatology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia on June 14, 2005

My first impression of the case was PHACE(S) syndrome. They can get posterior fossa malformations, haemangiomas, arterial anomalies, coarctation of aorta and cardiac defects, eye abnormalities, and occassionally sternal defects, which includes supraumbilical abdominal raphe and sternal cleft.

Treatment involves close follow-up of the patient. Careful attention to neurologic status and head circumference is mandatory. MRI and magnetic resonance arteriography can delineate arterial abnormalities. Haemangiomas over the mandible and neck makes them at risk of airway obstruction- observe for stridor or respiratory distress. A careful cardiac examination with measurement in all 4 extremities to screen for coarctation of the aorta is recommended. Infants with eye abnormalities should be referred to the ophthalmologist.

Steroids may be used if there is evidence of any life or function threatening, ulceration, heart failure or facial haemangioma that cause disfigurement. Pulsed dye laser may be used as an adjunct.

Kenneth A. Arndt, M.D. Professor of Dermatology, Harvard Medical School, SkinCare Physicians of Chestnut Hill, Chestnut Hill, MA, USA on Jun 15, 2005

I’d use systemic steroids and I’d start soon. PDL Vbeam might help the superficial component of the lesions as well but won’t induce complete involution. I’d probably do both. Let me know how it goes!

Bernice Krafchik M.D. Professor Emeritus of Pediatrics and Medicine, Division of Dermatology, Toronto Hospital for Sick Children, University of Toronto, Toronto, Canada on June 30, 2005

I usually start with prednisolone 2mg/kg /day. This may well be PHACES syndrome although the anti-thyroid medication may have added to the problems. ( I know about propylthiouracil, not about the other.) It is not Adams Oliver syndrome. I don't think that laser is indicated particularly in these thick lesions