|Comments from Faculty and Members
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
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