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Viewpoints in dermatology Correspondence

Correspondence

Scoring the proliferative activity of haemangioma of


infancy: state of the art
doi: 10.1111/j.1365-2230.2011.04323.x
We read with great interest the article by Janmohomed
et al.1 in a recent issue of this journal, in which they
proposed a new scoring system, the Haemangioma Activity
Score (HAS), to evaluate and monitor the activity and
treatment outcome of infantile haemangiomas (IH).
Although greatly needed, such a standard instrument for
uniform objective scoring is still not available, and beyond
doubt, the contribution of Janmohamed et al. adds much to
its development. Nevertheless, we suggest that the proposed system has two shortcomings, related to colour and
consistency, which might impede correct assessment of
clinical improvement.
Firstly, the newly proposed system scores each colour
present in the lesion, and the total score is then divided by
the number of items scored. Scoring the colour only,
without taking into account the size of the area with this
colour, records the presence of regression, but does not
reflect its extent. This score might be useful, under certain
conditions, for assessment at a single time point, but is
imprecise for multiple periodic assessments. In addition, the
final stage of IH, either post-treatment or when left to
natural regression, commonly presents with several telangiectatic vessels on a grey or skin-coloured background.
These remaining vessels still do not represent complete
regression, but cannot be associated with any of the colour
categories proposed in the system.
Secondly, actively growing, proliferating IH are very
tense on palpation, and a change in the consistency of the
superficial area of IH from tense to soft is usually the first
indication of treatment efficacy. This change in consistency
may or may not correlate with colour changes. Thus,
although it is not the most important sign of treatment
response, change in consistency does represent regression
and, in our opinion, should therefore be included in the
scoring criteria.
Scoring IH is challenging because of interindividual
differences in their behaviour and response to treatment.
Most authors use the subjective visual analogue scale
(VAS), the Global Assessment Scale, or a measurement of
the changes in surface area, volume or thickness of IH.2

The objectivity and, more importantly, the reproducibility


of these methods remain questionable.
The proposed scoring model provides a good evaluation
of activity, but might not be precise enough to reflect
minor changes (Fig. 1). An accurate scoring system is
crucial for the assessment of activity to guide therapeutic

(a)

(b)

Figure 1 An infantile hemangioma in a 6-month-old girl after (a)


4 and (b) 12 weeks of treatment with topical timolol maleate. The
improvement is obvious by the extension of the areas of regression,
the breaks in the border and the softening of the lesion; however,
the proposed Haemangioma Activity Score would give the same
rating for both time points, indicating no improvement.

 The Author(s)
CED  2012 British Association of Dermatologists Clinical and Experimental Dermatology, 38, 89101

89

Correspondence

decision and evaluation of treatment response. Hence, we


consider that the proposed HAS needs some minor
amendments to satisfactorily reflect meaningful clinical
changes with the necessary sensitivity to become a useful
clinical tool.
K. Semkova and J. Kazandjieva
Department of Dermatology and Venereology, Medical University-Sofia,
1 G. Sofiiski Boulevard, Sofia, Bulgaria
E-mail: kristina_semkova@yahoo.com
Conflict of interest: none declared.
Accepted for publication 22 November 2011

References
1 Janmohamed SR, de Waard-van der Spek FB, Madern GC
et al. Scoring the proliferative activity of haemangioma of
infancy: the Haemangioma Activity Score (HAS). Clin Exp
Dermatol 2011; 36: 71523.
2 Berk DR, Berk EJ, Bruckner AL. A novel method for calculating the Volume of hemangiomas. Pediatr Dermatol 2011;
28: 47882.

Scoring the proliferative activity of haemangioma


of infancy: to HAS or not to HAS? Reply from
author
doi: 10.1111/ced.12056
Semkova and Kazandjieva1 recently commented on the
Haemangioma Activity Score (HAS) we developed and
reported in a recent issue of Clinical and Experimental
Dermatology.2 Although every system will have its shortcomings, we believe that the HAS remains a valuable
option for scoring the activity of infantile haemangioma
(IH).
We would like to address two clinically relevant issues
raised by Semkova and Kazandjieva. First, they point out
that the HAS does not assess the residual lesions
(telangiectatic vessels) and IH size. We understand these
concerns, but we believe that scoring for both IH size and
telangiectatic vessels, which are in fact secondary efflorescences, is not a necessity for a small, simple and rapid
system such as the HAS, and thus we did not include these
items. The HAS, unlike other systems such as the recently
published system by Haggstom et al.,3 is not very timeconsuming and does not take much time during busy
consultations. IHs grow very rapidly, and most of the
changes occur in the first year of life. After proliferation,
regression starts, and this can last for up to 10 years.
During this long period, individual IHs do not change
greatly per year, compared to the changes that occur in the
first year. Therefore, there is no need for scoring the IHs
during the later years. However, during the first year of
life, a scoring system is necessary to (objectively) measure
(re)growth or regression and, perhaps more importantly, to

90

evaluate the effect of treatment. During this first year, the


colour and swelling (if present) do change a great deal.
Consequently, the score will be different in IHs at different
time points, meaning that size does not have to be taken
into account. We saw the effectiveness of the HAS in the
early stages of treatment. It does not seem relevant that, for
example, a patient at the age of 5 years had IH in
regression with a HAS of 0.5 and at the age of 6 years
has a HAS of 0 but with telangiectatic lesions. Our score is
not a severity score that tells you whether or not to treat,
but rather an activity score that reflects whether the
therapy is working. Therefore, this system can also be used
in research. The choice of treatment is in our opinion
dependent on a number of factors, such as location (for
instance, rapidly growing IHs around the eye are more
problematic) or appearance (e.g. the presence of ulceration
or telangiectatic lesions in regressed IHs that provoke
cosmetic distress), and thus we consider that there is no
demand for severity scores.
Second, Semkova and Kazandjieva proposed that
consistency of the lesion should be taken into account.
We deliberately left this out, and in fact, considered
swelling of the IH as an alternative. In that context,
there is some overlap, but we use a different terminology.
In addition, consistency cannot be evaluated on photographs, and the fact that HAS can be performed on
photographs is an extremely important aspect of this
method.
We are currently planning a large validation study to
validate the HAS. We also encourage others to validate the
HAS and, if desirable, to compare the HAS with other
assessments, such as change in lesion consistency.
S. R. Janmohamed,1,2,3 F. B. de Waard-van der Spek,3,4
G. C. Madern,1 P. C. J. de Laat,5 W. C. J. Hop6 and
A. P. Oranje2,3,7
1
Department of Paediatric Surgery, 2Department of Paediatrics,
Division of Paediatric Dermatology, 4Department of Dermatology
and Venereology, 5Department of Paediatrics, and 6Department of
Biostatistics, Erasmus MC, University Medical Center, Rotterdam,
the Netherlands; 3KinderHaven, Havenziekenhuis, Rotterdam,
the Netherlands; and 7Department of Dermatology,
Maasstadziekenhuis, Rotterdam, the Netherlands
Email: a.oranje@inter.nl.net
Conflict of interest: none declared.
Accepted for publication 13 September 2012

References
1 Semkova K, Kazandjieva J. Scoring the proliferative
activity of haemangioma of infancy: state of the art. Clin
Exp Dermatol 2013; 38: 8990.
2 Janmohamed SR, de Waard-van der Spek FB, Madern GC,
et al. Scoring the proliferative activity of haemangioma of
infancy: the Haemangioma Activity Score (HAS). Clin Exp
Dermatol 2011; 36: 71523.

 The Author(s)
CED  2012 British Association of Dermatologists Clinical and Experimental Dermatology, 38, 89101

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