Abortive hemangiomas. Description of clinical and pathological findings with special emphasis on dermoscopy

ARTICLE

Auteur(s) : Fernando Toledo-Alberola¹, Isabel Betlloch-Mas¹, Laura Cuesta-Montero¹, Irene Ballester-Nortes¹, José Bañuls-Roca¹, Eduardo Calonje², María Teresa Martínez¹

¹Dermatology Service, Hospital General de Alicante, Avenida Pintor Baeza n° 12, CP 03010, Alicante, Spain
²Department of Dermato-Histopathology, St. Thomas's Hospital, London, United Kingdom

accepté le 8 F�vrier 2010

Infantile hemangiomas (IH) are the most frequent benign neoplasia in childhood [1]. Their well-known clinical and evolutive characteristics allow them to be easily diagnosed in most cases.

Up to now IH have been considered to develop in 3 clearly defined stages: they appear in the first weeks of life and undergo a phase of rapid growth (up to 8-12 months), followed by a period of spontaneous involution (from 1 to 12 years) and finally an involuted phase with possible residual lesions.

Up to a third of IH may be present at birth, in the form of telangiectatic papules, pinkish macules, pale areas or resembling hematomas, and these have been called precursor lesions [2]. These lesions are IH in very early stages and are difficult to distinguish from other lesions present at birth, such as congenital hemangiomas (RICH or NICH) or capillary malformations, whose prognosis and treatment are different.

In contrast, IH express an erythrocyte-type glucose transport protein (GLUT-1) in their endothelial cells, which is a highly specific marker of IH, enabling them to be differentiated from vascular lesions [3].

We describe the clinical, dermoscopic and histiological data of two patients with congenital lesions suggesting IH, whose immunohistochemical GLUT-1 confirmed this diagnosis, although they did not show the characteristic course of this type of lesion.

Case 1

On physical examination, two slightly atrophic, bluish-erythmatous plaques were seen, with a few erythematous papules, telangiectatic in appearance, covering almost all the gluteal zone and lateral aspect of her left ankle (figure 1A). The rest of the physical examination was normal.

On dermocospy, multiple oblong erythematous areas were seen, which contained fine, reddish, telangiectatic vessels within a broader network of bluish vessels (figure 1B).

The differential diagnosis between an IH precursor lesion and capillary malformation was considered. Six weeks later (at the age of two and a half months) it was verified that the plaques had not increased in size, but the number of telangiectatic papules had increased. A skin biopsy of 3 mm was carried out, which showed dilated capillary vascular lumens in the superficial dermis, together with capillary clumps in the form of lobules in the middle dermis, deep dermis and around appendages (figure 1C). GLUT-1 stain was positive for endothelial cells (figure 1D). After 6 months of follow-up the lesions remain unchanged.

Case 2

On physical examination a greyish, erythematous plaque was seen, measuring 5 × 3 cm, with a bluish peripheral halo, slightly atrophic in appearance, and telangiectatic-like papules at the level of the right groin (figure 2A). There were no other findings of note on physical examination.

On dermoscopy, multiple, oval, erythematous areas were seen, yellowish in tone, in which there were large reddish tortuous vessels. Around these zones, there were greyish-white areas with occasional bluish vascular structures (figure 2B). The differential diagnosis between IH in the involution phase and capillary malformation was considered. A 3 mm biopsy showed the existence of telangiectatic vessels with prominent endothelium in the superficial and middle dermis, together with fibrosis in the papillary and reticular dermis (figure 2C). The endothelial cells showed positive immunohistochemical GLUT-1 (figure 2D). After 8 months of follow-up the lesions remain unchanged.

Discussion

These vascular markers are non-specific and do not allow these lesions to be differentiated from other vascular proliferations. North et al., [3] after studying a series of 253 vascular lesions, found that 97% (139/143) of IH had an immunohistochemistry highly positive for the erythrocyte glucose transport protein (GLUT-1), irrespective of the stage they were at, whereas it was negative for other vascular lesions, except for a small number of angiosarcomas, which may show a weakly positive focal reaction.

This protein is not present in vessels of the dermis nor in normal subcutaneous cell tissue; however, it may be expressed in the microvascular endothelium of zones with a blood-tissue barrier function, such as the CNS and placenta [4, 5], whose embryological relationship with IH has been the subject of numerous studies [6].

The use of this highly specific marker has increased the diagnostic specificity and enabled IH to be studied as a single clinico-pathological entity [7], differentiating them from other benign vascular neoplasias and vascular malformations. In our two cases, immunohistochemical expression, which was positive for GLUT-1, allows us to affirm that they were true IH and not congenital hemangiomas (RICH or NICH) or vascular malformations (table 1). These authors, North et al., considered the possibility that certain IH which were completely developed at birth were GLUT-1 inmmunopositive; however, this hypothesis was not proved.

Subsequently, Corella et al. studied 4 patients with lesions from birth, which were similar to IH precursors but did not present the rapid growth phase and on biopsy showed some capillary-like ectatic vessels with a positive immunohistochemical GLUT-1 stains. These findings confirmed that the lesions were true IH, with an intra- uterine growth that ceases at birth, and which remain stable for the first few years of life, after which spontaneous involution probably occurs. These IH are called abortive or minimal-growth hemangiomas (AH) [8].

The presence of lesions at birth in the form of IH precursors, their diagnostic confirmation by positive immunohistochemical GLUT-1, and the lack of characteristic postnatal growth, allow us to call the lesions of our two patients AH, a subtype within the wide range of IH, whose evolutionary characteristics are different from those of other lesions.

Clinically, AH present as erythematous or bluish plaques or spots with multiple fine telangiectasias, on normal or pale pink skin. They are present from birth and do not subsequently grow [8]. In our first patient, we believe that the presentation of two lesions adopting a segmental pattern is an example of possible mosaicism in this type of lesion. AH probably appear in very early phases of development of the embryo and the presence of post-zygotic mutations may lead to the existence of an abnormal clone of cells that, if they affect all of the cell types making up the skin, would explain the appearance of genomic mosaicisms with the classic archetypal patterns [9].

AH share certain histological characteristics with IH. They present as non-encapsulated masses of pericytic and endothelial cells, forming small round vascular lumens. Two histological patterns have been described: 1) telangiectatic vessels in the papillary dermis [8], as in our second case; 2) capillary clumps in the form of lobules in the reticular dermis and subcutaneous cell tissue [8], similar to our first case.

With regard to dermoscopy, this is a non-invasive technique that makes it possible to significantly improve our diagnostic accuracy in vivo by visualizing and interpreting structures that are not visible to the naked eye [10]. In this context, if we attempt to establish a correlation between the clinical, dermoscopic and histological findings of AH, we may suppose that the erythematous telangiectatic vessels, both fine and thick and tortuous, correspond to superficial vascular dilations – the erythema of these oval areas is related to dilation of the tumoural vessels at the perianexial and middle dermis levels; and the bluish vessels are related to vascular dilations in the deep dermis. The greyish-white zones seen on physical examination and dermoscopy are probably zones in which the lesion has started to regress and histologically they correspond to areas of fibrosis.
Table 1 Differential diagnosis between CH, IH, AH and CM

Conclusion

In the future, with a larger series of patients, the dermoscopic pattern of HA will be described. We therefore propose that dermoscopy is a useful diagnostic tool in the case of AH, which allows diagnosis without the need for a biopsy.

Acknowledgements

References

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