Superimposed segmental manifestation of cherry angiomas

ARTICLE

ejd.2011.1513

Auteur(s) : Teresa Jaeger, Christian Andres, Rüdiger Hein, Johannes Ring, WenChieh Chen wenchieh.chen@lrz.tum.de

Department of Dermatology and Allergy, Technische Universitlsquät München, Munich, Germany

Reprints: W. Chen

Cherry angioma, or senile angioma, is probably the most common cutaneous vascular lesion. It increases in number and size with time after its onset in adulthood. Histopathology demonstrates the capillary nature of the proliferating vessels [1]. Little is known about its etiology and pathogenesis. Association with viral infections, hormone factors, immunosuppression and exposure to various chemical compounds have been reported [1, 2].

In autosomal dominant skin disorders, lesions in a rather pronounced segmental distribution superimposed on the ordinary non-segmental phenotype are sometimes noted [3]. Such type 2 segmental manifestation has been observed in at least 20 different autosomal dominant skin diseases [3], with a strikingly high frequency documented in cutaneous leiomyomatosis, glomangiomatosis and disseminated superficial actinic porokeratosis. A similar superimposed segmental involvement has been described in several common disorders with a polygenic background, such as psoriasis, lichen planus, or vitiligo [4]. Here we report a case of eruptive cherry angiomas arranged in a superimposed segmental manifestation.

Case report

A 62-year-old woman presented with multiple, asymptomatic, red-to-violaceous, up to 5 mm, dome-shaped to polypoid papules, being sharply midline-demarcated and predominantly clustered on the left lower quadrant of the abdomen with extension via the left flank area to the left half of the back (figure 1A). According to the patient, the skin lesions first appeared eruptively at the age of 30 years, about one year later after she gave birth to her second child, and continued to increase in such a segmental distribution for a time period of approximately 10 years, then remained unchanged. Both her children were breast-fed for 6-9 months. For the past 10 years, after the menopause, similar lesions gradually developed with age on the trunk and proximal extremities but in a random non-segmental pattern (figure 1A) . On the rear side, we observed a segmental arrangement of multiple cherry angiomas on the dorsal side of the right thigh, as well as several non-segmental lesions scattered on the back and left thigh (figure 1B). Further general examination and laboratory data are otherwise unremarkable, and her personal and family medical histories are irrelevant. Histopathological examination showed a well-circumscribed vascular lesion composed of numerous dilated blood vessels in the papillary dermis with loss of the overlying epidermal rete ridges (figure 1C).

Discussion

Two types of segmental manifestations of autosomal dominant genodermatoses have been proposed: Type 1 reflects heterozygosity for a postzygotic new mutation, whereas type 2 results from loss of the corresponding wild-type allele occurring in a heterozygous embryo, giving rise to rather pronounced segmental lesions that are superimposed on the ordinary non-segmental phenotype [3]. A molecular confirmation of the concept of type 2 segmental manifestation has recently been demonstrated in a case of Hailey-Hailey disease [5]. Among the described examples of type 2 segmental manifestation, only glomangiomatosis and blue rubber bleb nevus syndrome involve the vessels, whereas the others are mainly of epidermal or follicular origin [3]. Sporadic cases of angioma serpiginosum may also represent a type 2 segmental manifestation [6]. As a rule, type 2 segmental involvement of an autosomal dominant skin disorder tends to appear at a rather young age, thus preceding the onset of non-segmental lesions by years [3].

On the other hand, more and more polygenic skin diseases have also been observed to show a segmental arrangement of the lesions associated with a milder non-segmental involvement, the so-called superimposed segmental manifestation [3, 4]. Examples include psoriasis vulgaris, pustular psoriasis, atopic dermatitis, lichen planus, lichen planopilaris, systemic lupus erythematosus, dermatomyositis, pemphigus vulgaris, vitiligo, graft-versus-host disease, granuloma annulare, erythema multiforme, ibuprofen-induced drug eruption, and infantile hemangioma [3, 4, 7-9]. Our case represents a further example of superimposed segmental manifestation of a common skin disorder with a polygenic background.

It is unclear whether the eruptive occurrence of cherry angiomas in our patient was triggered by hormonal changes associated with breastfeeding. However, a possible pathogenic role of prolactin has been observed in two women with hyperprolactinemia [1]. On the other hand, a recent study showed that a decreased mir-424 expression and an increased levels of MEK1 (mitogen-activated protein kinase) or cyclin E1 may cause abnormal cell proliferation in cherry angiomas [10].

In summary, cherry angiomas can occur in a superimposed segmental manifestation. Hormonal changes may be a triggering factor.

Disclosure

Acknowledgments: We would like to thank Prof. Rudolf Happle for his critical comments. Financial support: none. Conflicts of interest: none.

References

1. Sangüeza OP and Requena L. Pathology of vascular skin lesions: Clinicopathologic correlations. Totowa, New Jersy: Humana Press, 2010.

2. De Felipe I, Redondo P. Eruptive angiomas after treatment with cyclosporine in a patient with psoriasis. Arch Dermatol 1998 ; 134 : 1487-1488.

3. Happle R. Superimposed segmental manifestation of both rare and common cutaneous disorders: a new paradigm. Actas Dermosifiliogr 2009 ; 100 : Suppl. 177-85.

4. Happle R. Superimposed segmental manifestation of polygenic skin disorders. J Am Acad Dermatol 2007 ; 57 : 690-699.

5. Poblete-Gutiérrez P, Wiederholt T, König A, et al. Allelic loss underlies type 2 segmental Hailey-Hailey disease, providing molecular confirmation of a novel genetic concept. J Clin Invest 2004 ; 114 : 1467-1474.

6. Chen W, Liu TJ, Yang YC, Happle R. Angioma serpiginosum arranged in a systematized segmental pattern suggesting mosaicism. Dermatology 2006 ; 213 : 236-238.

7. Bussmann C, Happle R, Baar W, Bieber T, Haidl G, Novak N. Superimposed linear lichen planopilaris: another polygenic disorder exemplifying a new genetic concept. Eur J Dermatol 2010 ; 20 : 269-270.

8. Happle R. Superimposed segmental hemangioma of infancy. Dermatology 2010 ; 220 : 180-182.

9. Boente Mdel C, Nadra G, Asial R, Happle R. Pronounced linear calcinosis in a boy with mild dermatomyositis A further possible example of superimposed segmental manifestation of a polygenic disorder. Dermatology 2009 ; 219 : 155-157.

10. Nakashima T, Jinnin M, Etoh T, et al. Down-regulation of mir-424 contributes to the abnormal angiogenesis via MEK1 and cyclin E1 in senile hemangioma: its implications to therapy. PLoS One 2010 ; 5 : e14334.