Angioma serpiginosum with sole involvement

ARTICLE

Auteur(s) : Dilek Bayramgurler¹, Deniz Filinte², Rebiay Kiran¹

¹Dept of Dermatology, Kocaeli Universitesi Dermatoloji AD Umuttepe, 41080 Kocaeli, Turkey
²Dept of Pathology, Kocaeli Universitesi Dermatoloji AD Umuttepe, 41080 Kocaeli, Turkey

accepté le 11 Juin 2008

Angioma serpiginosum (AS) is a rare, acquired, vascular disorder, asymptomatic, and histopathologically characterized by clusters of dilated capillaries in the papillary dermis with multiple, red to violaceous, punctate macules clinically [1, 2] The lesions evolve in groups forming a serpiginous pattern or small rings [3] and rarely follow the lines of Blaschko [4-6]. The lesions are mostly located on the lower extremities [1, 3] but any region of the body might be involved, except the palms, soles and mucocutaneous junctions [3]. We report a case of AS following the lines of Blaschko along the whole left lower extremity, also involving the left sole.

Case report

Dermatological examination revealed multiple, grouped, punctuate, red to violaceous non-blanchable macules extending from the left thigh down the left leg and to the left plantar surface in a linear distribution (figure 1). Genital and oral mucous membranes were normal.

Histopathological examination of the punch biopsy specimen taken from the medial part of the sole showed dilated capillaries in the upper dermis without any pigment incontinence or inflammatory findings. The epidermis was hyperkeratotic but it was consistent with the biopsy site (figure 2). Immunohistochemical examination of the involved blood vessels showed the absence of estrogen and progesterone receptors. Routine laboratory examination was normal. Systemic examinations, including fundoscopic examination of the eyes, also revealed no abnormalities. Based on her clinical and histopathological findings the patient was diagnosed as AS and reassured.

Discussion

It is not clear yet whether AS represents a nevoid vascular malformation or a vascular nevus [7, 8]. AS lesions following the lines of Blaschko have rarely been reported as noted above. Moreover, Chen et al. [8] presented a 15-year-old girl with AS presenting in an asymmetric, systematized, segmental pattern in their recent report. They reported that these 2 cutaneous patterns reflect cutaneous mosaicism and suggested that AS might be best categorized as a vascular nevus. On the other hand, familial cases show a rather symmetrical, non-segmental involvement [10]. Since more than 90% of cases are females and the disease progresses during pregnancy, increased levels of estrogens have been blamed in the etiology [6, 9]. On the other hand, Erkek et al. [9] revealed no estrogen or progesterone receptors within the involved blood vessels in an immunohistochemical examination of their middle aged woman patient, as well as a normal hormonal profile, suggesting no role of hormonal stimulus in the pathogenesis of AS. Our immunhistochemical analysis also revealed the absence of estrogen and progesterone receptors within the involved vessels. We think that these observations might be a clue that those hormones do not play a role in the initiation of this condition. An abnormal vascular response to cold was another factor which has been proposed in the pathogenesis of AS [6]. There was no cold exposure in our patient.

Systemic involvement is rare in AS. Only 3 patients, associated with retinal and spinal nerve involvement in one [11] and retinal involvement in the other [6, 12], have been reported in the literature. Ocular examination of our patient revealed no abnormal findings.

The disease is usually asymptomatic. Spontaneous resolution with partial regression may occasionally be seen. If the lesions cause severe disfigurement, pulsed dye or argon laser treatment can be used with successful results. We present this case to emphasize that involvement of the sole may also be seen in AS. As reported before [9], we also think that hormones do not play a direct role in the pathogenesis of this condition, on the basis of an absence of estrogen and progesterone receptors in the lesions. AS lesions arranged in a segmental pattern reminiscent of the lines of Blaschko, as in our case, are not rare and this finding might be a manifestation of cutaneous mosaicism which was first reported by Chen et al. [8] recently.

Acknowledgements

References

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