Journal of Clinical and Aesthetic Dermatology

MAY 2018

An evidence-based, peer-reviewed journal for practicing clinicians in the field of dermatology

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46 JCAD JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY May 2018 • Volume 11 • Number 5 C A S E R E P O R T macular area similar to the "port-wine" stain. Over the course of time, it transforms into bluish-black, verrucous, and hyperkeratotic plaques and nodules. This type of lesion can be single or grouped and can have a linear or serpiginous arrangement. The dimensions of the lesions are typically 4- to 8cm. These lesions occur unilaterally on the lower extremities 95 percent of the time. Reactive epidermal acanthosis, papillomatosis, hyperkeratosis, ectatic vessels that begin in the papillary dermis and extend into the reticular dermis or subcutaneous adipous tissue are seen histopathologically. 7 The ectatic vessels in the lesions of our patient extended into the reticular dermis, and a prominent papillomatosis was seen. A moderate hyperkeratosis appeared only in the hyperkeratotic-looking area. Immunohistochemically, the dermal blood vessels were positive for both CD31 and CD34. 13 Considering the clinical features, the way the lesions changed over time, and histopathological features of the keratotic angiomatous lesions of the patient, we believe the lesions were more consistent with VH than with AC. The third type of the lesions clinically appeared to be AS. AS is a rare malformation of the blood vessels. The lesions are acquired, multiple, red to violaceous, punctate macules usually located in the lower extremities of girls under the age of 15 years. They enlarge peripherally, while those at the center fade, giving rise to a serpiginous pattern. They can occur in clusters and rarely follow Blaschko's lines. 3 Histologically, AS is characterized by clusters of ectatic capillaries in the papillary dermis. 2,3 Sometimes, the lesions are hyperkeratotic. 2 Considering that the lesions were present since birth, that the papules arose from punctate macules, and that the vessels extended into the reticular dermis, we ruled out AS. We believe the punctate patchy macules were more likely an early stage of VA. In the histopathology, intertwined angiomatous and lymphamatous vessels were found in many areas. The third specimen was positive for CD34, D2-40, and CD31. Moreover, in one of the areas with a lymphangiomatous lesion, a big lymphatic vessel was negative for CD34 while the small vessels just under it were positive (Figure 2C). Debelenko et al 15 stated that none of the nine verrucous angiomas were positive for D2-40 in their study. On the other hand, selective D2-40 immunoreactivity of lymphatics has been observed in normal tissues. It is unknown whether D2-40 reacts with the same or different antigens in normal lymphatics and vascular tumors. Although some markers are specific for lymphatics, they exhibit different expression patterns during prenatal development. Because some markers are expressed in both lymphatics and blood vessels in the early embryonal period, the respective immunopositivity might not indicate the lymphatic origin of a vascular tumor. In fact, because of these different staining features of vascular structures in different stages of their development, correctly interpreting the histopathology of congenital vascular tumors is a challenging and complex process. Some congenital vascular lesions might be misdiagnosed if the diagnosis is based on solely on clinical appearance without histopathological and immunohistochemical analysis, while others cannot be specifically diagnosed despite histopathological and immunohistochemical analysis. For example, Tennant et al 16 clinically diagnosed 14 similar-looking hyperkeratotic vascular lesions (most of them were raised, red-to-purple in color, variably keratotic with irregular borders, and located on an extremity) as VH, AK, AC, or capillary-venous or capillary- lymphatic malformations before a detailed histopathological examination was made. After histopathological and immunohistochemical analysis, none of the lesions were diagnosed as AK, 11 of the 14 lesions were diagnosed as VH, and three of the lesions were diagnosed as combined vascular malformations composed of capillaries, lymphatics, and veins. Tennant et al 16 also reported that, with the exception of the clinical verrucous appearances, the VHs in their patient sample had some common clinical and histopathological features, such as being present since birth, having proportionate growth, exhibiting hyperkeratotic epidermis, and containing thick-walled blood vessels that involved the entire dermis and subcutis. Our patient's verrucous-hemangiomatous lesions had similar characteristics. Additionally, Popadic et al 7 reported that there were changes in the appearance of their VH during the six-month follow-up period, according to both clinical and dermoscopic digital images. According to their report, the lesion was initially a solitary, purplish-brown plaque with a moderately rough surface. But three months later, the lesion showed significant regression and color change (turned bluish-purple). After an additional three months, the lesion had enlarged and developed into a dark, bluish-black, raised nodule with irregular borders and a hyperkeratotic surface. Based on our patient's history, her angiomatous-verrucous lesions first emerged as erythematous, punctate, smooth patches that later developed hemangiomatous vesicles, which became hyperkeratotic. The manner in which the lesions evolved was compatible with a VH diagnosis. Yet, Kim et al 17 described a 15-year-old boy who developed a solitary AK in an area of LC following repeated local trauma. They reported that, even though it was possible that the AK and LC arose independently from each other, they believed the AK was formed in the underlying LC through repeated injuries. Chen et al 2 reported a 48-year-old female patient with a late onset of AS whom developed atypical distributions (i.e., the lateral side, sole, and toes of her left foot) and had atypical clinical and histopathological (e.g., epidermal hyperkeratosis) findings. The authors reported that the lesions progressed quickly after the patient started hormone replacement therapy, and then slowed in progression after the therapy was stopped. They hypothesized that the increase in hormones induced receptors located on the surface of the endothelial cells in the skin lesions and contributed to the progression of the lesion. Similarly, regarding the genesis of AS, Neumann et al 18 suggested that dermal vessel damage due to exposure to cold might lead to an abnormal vascular response, and the formation of new capillaries might subsequently aggregate to form larger ectatic vessels. However, the lesions on our patient had been present since birth, and she did not report any exposure to repeated trauma, severe cold, or hormonal therapy. Unlike these similar combined and unusual congenital vascular lesions reported in the literature, the different lesions on our patient showed many transitions from one zone to an another, clinically and histologically. Even though, in whole, the lesion had clinical characteristics of five different types of lesions, after considering how the combined lesions changed over time, along with their intertwined histopathological features and unusual histopathological results, we concluded that the lesion was a combination of LA, LK, and VH—a hybrid entity not yet reported in the literature

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