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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44 JCAD JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY May 2018 • Volume 11 • Number 5 C A S E R E P O R T examination was performed, and routine laboratory testing included a complete blood count; platelet aggregation; prothrombin time; partial tromboblastin time; bleeding time; erythrocyte sedimentation rate; and estrogen and progesterone levels, and biochemistry panel. No pathology was detected. The patient had no neurological or additional vascular findings. A superficial tissue ultrasonography of the lesional area did not show any subcutaneous vascular involvement. The Doppler angiography of the left leg, abdominal ultrasonography, and magnetic resonance imaging of the left leg, pelvis, and abdomen showed no abnormalities. Excisional skin biopsies were taken from the lesions, and preliminary diagnoses of lymphangioma (LA), LK, hemangioma (HA), angiokeratoma circumscriptum (AC), VH, and AS were made. Histopathological examinations of the biopsy specimens showed three distinct histological features. The first biopsy specimen was taken from the area around the border of what clinically appeared to be LA and LK, and showed dilated vessels and a slight perivascular lymphocytic infiltration in the papillary dermis in both sites. Additionally, mild acanthosis and mild focal hyperkeratosis were detected in the epidermis in the LK site (Figure 2A). Immunohistochemial staining of this area revealed prevalence of D2-40 (Figure 2B), but not CD34 (Figure 2C). An examination of the second specimen, which was taken from the area around the border of what clinically appeared to be HA and keratotic-HA, indicated papillomatosis and ectatic vessels in the papillary dermis and certain areas of the upper reticular dermis. These vessels had perivascular lymphocytic infiltrations and were full of erythrocytes. On the epidermis of the HA-appearing side of the lesion, there was a slight irregular acanthosis, while the other side resembling keratotic- HA had a slight hyperkeratosis (Figure 2D). Immunohistochemical staining of this area revealed presence of CD34 (Figure 2E). The third specimen, which examined the punctate macules that clinically resembled AS, showed a slight irregular acanthosis and slight focal hyperkeratosis in the epidermis, as well as ectatic vessels in the papillary dermis and upper reticular dermis (Figure 2F). Immunohistochemical staining of the endothelial cells revealed a diffuse reticular pattern of CD34 (Figure 2G) and limited presence of CD31 (Figure 2H) and D2-40 (Figure 2I). With the histopathological findings, the diagnoses of the lesions were made as LC, LK, and VH. Ablation of lesions using an erbium- doped yttrium aluminum garnet laser (Er:YAG) laser was planned. The patient, however, opted not to undergo invasive treatment, and thus a topical treatment regimen using imiquimod 5% was prescribed (application 3x/day). Unfortunately, due to lack of tolerance to the treatment (the patient experienced severe irritation) and with no improvement in the lesions, imiquimod therapy was discontinued after three months and the patient was lost to follow-up. DISCUSSION Diagnosis of intertwined lesions, such as those presented in our case, can be challenging to the clinician. Current diagnostic criteria might not be helpful when dealing with a legion of unfamiliar etiology or unusual appearance, location, or histopathological results. The first type of lesions found on our patient clinically appeared to be LA and LK. Lymphangioma is a rare and benign proliferation of the lymphatic system. 4,10 There are three known types of LA: circumscriptum (or capillary), cavernous, and cystic. 10 LC is characterized by clustered (localized) or diffused (classical) translucent vesicles that measure 1 to 5mm in diameter and are filled with clear lymphatic fluid. Congenital, or primary, forms are usually present at birth and represent the malformations of deep dermal and subcutaneous lymphatics with a secondary dilatation of superficial lymphatics. 11 The localized forms occur less frequently and appear as small, discrete lesions that can appear anywhere on the body at any age. 4 The lymphatic lesions present on our patient were congenital and localized and did not show any subcutaneous involvement. Some of the lesions were hyperkeratotic or verrucous in appearance. 4,10 We hypothesized that the appearance of the lesions could have been due to the tissue organization, epithelial changes, and/or hyperkeratosis. 4,12 The main histopathological finding was ectatic lymphatics in the papillary dermis. Scattered lymphocytes in the stroma were also seen. The lymphatic endothelial cells were positive for D2-40. 11 D2- 40 is a sensitive and relatively specific marker for the lymphatic endothelium but is not expressed in endothelial cells of the blood vessels. Though CD34 are occasionally present in lymphatics, according to Pusztaszeri et al, 13 CD34 is not usually present in lymphatic vessels. However, FIGURE 1. Clinical appearance of the lesions—distant (A) and closer (B, C) views

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