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A Recurrent Case of Targetoid Hemosiderotic Hemangioma: a Case Report and a Comprehensive Review of the Literature

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A Recurrent Case of Targetoid Hemosiderotic Hemangioma: a Case Report and a Comprehensive Review of the Literature Acta Dermatovenerol Croat 2020;28(4):228-232 CASE REPORT A Recurrent Case of Targetoid Hemosiderotic Hemangioma: A Case Report and a Comprehensive Review of the Literature Roxana-Maria Chiorean1, Alexandra Krebs1, Anas Al Kadri1, Anton Mayet2, Lorenz Becker2, Sorina Danescu3, Rodica Cosgarea3, Erik Senger1 1Private Practice of Dermatology “Dr med Erik Senger”, Seligenstadt, Germany; 2Private Practice of Dermatopathology “Dr Becker and Mayet”, Frankfurt, Germany 3Department of Dermatology, University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, Romania Corresponding author: ABSTRACT Targetoid hemosiderotic hemangioma is an acquired vascular malfor- Roxana Chiorean, MD mation of unknown origin. We report the case of a 31-year-old man with a recur- rent and spontaneous regressive targetoid hemosiderotic hemangioma. Diagnosis Frankfurter Str 90 relied on clinical and histological findings. Physical examination revealed presence PLZ 63110, Rodgau of an approximately 2 cm targetoid lesion located on the left arm, and associat- Germany ed with pain after pressure. No trigger agent (trauma, insect sting) was reported. Dermoscopy showed a group of red lacunae centrally, encircled by an intermedi- [email protected] ate yellow circular homogenous area and a red violaceous homogenous ring in the periphery. The histopathological examination and the immunohistochemical Received: March 6, 2019 staining of the lesion were characteristic for a hemangioma-like proliferation of vessels in the upper part of the dermis, similar to a targetoid hemosiderotic an- Accepted: November 16, 2020 gioma. We also review epidemiological, clinical, and histopathological findings in 6 similar cases presented in the literature. Spontaneous regression and recurrence have rarely been described in this type of skin lesion. KEY WORDS: targetoid hemosiderotic hemangioma, recurrent, regressive INTRODUCTION Targetoid hemosiderotic hemangioma (hobnail structures. The obstruction of some lymphatic vessels hemangioma) is a rare benign vascular tumor de- could induce inflammation, fibrosis, and interstitial scribed for the first time in 1988. The female-to-male hemosiderin deposits (2). The targetoid appearance ratio reported in the literature is 1:1, with an age of could be explained by hemorrhage from the vascular onset ranging from 5 to 67 years (1). proliferation, causing a purpuric ring with hemosid- The etiology of this disease is incompletely elu- erin deposits. cidated. A commonly accepted theory is that it may The diagnosis relies on clinical and histological occur on a pre-existing hemangioma, after a trauma. findings. Clinically, hemosiderotic hemangioma typi- Trauma could induce the formation of thrombi and cally appears as a solitary small targetoid lesion with microshunts, with pressure from the capillaries caus- a central red/ purple and/or brown papule, encircled ing the filling of the lymph spaces with erythrocytes, by a pale area and peripheral ecchymotic ring. It is contributing to the formation of aneurysmal micro- usually located on the limbs and trunk, but unusual 228 ACTA DERMATOVENEROLOGICA CROATICA Chiorean et al. Acta Dermatovenerol Croat Targetoid hemosiderotic hemangioma 2020;28(4):228-232 presentation on the scalp has also been described (3). diagnosis includes patch stage and lymphangioma- Clinical differential diagnosis includes Kaposi’s sarco- like variants of Kaposi’s sarcoma, well-differentiated ma, hemangioma, dermatofibroma, melanocytic ne- angiosarcoma, retiform hemangioendothelioma, vus, insect bite reaction, and solitary angiokeratoma Dabska tumor, and benign lymphangiomas. In con- (4). trast to Kaposi sarcoma, targetoid hemosiderotic Dermoscopy most frequently shows centrally lo- angioma does not stain positively for human herpes- cated red lacunae and a homogenous red-violaceous virus 8 (6). area in the periphery. Some lesions may also present The evolution of this benign tumor is different. It is a delicate pigmented network in the periphery (5). permanent in most of the cases, and it is generally ac- Histologically, hemosiderotic hemangioma pres- cepted that it does not resolve itself. An excision may ents a non-circumscribed dermal vascular prolifera- be indicated, if the lesion is cosmetically disturbing tion with thin-walled dilated vessels in the papillary (7). To our best knowledge, there are only a few case dermis continued with narrower, slit-like endothelial reports of targetoid hemosiderotic hemangioma with lined spaces in the deep reticular dermis, dissect- recurrent evolution in the literature (4,7,8). ing between dermal collagen bundles. Extravasated erythrocytes and hemosiderin deposits can be ob- CASE REPORT served in the dermis. An inflammatory lymphocytic A 31-year-old male patient from an urban area interstitial or perivascular infiltrate is often seen. The presented in our department in August 2016, with a lymphatic origin of targetoid hemosiderotic angio- 1-week history of spontaneous occurrence of a targe- mas has been confirmed by recent studies, which toid lesion, located on the left arm (Figure 1, A). He demonstrated the positive immunohistochemical also reported pain on pressure. The patient reported staining of these lesions for CD31, vascular endothe- that the lesion had occurred one year before, approx- lial growth factor receptor-3 (VEGFR-3), the lymphat- imately 2-3 times, in the same place. The lesion had ic specific antipodoplanin monoclonal antibodies an approximate duration of 10 days and then disap- (D2-40), and also a lack of CD34 staining. The absent peared completely between cycles. The skin in the or minimal proliferative nature of this vascular lesion region looked normal, without evidence of a papule, has been suggested by a consistent very low Ki-67 postinflammatory hyperpigmentation, or scarring. proliferation index. The histopathologic differential Personal medical history was not significant. Figure 1. Clinical, dermoscopy, and histopathological features of our patient at diagnosis. (A) Clinical aspect at diagnosis. (B) Dermoscopy at diagnosis. (C) Clinical aspect after regression. (D) Histopathological aspect, low magnification. € Histopatho- logical aspect, high magnification. Hobnail endothelial spaces in the center of the lesion. ACTA DERMATOVENEROLOGICA CROATICA 229 Chiorean et al. Acta Dermatovenerol Croat Targetoid hemosiderotic hemangioma 2020;28(4):228-232 Physical examination revealed the presence of an Excision is recommended in case of esthetic issues. approximately 2 cm targetoid lesion with a central Spontaneous total regression and cyclic changes, red papule, encircled by a pale area and a peripheral such as swelling and intermittent changes in lesion ecchymotic ring, located on the left arm. Dermosco- size and rim without complete regression, have been py showed a group of red lacunae centrally, encircled reported in association with menstruation (4) or in by an intermediate yellow circular homogenous area children (6). and a red violaceous homogenous ring in the periph- Regression represents a common feature in infan- ery (Figure 1, B). tile capillary hemangiomas. This event is supposed to Histopathologic examination was performed on be caused by apoptosis, as a higher number of apop- two samples. The histopathological examination of totic cells was found in these hemangiomas, com- the sample from the middle of the lesion showed a pared to lobular capillary hemangioma. Regression slightly irritated epidermis. In the dermis, we observed was also reported in tufted angiomas (9). slit-like proliferations of vessels, with prominent endo- We report the case of a 31-year-old man with a re- thelium in some places, erythrocytes, a sparse inflam- current targetoid hemosiderotic angioma, appearing matory infiltrate, and few plasma cells (Figure 1, D, and disappearing in the same place without leaving E). The histopathological examination of the sample any scars. The patient reported 2-3 similar episodes in from the margin showed a very sparse finding, with the past year. No trigger agent (trauma, insect sting) few erythrocytes and few prominent vessels. The im- was reported. munohistochemical staining of the sample from the To our best knowledge, there are only 6 cases of middle showed positive staining of the vessels from recurrent targetoid hemosiderotic hemangioma re- the dermis for CD31, positive staining of some ves- ported in the literature (see Table 1). The age of the sels for D2-40 (podoplanin), and negative staining for patients ranged between 9 and 40 years. Trauma (bee HHV8. Actin (alpha smooth muscle) staining showed sting) was incriminated as a potential trigger in only muscular differentiated cells around the vessels. MIB1 one patient (case 5) (Table 1). Occurrence at men- staining showed no obviously proliferating endothe- struation was mentioned in the same patient (case 5) lium. Therefore, the lesion was evaluated as a heman- (Table 1). The lesions were situated on the trunk in 2 gioma-like proliferation of vessels in the upper part patients (case 2 and 5), upper limbs in 3 patients (case of the dermis. The histology was similar to a targetoid 1, 3, and 6) and lower limbs in 1 patient (case 4). Clini- hemosiderotic angioma. cally, all lesions presented a central papule with an No local or systemic treatment was administered. ecchymotic ring in the periphery in 3 patients (case 1, The hemangioma completely disappeared without 4, 5), an erythematous ring in 1 patient (case 2), and leaving any scar a few days after the punch biopsy a
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