Pediatric Dermatology Vol. 31 No. 3 281–285, 2014

Superficial Hemosiderotic Lymphovascular Malformation (Hobnail Hemangioma): A Report of Six Cases Joel C. Joyce, M.D.,* Phillip J. Keith, M.D.,† Sara Szabo, M.D., Ph.D.,‡ and Kristen E. Holland, M.D.* *Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin, †Department of Dermatology, Mayo Clinic, Rochester, Minnesota, ‡Department of Pathology and Laboratory Medicine, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin

Abstract: Hobnail hemangioma (HH), initially termed targetoid hemosiderotic hemangioma, is a rare, often solitary lesion classically characterized by a central brown or violaceous papulonodule surrounded at times by an ecchymotic halo. This lesion is typically found on the trunk or limbs of children or young to middle-aged adults. Numerous case reports have found HHs to have a reproducible histologic appearance. Although the exact histogenesis of these lesions is unknown, multiple recent immunohistochemical studies suggest a lymphatic origin of these lesions. We present six cases of children with HHs with classic histology but with variability in their clinical appearance. Because the clinical presence of a targetoid halo is inconsistent and the hobnail phenomenon is not specific, we favor the designation of superficial hemosiderotic lymphovascular malformation instead of HH or targetoid hemosiderotic hemangioma as a more unifying term for this rare clinical entity. By eliminating confounding terminologies (in this case, incorporation of “hemangioma” in the name of this entity), we also hope to encourage a swifter change in practice to move away from erroneous diagnostic considerations.

In 1988 Santa Cruz and Aronberg (1) described a vascular lesion appearing clinically as a violaceous papulonodule with a peripheral targetoid halo, for which they coined the term targetoid hemosiderotic hemangioma (THH). THH was described in the original case report and by subsequent authors as

having a histologic pattern of dilated ectatic vascular channels in the superficial dermis lined with a single layer of hobnail epithelial cells, often with intraluminal papillary projections, fibrin thrombi, and extravasated erythrocytes with hemosiderin deposition. Associated slit-like or angular vascular spaces

Address correspondence to Joel C. Joyce, M.D., Department of Dermatology, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, or e-mail: [email protected]. DOI: 10.1111/pde.12294

© 2014 Wiley Periodicals, Inc.

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resembling lymphatic-like lumina extend through the collagen of the reticular dermis (and occasionally into subcutaneous tissue) (1,2). In 1999 Guillou et al (3) proposed the histologic descriptive term “hobnail hemangioma” (HH) in stead of THH. Although there has been debate over the histogenesis of the vascular endothelium of HH, Mentzel et al (2) first suggested a lymphatic origin of HH with the demonstration of positive CD31 staining in HH with concurrent CD34 negativity. Subsequent reports (4–6) have demonstrated immunohistochemical staining of lesional vessels of HH with the D2-40 antibody (against podoplanin) (7), which is clinically used to differentiate lymphatic endothelium from blood vascular endothelium and thus in general to assign lymphatic derivation and phenotype to vessels. In 2012 Trinidade et al (5) coined the term “superficial hemosiderotic lymphatic malformation” as the most representative name of the vascular lesion variably called THH and HH. Henceforth we will use the nearly identical term “superficial hemosiderotic lymphovascular malformation” (SHLM). There are multiple case series (1–5,8) outlining the clinical and histopathologic features of SHLM in the adult population, but knowledge surrounding the pediatric presentation is limited to case reports (8–12) and one case series (6). We present a series of six pediatric patients to characterize the variable clinical features found in the setting of a consistent histologic diagnosis of SHLM to allow for better diagnosis and understanding of these lesions in children. METHODS Institutional review board approval was obtained for a retrospective chart review of children diagnosed with HH at Children’s Hospital of Wisconsin (CHW). Patients were identified by searching the CHW pathology database for all patients diagnosed with HH or THH from April 2002 through February 2011. Records were reviewed noting patient demographic information, age of onset, location, symptoms, clinical history, clinical description, and pathology reports. Associated clinical images (if available) were also reviewed. RESULTS Table 1 describes the demographic and clinical characteristics of the six cases and the results of D2-40 antibody staining. The diagnosis of SHLM was confirmed in all six cases according to pathologist evaluations. There were three boys and three girls,

ranging in age from 4 to 14 years old. The lesions in our patient cohort were primarily located on the upper and lower (Fig. 1) extremities, with the exception of one SHLM, which occurred on the face (patient 2). Two (patients 2 and 3) of the six cases reported pain with minor trauma to the lesions. All patients reported a clinical history of fluctuating morphology with color and/or size changes. Colors reported ranged from “bruise-like” blue-purple discolorations to tan to red changes and yellow to green to blue color progression. Size changes included slow progressive growth, as well as a history of involution and recurrence (patient 5). Two of the six cases (patients 2 and 5) had the lesions excised without recurrence and the remainder had clinical observation. There are no reports of recurrence for any of the described cases. Histologic findings of our cases included dilated vascular spaces at varying levels of the dermis, with hemosiderin deposition and hobnailing of endothelial cell nuclei (Fig. 2A–C). On immunohistochemical stains for D2-40, all six lesions showed positivity (Fig. 2D), although with variable intensity and extent. DISCUSSION SHLMs have most commonly been reported to occur on the trunk and extremities of young to middle-aged adults, with rare occurrence elsewhere (1–3,13). Mentzel et al (2) and Guillou et al (3) have approximated the median age of patients at presentation to be 30.5–31 years. Both case series included children. Pediatric case reports have reported a range of ages at presentation, from birth to late teens (9–12). A recent pediatric case series noted an age range of presentation between 5 and 16 years (median 11 years) (6). Our cohort had a median age at presentation of 9.75 years. Several case reports have reported congenital lesions (9,12,14). None of our patients reported congenital lesions, but one reported onset at 14 months. The lesions in our patient cohort were primarily located on the upper and lower extremities, which is consistent with those reported in other pediatric and adult cases, with the exception of patient 2 (central forehead), although a facial HH has been reported in another pediatric case (14). Two of our patients had clinical histories significant for pain or tenderness with minimal trauma to the lesion. Published reports in the literature note variability of symptomatic and asymptomatic lesions (6,8,10,11). All of our patients reported clinical histories of variably changing lesions in terms of color and/or size (Table 1). Although

Joyce et al: Reclassification of Hobnail Hemangiomas

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TABLE 1. Demographic and Clinical Characteristics of Reported Cases Patient

Age, years

Sex

Lesion duration

D2-40 positive

Location

Symptoms

Clinical history

Clinical description

Blue-purple spot that initially resembled a bruise, with color changes to pink to light brown every few weeks or months without a change in size Variation in color (yellow to green to blue) that occasionally swells without an overall change in size with reported intervals of involution

8-mm 9 10-mm slightly raised, flat-topped, well-circumscribed, light brown indurated plaque

Yes

13-mm 9 15-mm ecchymotic, wellcircumscribed, brownred nodular plaque with slight green rim; central firmness and soft compressible margins; slightly mobile 10-mm 9 6-mm redbrown plaque with dark brown-black scattered crusted vesiculopapules 4-mm 9 5-mm redbrown papule

Yes

1

8.5

Male

8 months

Left anterior shin

Asymptomatic

2

4

Female

2 years

Central forehead

Painful and tender

3

14

Female

6 years

Left anterior shin

Tender with one bleeding episode

Slow progressive growth

4

7

Male

4 years

Left medial thigh

Asymptomatic

5

12

Male

Unknown (longstanding)

Left forearm

Asymptomatic

Thought to be a slowly growing nevus with color changes including tan to red to purple History of involution with recurrence

6

11

Female

14–16 months

Right posterior elbow

Asymptomatic

Figure 1. Fourteen-year-old girl (patient 3) with 10-mm 9 6-mm tender red-brown plaque on her left shin with focal brown-black crusted papules within (enlarged view, lower right).

some changes may be secondary to trauma, this common history was not elicited in all cases. Patient 5 reported occasional complete involution, a phenomenon

Fading of coloration over the last 6 months

2-mm red vascular papule with surrounding browngray macule 11-mm 9 10-mm illdefined brown to faint purple-red papule

Yes

Yes

Yes

Yes

that has previously been reported in the literature (11). SHLMs are dynamic lesions whose variable appearance makes a clinical diagnosis challenging. The peripheral halo of discoloration is consistent with dermal hemosiderin deposition in association with abnormal vessels. This has been variably interpreted in the published literature as an effect of trauma on a preexisting “hemangioma” (9) or as hemosiderin deposition independent of a preexisting “hemangioma” (15). It is more likely though that in the vicinity of these abnormal vessels of lymphatic phenotype there is erythrocyte microshunting or leakage, with a variably aggravating role of superimposed trauma (2,4,5), which may be subclinical on occasion. At clinical presentation, none of our patients had a peripheral halo surrounding their primary lesion. Santa Cruz and Aronberg (1) originally reported that the central papulonodule will persist and the peripheral ecchymotic ring will expand before resolving spontaneously as the hemosiderin deposits are reab-

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A

B

D

C

Figure 2. Histopathology of cases. (A) Patient 4. Dilated lesional vascular channels in the superficial dermis (hematoxylin and eosin [H&E] 109) and (B) a higher-power view of the endothelial lining with hobnailing of nuclei protruding button-like into the vascular space (H&E, 409). (C) Patient 5. Hemosiderin deposition in the middermis (H&E, 409). (D) Patient 4. Endothelial lining with positive immunohistochemical stain (D2-40, 409).

sorbed, but multiple subsequent reports have noted the heterogeneity of clinical appearance not solely related to lesional age and a reproducible progression of clinical stage (2,5). It is possible that our patients and perhaps some of those reported in the literature without a peripheral halo characteristic of a THH (2,3) had a surrounding ring that resolved before the time of examination, although it is also possible that the lesions of our patients underwent color changes without the presence of a peripheral halo. Cyclic morphologic changes of SHLM including color changes have been reported in children (11) and adults (8,15). Because of the small size and benign nature of SHLM/HH, surgical treatment is not always performed. The largest case series on SHLM/HH (2) reported 53 cases that were treated using simple local excision, without evidence of metastases or recurrence at follow-up. The recent pediatric case series by Al Dhaybi et al (6) noted that 6 of 12 cases had surgical excision without recurrence. Information regarding surgical outcomes of SHLM/HH excisions in children is scarce. Two of our six (patients 2 and 5) cases underwent surgical excision without recurrence. Our experience, as well as that of the case series above, suggests that SHLMs are amenable to primary surgical excision with a low risk of recurrence.

In their initial report of the newly described THH, Santa Cruz and Aronberg (1) postulated that the lesion may have multiple histologic components of different origin, specifically that the superficial component represented blood vessel derivatives and the deeper angular component represented lymphatic spaces, although the development of anti-podoplanin monoclonal antibodies (D2-40) specific to lymphatic endothelium (7) and their use in staining of SHLM specimens throughout the superficial and deep dermis supports a histogenesis from lymphatic endothelial cells (2) or induction of lymphatic phenotype during pathogenesis. The more consistent CD31 positivity on immunohistochemical stains, with rarer and lesser positivity for CD34, further supports these considerations of a lymphatic immunophenotype in contrast to a blood vascular phenotype. These clinically benign lesions must be differentiated from malignancies such as patch-stage Kaposi’s sarcoma, low-grade angiosarcoma, retiform hemangioendothelioma, epithelioid hemangioma, melanoma, and progressive lymphangioma because of their similar clinical features, and immunohistochemical staining with D2-40 may be contributory. On a small biopsy specimen or on specimens of secondarily altered lesions, a socalled progressive lymphangioma may still prove to be a challenge (2,3,5), because both are malformations in their nature rather than neoplastic proliferations.

Joyce et al: Reclassification of Hobnail Hemangiomas

The variable clinical presentation of SHLMs presents a diagnostic challenge to both pediatric and adult dermatologists. Clinicians must be aware that while a peripheral halo may aid in the diagnosis of these lesions, it is not a reliable diagnostic feature as it is often absent. Furthermore, while hobnailed endothelial cells are often found histologically, they can be present in other vascular lesions. Given the variable clinical presentation and newer information relevant to their immunohistochemical lineage and pathogenesis, we reiterate favoring the term SHLM as proposed by Trindade et al (5), believing this to be a more accurate description of the entity, and would discourage continued use of the historical nomenclature (THH and HH). In light of the inconsistent clinical morphology of these lesions and the changing nomenclature since their original description, biopsy and histologic evaluation of lesions suspected of being SHLM can be considered when clinically indicated to make the correct diagnosis.

5.

6.

7. 8. 9. 10. 11. 12.

REFERENCES 1. Santa Cruz DJ, Aronberg J. Targetoid hemosiderotic hemangioma. J Am Acad Dermatol 1988;19:550–558. 2. Mentzel T, Partanen TA, Kutzner H. Hobnail hemangioma (“targetoid hemosiderotic hemangioma”): clinicopathologic and immunohistochemical analysis of 62 cases. J Cutan Pathol 1999;26:279–286. 3. Guillou L, Calonje E, Speight P et al. Hobnail hemangioma: a pseudomalignant vascular lesion with a reappraisal of targetoid hemosiderotic hemangioma. Am J Surg Pathol 1999;23:97–105. 4. Franke FE, Steger K, Marks A et al. Hobnail hemangiomas (targetoid hemosiderotic hemangiomas)

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are true lymphangiomas. J Cutan Pathol 2004;31:362– 367. Trindade F, Kutzner H, Tellechea O et al. Hobnail hemangioma reclassified as superficial lymphatic malformation: a study of 52 cases. J Am Acad Dermatol 2012;66:112–115. Al Dhaybi R, Lam C, Hatami A et al. Targetoid hemosiderotic hemangiomas (hobnail hemangiomas) are vascular lymphatic malformations: a study of 12 pediatric cases. J Am Acad Dermatol 2012;66:116–120. Kahn HJ, Marks A. A new monoclonal antibody, D240, for detection of lymphatic invasion in primary tumors. Lab Invest 2002;82:1255–1257. Christenson LJ, Stone MS. Trauma-induced simulator of targetoid hemosiderotic hemangioma. Am J Dermatopathol 2001;23:221–223. Bingham LG, Davis DM. Clinicopathologic challenge: targetoid hemangioma. Int J Dermatol 2008;47:991– 992. Ibrahim M, Shwayder T. Hobnail hemangioma in a nine-year-old boy: a rare case presented with dermoscopy. Dermatol Online J 2010;16:7. Tan C, Zhu W, Lai R. A recurrent case of targetoid hemosiderotic hemangioma. Acta Derm Venereol 2008;88:181–182. Yoon SY, Kwon HH, Jeon HC et al. Congenital and multiple hobnail hemangiomas. Ann Dermatol 2011;23:539–543. Morales-Callaghan AM, Martinez-Garcia G, Aragoneses-Fraile H et al. Targetoid hemosiderotic hemangioma: clinical and dermoscopical findings. J Eur Acad Dermatol Venereol 2007;21:267–269. Santonja C, Torrelo A. Hobnail hemangioma. Dermatology 1995;191:154–156. Christenson L, VanBeek M, Davis D. Targetoid hemosiderotic hemangioma occurring in a father and son. Arch Dermatol 2000;136:1571–1572.

Superficial hemosiderotic lymphovascular malformation (hobnail hemangioma): a report of six cases.

Hobnail hemangioma (HH), initially termed targetoid hemosiderotic hemangioma, is a rare, often solitary lesion classically characterized by a central ...
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