Dermatology Online Journal

A young woman with multiple cutaneous epithelioid angiomatous nodules (CEAN) on her forearm: A case report and follow-up of therapeutic intervention
Ladan Dastgheib¹ MD, Fatemeh Sari Aslani² MD, Mozhdeh Sepaskhah¹ MD, Nasrin Saki^1,3,* MD, Dorna Motevalli³
Dermatology Online Journal 19 (3): 1

1. Dermatology Department and Shiraz Skin Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
2. Department of pathology, Shiraz University of Medical Sciences, Shiraz, Iran
3. Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran
* Corresponding author

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Abstract

Cutaneous epithelioid angiomatous nodule (CEAN) is regarded as a very rare entity of benign vascular hyperplasia that clinically manifests as mainly single, small, rapidly-growing reddish to bluish nodules or papules, mainly on the head and neck of adults. Most patients present with solitary lesions. We present a 39-year-old female with multiple, rapidly-growing, erythematous nodules on her left forearm during a one-year period. The overall histologic picture suggested Cutaneous Epithelioid Angiomatous Nodule (CEAN). In this case we tried different treatments and compared the results.

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Introduction

Figure 1	Figure 2
Figure 1. Multiple, rapidly-growing, erythematous nodules on left forearm Figure 2. Well-circumscribed elevated dermal vascular nodule with intact epidermis. (x40)

Our patient had no history of trauma, infection, or previous skin problems. She denied any travel to other cities or countries during the 3 years before onset of the lesions. There was no family history. Drug history was negative. She was an otherwise healthy woman. Her problem started one year prior to presentation with the development of a single purple red papule on her forearm that rapidly enlarged. Increasing numbers of similar papules developed during a 1-year period to involve her entire left forearm. There was a history of oozing of serosanguinous or bloody discharge from the lesions. Pruritus was also present. Upon dermatologic physical examination the papules and nodules were purple red with a vascular hue, clustered, with central crust on top of some papules. General physical examination was normal. There was no lymphadenopathy. Excisional biopsy was done from one of the nodules to rule out Kaposi sarcoma, angiosarcoma, or angiokeratoma.

Figure 3	Figure 4
Figure 3. Varying sized channels filled by red blood cells lined by large epithelioid endothelial cells. Some large epithelioid cells with prominent nucleoli are seen. (x250) Figure 4. Varying sized channels filled by red blood cells lined by large epithelioid endothelial cells. Some large epithelioid cells with prominent nucleoli are seen. (x400)

Microscopic examination (H&E) showed a well circumscribed superficial dermal unilobular nodule with intact overlying epidermis. There was proliferation of thin walled vascular channels lined by epithelioid cells and solid proliferation of large polygonal epithelioid cells with vesicular nuclei and conspicuous nucleoli. Cytoplasm was abundant and contained intracytoplasmic vacuoles. Some normal mitotic figures were present. There was mild perivascular mostly lymphocyte infiltrate admixed with some eosinophils at the periphery. No lymphoid aggregate was seen (Figures 2, 3, 4, and 5).

Figure 5	Figure 6
Figure 5. Perivascular mild infiltration of lymphocytes and eosinophils. (x250) Figure 6. CD34 staining high lights vascular channels. Cell population between vascular channels were negative. (x100)

In the immunohistochemistry microscopic examination, CD34 staining highlighted vascular channels. The cell population between the vascular channels did not exhibit staining. Factor VIII related antigen staining was strongly positive in the endothelial cells in the lining of vascular channels. Some of the proliferating epithelioid cells showed week staining (Figures 6 and 7). A&B Immununohistochemical staining for smooth muscle actin (SMA) was done. Endothelial cells lining the channels as well as some of the intervening epithelioid cells were stained. The overall histologic picture was consistent with CEAN (Figure 8).

Figure 7	Figure 8
Figure 7. Factor VII related antigen staining shows strongly positive endothelial cells lining vascular channels. Some of proliferating epithelioid cells show week staining. (x250) Figure 8. Immununohistochemical staining for smooth muscle actin (SMA). Endothelial cells lining the channels as well as some of the intervening epithelioid cells are stained. (x400)

Discussion

Cutaneous epithelioid angiomatous nodule (CEAN) is regarded as a very rare entity of benign vascular hyperplasia that was first described by Brenn and Fletcher in 2004 in 15 patients [1]. Since then there are few case reports and case series in the literature.

CEAN clinically manifests as mainly single, small, rapidly-growing reddish to bluish nodules or papules, mainly on the head and neck of adults with no sex predilection [1, 2, 3].

Histopathologically, the lesions are characterized by a well circumscribed, unilobular superficial nodule with proliferation of large epithelioid endothelial cells in the dermis or submucosa and a background of eosinophils, lymphocytes, and plasma cells. These lesions are not surrounded by a capsule or pseudocapsule. The cytoplasm of these epithelioid cells is pinkish and abundant containing prominent vesicular nuclei and intracytoplasmic vacuoles. Mitotic figures may be seen in some cases but atypical mitoses or nuclear atypia is always absent. [1, 4]. The epithelioid cells are positive for factor VIII related antigen, CD31, CD34, D2-40, AM and in some reports the cells were positive for estrogen receptor [5].

The etiology of CEAN is not clarified yet, but the benign course and fast growth represents a reactive condition [2]. No association with infection, trauma, or immune suppression has been reported. Differentiation of CEAN from malignant epithelioid vacular lesions such as epithelioid angiosarcoma and epithelioid hemangioendothelioma is important. CEAN is not metastatic. Malignant lesions are larger and less circumscribed than CEAN. Some authors postulated that CEAN is a variant of angiolyphoid hyperplasia with eosinophilia [6, 7, 8]. Some others suggest that CEAN is close to pyogenic granuloma [4]. Most authors concluded that CEAN is a distinct pathologic entity in the spectrum of epithelioid vascular proliferations [1, 2].

Association with other conditions was reported in 2 case reports; Shiomi et al. reported a case of CEAN following pre-existing capillary malformation [9]. McLemore et. al presented a case of CEAN on the chest of a patient with breast cancer for 30 years [5].

Pavlidakey PG,etal did a literature search for all reported CEAN lesions. The age range was 10-84 years with equal sex ratio. The common sites in decreasing order were head and neck, trunk, extremities, and vulva. Of the 51 patients, only 5 patients had multiple lesions. In all cases, excision was curative with no recurrence [10].

Epithelioid vascular lesions including epithelioid hemangioma, angiolymphoid hyperplasia with eosinophilia, epithelioid hemangioendothelioma, bacillary angiomatosis, and epithelioid angiosarcoma are among the differential diagnosis of CEAN.

Simple surgical excision is the treatment of choice for single CEAN that had been suggested by Benn and Fletcher [1].

Figure 9
Figure 9. Response to treatment A-intralesional steroid and cryotherapy B-cryotherapy C-scar (site of excisional biopsy)

To our knowledge there are only six cases of multiple cutaneous epithelioid angiomatous nodule reported in the literature and none of them had as extensive involvement as our case [10, 11]. The presented case has involvement of an extremitiy, which is a less common site for CEAN than the head and neck area. Because almost all the reported cases have been single lesions, excisional surgery was the only reported treatment; unfortunately no clear documented therapies other than surgery are reported in the literature.

Alternative therapy is more important for multiple lesions; surgical excision may not be feasible. In our case we tried different treatments and compared the results. We started with confirmed therapies for aniolymphoid hyperplasia with eosinophilia such as topical and intralesional steroid, cryotherapy, and surgery. Among these, topical and intralesional steroid were ineffective. One of the lesions was treated with 2 injections of intralesional triamcinolone (20 mg/ml) 1 month apart with no improvement. After 2 months of topical mometasone under occlusion no improvement was seen. Cryotherapy was attempted on some lesions with acceptable results and no recurrence after 2 sessions of cryotherapy using cryo-spray with 45s and one freeze-thaw cycle. There was no recurrence after 3 months follow up. One of the lesions was completely excised and no recurrence was seen in 3 months follow up (Figure 9). Therapeutic results of cryotherapy alone and cryotherapy combined with intralesional steroid injection where the same.

References

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