A Greyish-Black Nodule on the Abdominal Skin - Uncommon Morphology and Location of Epithelioid Hemangioma.

Sir,

A 62-year-old woman presented to one of us (SV) with a solitary grayish black oval plaque with a surrounding brownish halo on the abdomen for 3 years. It gradually increased in size to 2 cm across at presentation [Figure 1a]. It was rough-surfaced, asymptomatic, and with a firm to hard consistency. There was no history of bleeding on trauma. She had no other comorbidities except ulcerative colitis, for which she was prescribed salazopyrine. Her hematological, biochemical, and radiological profile, including levels of eosinophils, IgE, and abdominal ultrasound, were normal.

Figure 1

a: Greyish black keratotic oval plaque on the abdomen. b: Dermatoscopy of the plaque. Blue arrow showing white reticular lines, yellow star showing red lacunae, red arrow showing delicate reticular pigmentary lines (Polarized View Dermlite DL3N, 3Gen, USA. 10×). c: Hyperplastic epidermis with irregular elongation of rete ridges. Upper dermis shows clustering of blood vessels with thickened walls lined by epithelioid endothelial cells. Low power 100×. d: Thickened walls with Plump epithelioid endothelial cells lining the lumina of thick-walled vessels. Perivascular lymphocytes and eosinophils also spilling into the interstitium. High power 400×. The stain is Hematoxylin& Eosin. (H&E)

Dermoscopic examination showed white reticular lines (suggestive of hyperkeratosis) and red lacunae (suggestive of dilated vascular channels) with a delicate reticular pigmentary network at the periphery [Figure 1b]. Punch biopsy showed hyperplastic epidermis with upper dermis showing clustering of variably sized elongated blood vessels with thickened walls lined by plump endothelial cells [Figure 1c, d]. Additionally, there was perivascular and interstitial infiltrate of lymphocytes and eosinophils. Immunohistochemical study with positive CD31 and CD34 [Figure 2a] markers confirmed the vascular nature of the tumor. Final diagnosis ruled in the favor of epithelioid hemangioma (EH). The lesion regressed significantly when treated with clobetasol propionate plus 3% salicylic acid ointment and timolol drops for approximately 6 months.

Figure 2

a: Significant regression of the plaque after 6 months treatment

Epithelioid hemangioma (EH) and angiolymphoid hyperplasia with eosinophilia (ALHE) are uncommon benign vasoproliferative disorders of unknown etiology, and both terms are often used interchangeably. It is commonly seen in individuals aged 20–50 years, with a mean age of onset of 30–34 years.[12] The onset at the age of 60 years, as in our patient, is uncommon. Lesions typically are dome-shaped, erythematous, red-brown, violaceous, variably sized papules, plaques, or nodules with a smooth surface.[123456] Our case presented as an unusual hard grayish-black nodule with a verrucous surface instead. Head and face region, including scalp, forehead, ear, and periauricular region, are favored locations.[235] EH is rarely reported on the trunk as seen in this case.[23456] Twenty percent of patients may have peripheral blood eosinophilia without raised IgE and some may show regional lymphadenopathy. Thick-walled blood vessels, lined by large, plump endothelial cells with ovoid nuclei (called epithelioid, histiocytoid, or hobnail cells) projecting into the lumen, with intracytoplasmic vacuoles is a characteristic feature of EH.[234] Another typical feature is the presence of perivascular and interstitial infiltrates containing eosinophils and lymphocytes with 5%–15% of the infiltrate formed by the former.[2347] Kimura's disease, the closest differential diagnosis of EH, was ruled out by the absence of a soft tissue mass, absent lymphadenopathy on ultrasonography, no peripheral blood eosinophilia, normal serum immunoglobulin E, and absence of lymphoid follicles on histopathology.[237] Other differential diagnoses, namely lobular capillary hemangioma, hemangioendothelioma, angiosarcoma, Kaposi's sarcoma, and cutaneous metastasis, were ruled out by histopathology.[237]

EH grows slowly and persists indefinitely, though spontaneous resolution has been uncommonly reported.[237] Surgical excision has a recurrence rate of approximately 40% as incomplete excision is common.[234] Other modalities such as electrosurgery, cryotherapy, ablative laser surgery, photodynamic therapy, systemic, intralesional or topical corticosteroids, imiquimod, topical tacrolimus, and timolol have been used with inconsistent outcomes.[14]

In conclusion, we describe a grayish-black hyperkeratotic variant of EH on the abdomen for its unique clinical and dermoscopic appearance and the rarely described location. The beneficial outcome of treatment with the steroid salicylic acid cream along with timolol drops should be evaluated further.

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