Xanthelasma manifests as a yellowish-tan soft plaque occurs in the inner canthus in middle-aged individuals (Fig. 9.2). Large nodular xanthelasma is called xanthoma or tuberous xanthoma, which has a known association with Erdheim–Chester disease. Acute-onset eruptive xanthoma occurs in patients experiencing a rapid rise in serum triglyceride levels. Management should include systemic evaluation for the causative etiology and excision or laser [9] or radiofrequency-assisted vaporization of large cosmetically unacceptable lesions [10].

Microscopically, xanthelasma consists of lipid-laden macrophages in the superficial dermis, around blood vessels, and adnexa.

Juvenile xanthogranuloma of the eyelid may be a part of systemic affection seen as multiple fleshy superficial eyelid nodules with or without coexisting conjunctival, iris, and orbital involvement. The adult variant may be diffuse and associated with bronchial asthma (Fig. 9.3) [11]. Juvenile xanthogranuloma is known to spontaneously involute, thus qualifying for observation. Systemic corticosteroids are indicated in recalcitrant juvenile xanthogranuloma and as primary therapy for the adult variant [11]. Extensive, cosmetically disfiguring, and steroid-resistant lesions could be excised or treated with systemic chemotherapy or radiotherapy.

Xanthogranuloma consists of monomorphic infiltrate of histiocytes, intermixed with lymphocytes and the classic Touton giant cell with a wreath-like arrangement of nuclei and peripheral clear zone.

Malignant fibrous histiocytoma presents as a firm subcutaneous mass [13]. Dermatofibrosarcoma protuberans, which manifests as a rapidly growing eyelid nodule, is considered an aggressive form of malignant fibrous histiocytoma [14]. It has tendency for local invasion and is known to metastasize. The mainstay of treatment is complete surgical excision with wide margins. Because of risk of recurrence following excision, consideration should be given to histologic margin control and adjuvant radiotherapy [15].

Malignant fibrous histiocytoma differs from benign variant in exhibiting marked nuclear pleomorphism, high mitotic activity, pericytoma-like areas with foci of xanthoma cells, and multinucleated giant cells.

Pyogenic granuloma occurs anywhere in the eyelid, as a rapidly growing, pedunculated reddish-pink mass with or without superficial ulceration and may easily bleed on touch (Fig. 9.4). It usually follows trauma or surgery. Local excision of the lesion is curative [23].

Pyogenic granuloma consists of exuberant mass of proliferating radiating capillaries and edematous stroma with mixed inflammatory infiltrates. Intravascular papillary endothelial hyperplasia is a rare form of “pyogenic granuloma” in which the angiomatous proliferation is confined entirely within the lumen of a vessel [24].

Congenital capillary hemangioma usually manifests at birth or within the first months of life. There are two distinct clinical variants – superficial and deep. The superficial variant, better known as strawberry hemangioma, appears as a bright red to deep purple, lobulated, spongy, soft eyelid mass that typically blanches on application of direct pressure and engorges when the infant cries or strains. Contiguous conjunctival and orbital extension is known to occur. The superficial variant is localized to the epidermis and dermis, whereas the deep variant lies in the subcutaneous tissue and is bluish or blue gray in color.

Congenital capillary hemangioma grows rapidly in size and reaches its final size by 6–12 months of age. It then becomes stable and slowly involutes by 4–7 years of age [27]. About 70 % regress by the age of 7 years [27].

Histologically, capillary hemangioma of the eyelid consists of lobules of capillaries separated by sparse fibrous septae. The morphology of the lesion changes with age. An early immature lesion tends to have obliterated lumen with plump endothelial cells and occasional mitotic figures (Fig. 9.6), while in later stages the lumina increases and the endothelial cells get attenuated, with increasing fibrosis and fat infiltration.

In most instances, congenital capillary hemangioma is a sporadic condition but approximately 20 % of patients may manifest it as multiple tumors involving the cutaneous tissue elsewhere, central nervous system, liver, and gastrointestinal tract [25]. Systemic lesions, especially those found in association with Kasabach–Merritt syndrome, could aggressively proliferate and lead to hemorrhage, platelet consumption, disseminated intravascular coagulation, cardiac failure, and death [25].

The main ocular complications are amblyopia and strabismus. Amblyopia could be meridional because of induced astigmatism or because of stimulation deprivation secondary to mechanical ptosis. Because most lesions spontaneously regress, observation, refractive correction, and appropriate amblyopia management are the standard treatment. Active intervention is indicated if the lesion extensively involves the face or is ulcerated with episodes of bleeding and if there is mechanical ptosis with obscuration of pupillary axis or induced astigmatism with amblyopia. Extensive lesions are treated with oral prednisolone 1–2 mg/kg body weight tapered over 4–6 weeks. Application of topical clobetasol propionate may help [28]. Intralesional steroid injection (Fig. 9.5) is mainly reserved for eyelid and anterior orbital lesions. Most lesions regress after 1–3 injections of triamcinolone or a combination of dexamethasone and triamcinolone injected at 6–8 weekly intervals [29]. The recommended dosage per injection is 6 mg/kg body weight equivalent of prednisolone. Although uncommon, reported complications of intralesional steroid injection include central retinal artery occlusion, eyelid depigmentation, fat atrophy, eyelid necrosis, and adrenal suppression [29]. Alternative treatment modalities include interferon, laser sclerotherapy, and excision of circumscribed anterior lesions (Fig. 9.6).

In recent years several centers have reported the use of systemic β-blockers for the treatment of infantile hemangioma, with very promising results (Fig. 9.7) [30–32]. The dose that has been used is 0.5–2.0 mg/kg/day for several months, with significant reduction in the size of the hemangioma in all babies. Side effects such as bradycardia, hypotension, bronchospasm, hypoglycemia, and electrolyte disturbances have been reported, leading to a reduction in the dose of the drug. Topical timolol maleate 0.5 % was also found to be effective [33].

The lesions are ill circumscribed and bluish in color and may undergo slow progression. Epithelioid hemangioma also known as angiolymphoid hyperplasia with eosinophilia occurs as a nodular lesion in the eyelid. Kimura disease, which is predominant in Asian population, shares both clinical and histopathologic features with angiolymphoid hyperplasia with eosinophilia and may be clinically indistinguishable [36].

Cavernous hemangioma is composed of large dilated vascular channels filled with blood, hemosiderin-laden macrophages, scattered lymphoplasmacytic infiltrates, and secondary changes such as calcification, phlebolith, and fibrosis.