Peer Reviewed

What's Your Diagnosis?

A Girl With an Asymptomatic Mass on Her Eyelid

Volume 58 - Issue 12 - December 2018

  • Answer: Chalazion

    fig 1

    A chalazion, meaning “hailstone” in Greek, is a localized lipogranulomatous inflammation that affects the meibomian gland of the eyelid.1,2 The condition is also known as meibomian cyst. It is the most common inflammatory lesion of the eyelid and typically presents as a painless localized swelling on the eyelid.1

    INCIDENCE

    Chalazion is one of the most common eyelid disorders of all age groups.2,3 The exact incidence, however, is not known. The male to female ratio is approximately equal.4 Adio and colleagues reviewed the records of children seen over 1 year in the eye clinic of the University of Port Harcourt Teaching Hospital in Nigeria.4 A total of 846 children were seen. The male to female ratio was 1 to 1.07. Most patients were between 13 and 18 years of age. Four (0.47%) of the 846 had a diagnosed chalazion.

    ETIOPATHOGENESIS

    There are approximately 30 meibomian glands in the upper eyelid and 10 to 20 meibomian glands in the lower eyelid.5,6 Meibomian glands consist of holocrine branching tubuloalveolar modified sebaceous glands, unconnected with hair or erector pili muscle, and are arranged vertically on the posterior surface of the tarsus.5,7 A chalazion results from blockage of the meibomian gland by desquamated epithelial cells and/or lipid inspissation.5,7 Rupture of the meibomian gland with release of the inspissated lipid into the surrounding tissue elicits a granulomatous inflammatory reaction.6,7 Since meibomian glands are embedded in the tarsal plate of the eyelids, a chalazion due to blockage of the meibomian gland results in a mass on the conjunctival portion of the eyelid.8

    The etiology is multifactorial, including local and systemic inflammatory conditions. Factors predisposing to blockage of meibomian glands include poor lid hygiene, meibomian gland dysfunction, chronic blepharitis, seborrheic dermatitis, rosacea, vitamin A deficiency, Demodex brevis infestation, viral keratitis (adenovirus, Epstein-Barr virus, herpes simplex virus), pulmonary tuberculosis, hyperimmunoglobulin E syndrome, smoking, and bortezomib therapy.9-23

    HISTOPATHOLOGY

    Histopathologically, there is often a connective tissue pseudocapsule surrounding a lipogranulomatous lesion with lipid-filled, Touton-type giant cells.1,8 Characteristically, the nuclei of these cells are located around a central foamy cytoplasmic area that contains the lipid material.8

    CLINICAL MANIFESTATIONS

    Clinically, a chalazion presents with a firm, nontender, slowly growing, nodule on the upper or lower eyelid.24 The upper eyelid is more commonly involved due to the increased number and length of meibomian glands in the upper eyelid.6,25 The lesion is usually less than 1 cm in diameter.8 A chalazion may occur externally as a lump on the skin, as illustrated in the present case, or internally as a lump beneath the conjunctiva (Figure 2).24 The condition is usually solitary but can be multiple (Figure 3).

    Fig 2

    Fig 3

    A chalazion is usually painless and nonerythematous.24,26,27 A small chalazion may not be visible but is palpable as a mass or resistant area in the tarsus. Extratarsal presentation of chalazion has also been described, resulting from anterior perforation of the tarsal plate with inferior migration of meibomian gland secretions, resulting in a reddish intradermal nodule on the lower eyelid skin.28,29

    DIAGNOSIS

    The diagnosis is mainly clinical. Typically, a chalazion presents as a nontender, nonerythematous, slowly growing nodule on the eyelid with no evidence of inflammation, no punctum, and no discharge. Laboratory tests are usually not necessary.

    DIFFERENTIAL DIAGNOSIS

    Differential diagnosis includes, among others, hordeolum, cellulitis, idiopathic facial aseptic granuloma, epidermoid cyst, dermatofibroma, neurofibroma, pilomatrixoma, blue nevus, external angular dermoid, lipoma, milium, pneumo-orbita, pyogenic granuloma, seborrheic keratosis, sebaceous gland hyperplasia, amyloidosis, and neoplasms.1

    A hordeolum is an acute infection of the eyelid that is usually staphylococcal in origin. When it involves the gland of Zeis (sebaceous) or Moll (sweat), it is termed an external hordeolum, commonly referred to as a stye.30 Less commonly, the meibomian gland on the inner side of the eyelid can become infected, and it is termed an internal hordeolum. Characteristically, an external hordeolum presents as a painful, yellowish pustule in the eyelid margin, typically centered on an eyelash with surrounding erythema (Figure 4).

    fig 4

    An internal hordeolum, on the other hand, presents as a tender swelling within the tarsal plate.30 The lesion may resolve into a chalazion. A chalazion can be differentiated from a hordeolum by the absence of signs of acute inflammation. In contrast with a hordeolum, a chalazion is painless.10

    Cellulitis of the eyelid can be distinguished from a chalazion by diffuse swelling, erythema, and significant pain. The condition may be accompanied by fever and leukocytosis.

    An idiopathic facial aseptic granuloma typically presents as a painless, erythematous to violaceous, soft nodule on the eyelid or cheek.31-33 The lesion usually resolves spontaneously without scarring over several months to a year.31

    An epidermoid cyst, also known as epidermal inclusion cyst or epidermal cyst, typically presents as an asymptomatic, solitary, fluctuant to firm, dome-shaped mass that is attached to the skin but not attached to the underlying structure.34-36 A punctum is often noted.35 It has a tendency to grow slowly. Epidermoid cysts occur mainly on hair-bearing areas and often result from the trauma to the pilosebaceous unit.36 In non–hair-bearing areas, epidermoid cysts result from implantation and proliferation of squamous epithelium into the dermis secondary to trauma.36 Sites of predilection include the face, scalp, neck, and back.34-36

    A dermatofibroma is a common benign dermal lesion composed primarily of fibroblasts with excessive deposition of collagen in the dermis. The condition most frequently affects young adults and occasionally children and is more common in females than in males.37 Typically, the lesion presents as an asymptomatic red or reddish brown nodule.37,38 The lesion is attached to the skin but not to the underlying structure. Although the lesion can develop anywhere on the body, the lesion is most commonly found on the limb. The size varies from a few millimeters to 2 cm. Pinching of the lesion results in dimpling of the lesion (the dimple sign or Fitzpatrick sign).38,39 This can be attributed to tethering of the epidermis to the underlying lesion.

    A neurofibroma is a benign peripheral nerve-sheath tumor composed of an extracellular matrix and a heterogeneous mixture of Schwann cells and fibroblasts.40 Cutaneous neurofibromas often appear during puberty or pregnancy and increase in size and number with age and are found in almost all adults with neurofibromatosis type 1. These painless, flesh-colored, soft or elastic, nodular or pedunculated lesions often invaginate into the skin and exhibit the buttonhole sign when gentle digital pressure is applied to the surface. In contrast, subcutaneous neurofibromas, which are usually firm and painful, are deeply seated in the dermis and therefore are less circumscribed.40 A solitary neurofibroma on the eyelid may simulate a chalazion.41-43

    Typically, a pilomatrixoma presents as a firm to hard, solitary, painless nodule in the dermis or subcutaneous tissue covered by normal skin. The color varies from white, pink, blue, to red-blue.44 Most lesions measure 0.5 cm to 3 cm in diameter. The nodule may become hardened if the lesion is calcified. Calcification and ossification occurs in 70% to 85% and 15% to 20% of patients, respectively.45 Downward pressure directed at one end of the lesion may cause the other end to spring upward in the skin (teeter-totter sign).45 Multiple facets and angles may appear when the overlying skin is stretched (tent sign).45 An eyelid pilomatrixoma may masquerade as a chalazion.46 In one study, more than 75% of cases of eyelid pilomatrixoma occurred in patients younger than 13 years of age.47

    A common blue nevus typically presents as a solitary, smooth, well-circumscribed, dome-shaped papule or nodule that measures less than 1 cm in diameter.48,49 The lesion is homogeneously blue to blue-gray to blue-black in color and is asymptomatic. The characteristic blue color can be attributed to the deep dermal location of melanin and that shorter-wavelength blue light cannot penetrate deeply but is reflected back to the observer (Tyndall effect).48 It is commonly located on the face and dorsal surfaces of the hands and feet.49

    An external angular dermoid is a dermoid cyst located at the lateral orbital rim adjacent to the frontozygomatic suture.50 The cyst arises along bony sutures as a result of abnormal ectodermal sequestration during embryonic development. Clinically, an external angular dermoid presents as an asymptomatic, slow-growing, soft to firm, poorly mobile mass above the lateral eyebrow.25 The mass is subcutaneous, and the overlying skin is normal. The mass is usually noticed at birth or in the first year of life.

    A lipoma is a benign tumor composed of collections of mature adipocytes enclosed by a thin, fibrous capsule. Clinically, a lipoma presents as an asymptomatic, slow-growing, soft, well-circumscribed, lobulated, round or oval, dome-shaped or egg-shaped mass that is not attached to the overlying skin or underlying structures.51 The overlying skin is characteristically normal. A “slipping sign,” elicited by gently sliding the fingers off the edge of the tumor, is characteristic.51 The tumor will be felt to slip out from under. The size of a lipoma usually ranges from 2 to 5 cm in diameter. Although rare, a lipoma can occur on the eyelid.

    A milium is a benign, 1- to 2-mm, white or yellowish, dome-shaped, superficial keratinous cyst.52,53 Histologically, a milium appears as a small infundibular cyst that is lined with stratified squamous epithelium with a granular cell layer.53 The cyst contains laminated layers of keratin. The lesion is most common on the face.52

    A pneumo-orbita often results from facial trauma leading to airflow between the sinuses and the orbit.54 Clinically, a pneumo-orbita presents with an acute onset of ocular pain, edema, and hyperemia of the eyelid, exacerbated by expiratory movements such as sneezing and coughing.54

    A pyogenic granuloma, also known as lobular capillary hemangioma, is a common, acquired, benign vascular proliferation of the skin and mucous membrane. Clinically, a pyogenic granuloma presents as an asymptomatic soft, dome-shaped papule/nodule or a sessile or pedunculated papule/nodule with a smooth, glistening, erosive, or friable surface.55,56 The color is usually bright red to purple to dusky red initially because of the high number of blood vessels. With time, the vascularity decreases, and the lesion tends to become more collagenized and pink.56

    Characteristically, seborrheic keratosis presents as an asymptomatic, sharply demarcated, round or oval plaque with a “stuck on” warty appearance. It is typically brown but may be yellow, reddish, or black.57,58 Often, the lesion appears oily and shiny, hence the misnomer “seborrheic” (greasy) keratosis. The face is a site of predilection.

    Sebaceous hyperplasia is an asymptomatic common and benign proliferation of sebaceous glands. The condition usually presents as a solitary, or more commonly, as multiple discrete, yellow or flesh-colored, dome-shaped papules.59,60 Individual lesions are usually 2 to 5 mm in diameter. The face is most commonly affected.59

    Amyloidosis is characterized by abnormal extracellular accumulation and deposition of amyloid fibrils.61 Cutaneous amyloid deposits may occur with both primary or systemic amyloidosis.62 Primary cutaneous amyloidosis of the eyelid, especially if unilateral, may masquerade as a chalazion.61,62

    Benign tumors (eg, trichilemmal cyst, keratinous cyst, pleomorphic adenoma, sebaceoma, leiomyoma)63-67 and malignant tumors (eg, sebaceous carcinoma, squamous cell carcinoma, basal cell carcinoma, Meckel cell carcinoma, lymphoma, plasmacytoma)68-76 involving the eyelid may mimic a chalazion.

    Miscellaneous conditions that may masquerade as a chalazion include retained soft contact lens, lacrimal gland duct stone, para-canalicular abscess, and tuberculosis of the eyelid.50,77-80

    COMPLICATIONS

    A chalazion may be cosmetically unsightly.81 The lesion may become secondarily infected. Rarely, an infected lesion may lead to preseptal cellulitis, especially in an atopic individual.27 At times, the lesion may rupture and result in the formation of pyogenic granuloma. A chalazion may be associated with meibomian gland dysfunction or chronic blepharitis.25,82 If the lesion is large enough, it may cause visual problems such as astigmatism, decreased vision, and hyperopia by exerting pressure on the eyeball.3,10,15,25,80 Recurrence of chalazion is common.

    PROGNOSIS

    Without treatment, up to 25% of cases may resolve spontaneously within 6 months.83 Some untreated cases may take several years to resolve.84

    MANAGEMENT

    Up to 80% of chalazia resolve with conservative treatment consisting of eyelid hygiene, twice daily warm compresses for 10 to 15 minutes, and gentle lid massage.6,7,10,25,81,82 Warm compresses help to soften the plug of material so that it is easier to drain.6 The massage should be performed over the lesion, in the direction of the eyelashes to promote drainage of the obstructed gland.7,25 Topical antibiotic should be used if the lesion is infected.7 If the chalazion fails to resolve with conservative treatment and is cosmetically blemishing, a surgical approach should be considered.

    Intralesional corticosteroid injection is a therapeutic option for the treatment of chalazion.7,85 The procedure can be painful.85 On the other hand, subcutaneous corticosteroid injection into the looser extralesional tissue results in less pain.85 Because skin depigmentation may occur at the injection site, subconjunctival corticosteroid injection rather than subcutaneous corticosteroid injection is preferred, especially in patients with a darker complexion.3 Large lesions or lesions that fail to respond to 2 or 3 corticosteroid injections may require subconjunctival incision and curettage.2,5,26 Anterior orbital cyst formation may occur as a late complication of surgery for chalazion.86 A meta-analysis of 8 randomized control trials comparing incision and curettage (n = 264) with intralesional corticosteroid injections (n = 288) showed that incision and curettage was more effective than intralesional corticosteroid injections, with a success rate of 78% vs 60.4%, respectively.87 Combination of incision and curettage with corticosteroid injections is more comfortable for patients and can achieve a success rate of approximately 98%.88-90

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