Fig. 4.42 Carcinoma of the lid is usually basal cell, but squamous cell looks similar and is also common.

The lids, face and scalp are the most common locations for basal cell carcinoma, and, less often, squamous cell carcinoma, of the skin (Fig. 4.42). They are strongly related to exposure to the sun's ultraviolet rays. Basal and squamous cell carcinomas are the most common malignancies in humans , occurring in 1 in 5 Americans. All chronic, hard, nodular, umbilicated, ulcerated, vascularized lesions demand a biopsy. Recommend that everyone, especially fair-skinned individuals avoid excessive exposure to the sun.

Cutaneous horns (Fig. 4.43) are keratinized overgrowths of seborrheic keratosis, verruca, or squamous or basal cell carcinoma; therefore, a biopsy of the base is indicated.

Phakomatoses

Congenital syndromes, which include lesions of the brain, skin, and eye are called phakomatoses. The early onset of skin lesions in these infants and young children provide a red flag to alert one to other problems.

1. Tuberous sclerosis is a condition appearing in the first 3 years of life consisting of seizures, mental deficiency, and sebaceous adenomas. Seventy-five percent of patients die before age 20 (Figs 4.44 and 4.45).

Fig. 4.43 Cutaneous horn.

Fig. 4.44 Ash-leaf spots on the skin are multiple, depigmented macules with irregular borders. They are usually the first sign of tuberous sclerosis and appear in up to 90% of patients.

Fig. 4.45 Retinal astrocytoma in tuberous sclerosis. Areas of calcification give mulberry appearance. Courtesy of Dana Gabel Barnes Retinal Institute, St Louis, MO.

2.Sturge-Weber syndrome includes facial port wine capillary malformations (Fig. 4.46) and mental retardation in half of the patients. They should be monitored for early onset glaucoma and choroidal and CNS hemangiomas.

3.Neurofibromatosis is a condition that is inherited in an autosomal dominant pattern with incomplete penetrance. Tumors could affect the optic nerve, iris, retina, and skin of the lid (Fig. 4.47). Lisch nodules in the iris are present in 94% of patients (Fig. 4.48). Brown macular lesions occur early on and eventually in 99% of patients.

Leprosy is a chronic disease caused by acidfast Mycobacterium leprae. It is probably transmitted by the respiratory route and usually involves prolonged exposure in childhood.

Fig. 4.48 Lisch nodules on the iris of a patient with neurofibromatosis. Courtesy of S. J. Charles, FRCS and Arch. Ophth., Nov. 1989, Vol. 107,

p. 1572. Copyright 1989, Amer. Med. Assoc. All rights reserved.

Fig. 4.46 Sturge–Weber syndrome.

Fig. 4.47 Neurofibromatosis (von Recklinhausen disease) is characterized by neurofibromas of the skin (≠) and nervous system, and cafe-au-lait spots (ØØ), which are irregularly shaped brown macules. There are usually 5 or more, increasing in size from 0.5cm to 1.5cm in adults.

Fig. 4.49 Twenty-two year old from Cape Verde Islands with lepromatous leprosy. There are macular and erythematous nodular lesions on face, trunk, and extremities. Fite’s stain/acid fast bacilli in skin biopsy confirms the diagnosis.

Fig. 4.50 Leprosy causing a solid nodule on the ocular surface together with granulomatous iritis. Courtesy of Carly Seidman, BS and Arch of Ophth., Dec 2010, Vol 128, p. 1522. Copyright 2010, Amer. Med. Assoc. All rights reserved.

Fig. 4.51 Anterior blepharitis with crusting flakes on lashes. Courtesy of Michael Lemp, MD.

Anterior and posterior blepharitis

Blepharitis refers to inflammation or infection of the lid margin. It is extremely common and there is rarely a day that goes by that an eye doctor doesn't treat it or one of its sequelae, such as conjunctivitis, sties, chalazions, corneal ulcers, lid cellulitis, dry eye, or intolerance to contact lenses.

The anterior type manifests with crusting, redness, and ulcerative lesions around the lashes (Figs 4.51 and 4.52). It is often associated with scalp dandruff. The posterior type is due to infection of the meibomian glands. These glands normally secrete the oily meibum that helps minimize tear evaporation and lubricate the eye so as to minimize discomfort from the estimated 50,000 blinks per day. Many times, these glands become dysfunctional and produce a white, pasty secretion that predisposes the glands to infection (Figs 4.3 and 4.55). To minimize the incidence of infection, predisposed patients routinely cleanse the margin with baby shampoo or commercially available lid scrubs together with warm compresses which remove toxic debris and desquamated cells.

When infection does occur – usually due to staphylococcus – topical ophthalmic antibiotic

Fig. 4.52 Anterior blepharitis with crusting and ulcerative lesions around lashes. Courtesy of Michael Lemp, MD.

Fig. 4.53 Blepharoconjunctivitis in acne rosacea. This chronic condition is associated with engorged vessels and pustules on the nose, forehead, cheeks, and chin.

Fig. 4.54 Posterior blepharitis: dysfunctional meibomian glands are a common, chronic problem characterized by a toothpaste-like secretion. The glands are predisposed to infection causing posterior blepharitis and/or a chalazion.

drops and/or ointments with or without steroids may be used, Generic tobramycin (Tobrex), polymyxin/trimethoprim (Polytrim) solution, and bacitracin ointment are highly effective. Generic tobramycin/dexamethasone (Tobradex) also reduces the inflammatory response to staphylococcus toxins and scaling debris. Oral generic doxycycline has antibacterial and anti-inflammatory effectiveness; but because of its systemic side effects, is reserved for more recalcitrant episodes. Squeezing the meibomian glands between a sterile cotton applicator and your finger tip, followed by placement of Betadine on the lid margins helps speed recovery (Fig. 4.54). This technique of eleciting discharge is also diagnostic when the diagnosis of posterior blepharitis is in doubt. Patients should be reminded that each episode of blepharitis could be treated, but it often recurs.

Chalazions (Fig. 4.57) are cystic enlargements of the meibomian glands that are due to clogging of an orifice. They could last for months. A chalazion is often incised and

Fig. 4.55 Posterior blepharitis with foamy residue overlying the meibomian glands. This breakdown product of abnormal secretions may irritate the eye. Courtesy of Michael Lemp MD.

Fig. 4.56 Anterior and posterior blepharitis is often occur together and could cause sties and chalazions.

Fig. 4.57 Chalazions point internally.

drained (Fig. 4.58). If they become infected, antibiotic and/or steroid eye drops are added.

Sties are infections of the glands of Zeis and Moll around the lashes. These pimples are treated with hot soaks, local antibiotics, and incision. Systemic antibiotics are indicated if there is significant surrounding cellulitis.

Lid cellulitis is a diffuse infection often due to a sty, chalazion, bug bite, or cut. Lids are red and tender (Fig. 4.60). There may be adenopathy and fever. Rx: topical and systemic antibiotics. Shriveled skin, as in Fig. 4.19, is an initial indication that lid cellulitis is responding to treatment. Be cautious. When extensive, a CT scan could rule out involvement of sinuses or orbit.

Fig. 4.58 A chalazion clamp is used to minimize bleeding during incision and curettage.

Fig. 4.59 Sties point externally.

Fig. 4.60 Lid cellulitis.