CLINICAL CHALLENGES IN OCULAR DERMATOLOGY
Bernard H. Blaustein, O.D., F.A.A.O.
Learning Objectives: 1. To enhance the optometrist’s observational ability with regard
to dermatologic lesions that involve the eye and ocular adnexa
2. To enhance the understanding of the pathologic mechanisms
that underlie dermatopathologies so as to be better able to
recognize potential malignancies
3. To develop an appreciation for the use of a logical systematic
approach in the formulation of diagnoses and therapeutic
regimens of benign and malignant dermatologic lesions
I. Benign Skin Lesions
A. Contact dermatitis
- A dermatitis caused by an exogenous stimulus
- Mediated by a Type IV hypersensitivity reaction
- Pre-existing atopic dermatitis encourages the development of contact dermatitis.
- Clinical features of acute contact dermatitis include erythema which then
develops into tiny vesicles and then scales and crusts and intense pruritus
(itching); clinical features of sub-acute and chronic contact dermatitis lack the
extreme acute signs and symptoms but thickened rough skin and accentuation of
the skin lines develop.
- Pathophysiology involves induction phase, i.e. the introduction of the allergen
and the programming of T cells and effector phase, i.e. the activated T cells
respond to re-exposure of the allergen with the release of lymphokines
- Dermatitis spreads from the original contact site to other body parts because of
inadvertent transfer of the allergen by the hands and because activated T cells
circulate to other body parts.
- The most common complication is secondary bacterial infection.
- Treatment: remove the allergen, oral steroids (Medrol Pac), topical
corticosteroid ointment, e.g. triamcinolone or Aclovate , systemic
antibiotics to avoid secondary infection
B. Rosacea
- A chronic, idiopathic skin inflammation occurring in middle-aged patients
- Characterized by telangiectasia, papules, pustules and hypertrophy of the
sebaceous glands on the forehead, cheeks, neck, and bulb of the nose
- The face flushes abnormally when the patient ingests certain foods, e.g. alcohol,
hot foods or beverages, spicy foods or when the patient engages in strenuous
exercise and has a temperature rise.
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- Approximately, forty percent of patients experience recurrent anterior and
posterior blepharitis, peripheral corneal thinning and neovascularization,
peripheral corneal ulcers, recurrent hordeola, recurrent phlyctenulosis.
- Treatment: avoid triggers, systemic tetracycline, topical metronidazole ointment
C. Epidermal inclusion cyst (sebaceous cyst)
- Yellow-white lump with a central punctum filled with cheesy keratin secretion
- Occur along lid margin, canthi, and periorbital area
- Does not transilluminate
- Milia are tiny yellow-white miniature epidermal inclusion cysts
- Treatment: incision and drainage
D. Syringoma
- The most common of the sweat gland tumors
- Appear as multiple 1-3 mm cysts of eccrine glands ducts on the cheeks
- Are flesh-colored or yellowish papules commonly seen in females
E. Xanthelasma
- Flat, sharply circumscribed, yellowish lipid-laden plaques occurring
periorbitally
- Occurs because lipids leak out of blood vessels in the dermis; lipids are taken up
by macrophages
- Fifty percent of patients have elevated serum lipids
- Treatment: surgical removal via electric cautery or trichloracetic acid
F. Papilloma
- A benign lesion consisting of epithelial tissue covering a fibrovascular core
- If associated with a keratin horn, maybe pre-malignant
- May be non-viral or viral
- Non-viral papillomas are commonly referred to as skin tags or squamous
papillomas, usually occur singularly, and may be flat-based (sessile) or
have a stalk (peduculated).
- Commonly have a lobulated surface
- Viral papillomas (warts) (verucca ) are caused by stains of the human
papillomavirus and usually occur in clusters
- Viral papillomas will remit in 2-3 years
- Treatment of non-viral papillomas: excision and biopsy if associated with a horn
- Treatment of viral papillomas that do not remit: excision, salicylic acid,
application of an immune stimulator such as imiquimod (Aldara)
G. Molluscum contagiosum
- Appears as a round, white, waxy, dome-shaped lesion with a central umbilicus;
caused by a poxvirus and is contagious
- May be very small and be hidden by the lashes on the lid margin
- Lid margin lesions may exude viral particles and cause a viral conjunctivitis
- Treatment: cryosurgery
H. Actinic keratosis
- Dry, red, scaly lesion with excess keratin in the squamous layer (dyskeratosis)
occurring in older, fair-skinned patients
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- Lesions located on sun-exposed areas, e.g. ears, neck scalp, arms
- Often felt before it is seen
- May be a precursor to squamous cell carcinoma; biopsy to rule out malignancy
- Treatment: prophylaxis with appropriate sunscreens that block UVB;
cryosurgery
I. Keratoacanthoma
- Emanates from the neck of a hair follicle; occurring in sun-exposed areas
- Appears as a dome-shaped nodule surrounded by a smooth wall of inflamed skin
capped with an irregular crater with a central keratin core
- Grows rapidly, reaching a large size in days or weeks and then self limits and
scars
- May be a low-grade form of squamous cell carcinoma; biopsy to rule out
malignancy
- Treatment: Mohs’ micrographic surgery
J. Herpes zoster ophthalmicus
- A vesicular response which follows a dermatome and does not cross the midline
- The ophthalmic division of the trigeminal nerve is most often affected.
- If the tip of the nose is affected there is a high probability of ocular involvement.
- Preceded by several days of face pain
- Etiology is the varicella virus which resides in the Gasserian ganglion
- Occurs in debilitated or immunocompromised older adults
- Treatment: Acyclovir 800 mg 5X per day for 10 days; Famciclovir 500 mg 3X
per day for 10 days; Valacyclovir 1000 mg 3X per day for 10 days
- Treatment most effective if begun within 48-72 hours of outbreak
- Oral steroids in addition to acyclovir derivatives may accelerate resolution.
K. Capillary hemangioma
- A benign tumor of newly-formed blood vessels occurring at birth or shortly
afterbirth
- Small, endothelial-lined blood vessels proliferate into a mass of anastomosing
blood-filled channels
- The most common orbital tumor in children; often has a bluish-purple
appearance subcutaneous appearance that will blanch with pressure
- Lesion will remit in 4-7 years
M. Sturge-Weber syndrome
- A permanent cutaneous hemangioma involving a portion of the face
innervated by one or more divisions of CN V
- May be associated with cerebral calcifications leading to hemiplegias,
hemisensory loss, hemianopsias, seizures, mental retardation
- May cause hemangioma of the choroid (tomato ketchup appearance)
- If the ophthalmic division of CN V is involved, there is an increased risk of
neurologic and ocular involvement.
- If the episcleral venous plexus has A-V shunts, the episcleral venous pressure
will increase, blood will develop in develop in Schlemm’s canal, and glaucoma
may ensue.
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- Treatment: argon laser therapy to bleach skin; if the upper lid is involved assume
potential glaucoma and perform tonometry every 6 months until age 21
N. Benign pigmented lesions - freckles, nevi (moles), lentigos
1. Freckles (ephelides) - congenital and are due to an excess of melanin;
are located in sun-exposed areas and darken upon sun exposure
2. Acquired melanocytic nevi, AKA nevi or moles are an increased number of
melanocytes that are grouped in clusters and are located in the basal epidermal
layer. Acquired nevi proceed through several stages: junctional, compound,
intradermal
3. Junctional nevus– flat, brown 5-6 mm maculeconsisting of an increased
number of grouped melanocytes that have migrated to the epidermal-dermal
junction
4. Compound nevus - nests of melanocytes have migrated downward and are now
at the epidermal-dermal junction and in thedermis; lesion is slightly elevated
and lighter in color
5. Intradermal nevus – nests of melanocytes totally in the dermis; lesion has lost
all color and is elevated
6. Blue nevus - embryonal nests of melanocytes that have never reached the
epidermis and have not undergone the above-described migration; has a deep
blue/purple color
7. Lentigo simplex - an acquired brown macula due to an increase number of
melanocytes; located above the BM of the epidermis; melanocytes are not in
nests and do not migrate; arises in children and young adults; does not darken
with sun exposure
8. Lentigo senilis (solar lentigo, liver spot, age spot) is acquired and is larger
than a lentigo simplex. The borders may be somewhat irregular, a situation
which indicates some sun damage.
II. Malignant Skin Lesions
Suspicious lesions include sores that do not heal,sores that bleed frequently,
lesions that are nodular, lesions that distort the lid margins, lesions that
produce madarosis (loss of lashes), recurrent chalazia at the same location,
elevated
pigmented lesions
A. Basal cell carcinoma (BCC)
- Most common malignant skin tumor
- Most common cancer of the eyelid – over 90%
- Mostly occur on the side of the nose; also on neck and face of fair-skinned
patients who have had a great deal of sun exposure
- Slow-growing, invasive but not metastatic
- Has three common presentations: nodular, ulcerated, sclerosing
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- Nodular BCC presents as a raised lesion with nodular pearly borders,
telangiectatic vessels along its indurated base and a crusty eroded center that
often bleeds
- Ulcerated BCC presents as a non-nodular ulcerated lesion that bleeds and crusts
over
- Sclerosing BCC presents as a firm, flat subcutaneous lesion resembling a scar;
has much more malignant potential
- Treatment: Mohs micrographic surgery
B. Squamous cell carcinoma (SCC)
- 2nd most frequent skin cancer after BCC; 5% of all eyelid cancers
- Often arises from precancerous actinic keratosis and cutaneous horns
- Occurs on sun-damaged skin, scar tissue, burns, and chronically draining areas
- Has the potential for both local destruction and metastasis
- Often starts as a small firm erythematous papule; then enlarges with ulceration,
bleeding, and crusting
- Appearance of lesion is variable and may be confused with actinic keratosis,
keratocanthoma, BCC, seborrheic keratosis, sebaceous gland carcinoma
- Treatment: Mohs’ micrographic surgery
C. Sebaceous gland carcinoma
- 3-7% of all eyelid malignancies; very aggressive
- Upper lid 2-3 times more commonly involved than lower lid
- Originates in meibomian glands, glands of Zeis, and sebaceous glands of
eyebrows and caruncle and metastasizes to orbit and distant sites
- Presents as a firm, slowly growing, painless nodule with surface telangiectasias
and a yellowish color (resembles a chalazion)
- May spread to the conjunctiva and resemble chronic, recalcitrant
blepharoconjunctivitis or chronic meibomian gland dysfunction; chronicity can
last for years; loss of cilia is a clinical clue that the lesion is malignant
- Recurrent chalazia, particularly in the same location, may indicate SCC
- May be part of the Muir-Torre syndrome, in which the patient has colorectal
carcinoma and/or genitourinary malignancy.
- Treatment: If no metastasis to orbit – Mohs’ micrographic surgery; if orbital
involvement – exenteration
D. Malignant melanoma
- Melanocytes that have undergone malignant transformation; strong potential for
metastasis
- 3% of all skin cancers; 1% of eyelid malignancies, but accounts for 60-70% of
all deaths from skin cancer
- Risk factors include fair skin, blonde hair, a tendency to burn rather than tan,
severe sunburn episodes in childhood, family history, congenital nevi, multiple
dysplastic nevi, lentigo maligna
- Normal nevus is less than 6 mm in size, is uniform in color varying from
lightbrown to black, has a symmetrical shape such that there are matched
halveswhen the lesion is folded, has regular, distinct borders, and does not
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undergo change
- Dysplastic (atypical) nevusislarger than 6 mm, hasdisorderly and
haphazard colors, has anasymmetric contour and shape, has both flat and
elevated surfaces, borders are irregular and may be notched. A dysplastic
nevus may be a precursor of a malignant melanoma.
- Mnemonic for malignant changes in nevi:
A = Asymmetry (non-matching halves)
B = Border is irregular or notched
C = Color is variable (multiple colors within lesion)
D = Diameter is greater than 6 mm
E = Evolving (nevus is changing over time)
- Malignant melanomas have three clinical presentations: superficial spreading
melanoma, nodular melanoma, lentigo maligna melanoma
1. Superficial spreading melanomaapproximates 70% of all the malignant
melanomas; occurs in ages 40-50; presents with irregular notched borders, a
bizarre shape, and red, white and blue colors
2. Nodular melanomaapproximates 15% of malignant melanomas, occurs
between ages 50-60; nodular development signals deeper penetration into
dermis; presents as a dome-shape nodule with a dark brown, dark black,
reddish-brown or reddish-black color; often misdiagnosed as a blood blister or
dermal nevus
3. Lentigo maligna melanomaapproximates 5% of all malignant melanomas;
occurs in elderly patients above age 80; arises from a precursor - lentigo
maligna, AKA malignant melanoma in situ, after the lentigo maligna has been
present for many years
- The development of a dark brown ulcerating nodule within the initially flat
lentiga maligna implies vertical growth from the epidermis into the
dermis and signals the development of a malignant melanoma.
- Treatment for all types of melanoma: Surgery - early stage melanoma; surgery
and immunotherapy for more advanced stages of melanoma