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Bullous pemphigoid, varicose veins, dermatomyositis, myasthenia gravis, coma bullae, xerosis, atrophie blanche, Grover disease-a review of age-related clinical concerns and how to recognize them.
Most inflammatory dermatoses are less common in older persons than in young or middle-aged adults, but some inflammatory dermatoses-particularly vesiculobullous eruptions caused by circulating autoantibodies-are more common in patients older than 60 years.
Bullous pemphigoid, a chronic, autoimmune, subepidermal disease, mostly affects older persons in the fifth through seventh decades of life. The average age at onset is 65 years.
The various forms of bullous pemphigoid include generalized bullous, vesicular, vegetative, generalized erythroderma, urticarial, nodular, and acral. The last form is seen on this man’s hand.
Bullous pemphigoid, which manifests with tense blisters, usually starts on the arms and legs and rarely involves the mucous membranes. The differential diagnosis includes other blistering diseases, urticaria, and vesicular types of eczema. The diagnosis is established with a biopsy sample prepared for and examined with direct immunofluorescence.
Case and photo provided by Noah S. Scheinfeld, MD, JD
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Varicose veins are seen here on the leg of an older woman. Older adults are at increased risk for varicosities because the aging elastic lamina of the vein thins and its smooth muscle degenerates, resulting in irreversible venous dilatation. Women are particularly susceptible.
Varicose veins-normal veins that have dilated because of increased venous pressure-are the visible surface manifestation of underlying vascular insufficiency. They range in size from 1 mm to 1 cm in diameter and in color from green to red to blue.
Venous insufficiency involves incompetence of valves in the veins, which allows blood to move in a retrograde direction toward the leg rather than the heart. Symptoms include pain, soreness, burning, aching, throbbing, cramping, muscle fatigue, and “restless legs.”
New varicose veins can be easily distinguished from chronic varices by palpation.
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Dermatomyositis, a systemic disorder that manifests with inflammatory myopathy and cutaneous eruptions, often occurs in older patients. The typical age of onset is in the 50s and 60s.
The condition has a variety of cutaneous manifestations. Distinctive red, blue, or purple patches and plaques often are present. The characteristic heliotrope rash, an eruption over the eyelids and periorbital area, is seen in this woman. Gottron papules are another common feature.
Dermatomyositis can present with erythema of the face, neck, upper trunk, knuckles, elbows, and knees. Poikiloderma also may be noted in a photosensitive distribution. Diagnostic criteria include proximal muscle weakness, elevated serum muscle enzyme levels, diagnostic muscle biopsy, and characteristic changes on electromyography.
Dermatomyositis often is associated with underlying malignancy in older persons. The most common associated malignancies are those of the breast, colon, lung, ovary, stomach, and uterus. Ovarian cancer has the strongest association with paraneoplastic dermatomyositis.
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After 5 days of slurred speech, a man in his 70’s came to the ED. He had difficulty in swallowing and chewing and had been using his hand to hold his jaw closed. Phonation seemed weak and a bit nasal.
The patient had myasthenia gravis. The diagnosis was made based on symptoms of bilateral cranial nerve weakness in an older man without other symptoms. Almost one-third of cases present in the older population.
Myasthenia gravis usually presents with fluctuating weakness that worsens with prolonged muscle use. It typically starts in the cranial nerves, often causing diplopia, ptosis, trouble chewing or swallowing, or a soft/weak voice. With progression, the neck and shoulders may be affected next; eventually other skeletal muscles are involved.
The most feared complication of myasthenia gravis is respiratory failure.
Case and photo courtesy of Brady Pregerson, MD
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Patients who are receiving dialysis for chronic renal failure and those who have diabetic ketoacidosis, hypercalcemia resulting from hyperparathyroidism, and neurologic conditions are at increased risk for coma bullae, as shown here on the palm of an older woman.
Coma bullae typically are seen in older patients who have been admitted to the hospital after falling to the floor and lying unconscious for long periods. Also linked to these lesions are the use of opiates, tricyclic antidepressants, antipsychotics, glutethimide, and methaqualone.
Coma bullae occur on body areas to which pressure has been applied. They arise as tense blisters without much erythema or as urticarial plaques, which in most cases evolve to blisters. They are limited to cutaneous involvement and most often are observed over bony eminences.
Biopsy demonstrates the presence of a subepidermal bulla and eccrine gland necrosis. Direct immunofluorescence may demonstrate deposition of IgM and C3 in the dermal vessels.
Early recognition and treatment can prevent further consequences, such as penetration of lesions into muscle and adipose tissue.
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Xerosis, a common condition in older persons, is characterized by pruritic, dry, cracked, and fissured skin. Visible xerosis, which occurs most frequently on the legs, may be so severe as to overlap with ichthyosis, which manifests with thick, fish scale–like xerotic plaques, as seen here on an older man’s legs and feet.
Because epidermal integrity decreases with age, the prevalence of eczematous inflammatory dermatoses is higher among persons older than 60 years.
Xerotic skin has the appearance of cracked porcelain; the cracks arise from loss of water from the epidermis. Xerosis disrupts the desquamation process; powdery flakes develop and become visible on the surface of the skin. It tends to be more severe in winter, when humidity is lower.
Xerosis results in large part from physical changes in the skin that occur with normal aging. The decreased activity of sebaceous and sweat glands in older persons is one of the main contributing factors. Decreased skin thickness and decreased hydration also play key roles.
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The white plaque on this man’s leg is atrophie blanche, a type of scar that arises on the lower leg after a skin injury in persons with vascular insufficiency. The incidence of atrophie blanche increases with age and with the presence of comorbid conditions, such as diabetes mellitus.
Petechial or purpuric papules or hemorrhagic bullae initially develop at the site of an injury and then become necrotic and form ulcers. In time, atrophie blanche appears as a star-shaped or polyangular, ivory-white depressed atrophic plaque. Sometimes prominent petechiae are present within the scar, and there may be an increase in surrounding pigmentation.
Atrophie blanche may occur in association with or after Raynaud phenomenon, livedoid vasculitis, livedo reticularis before ulceration, venous insufficiency, and diabetic vascular disease. It is thought to result from an underlying vasculitis or coagulopathy.
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Grover disease, characterized by discrete erythematous to red-brown keratotic papules, is most common in white men older than 65 years. It probably is related to xerosis.
The papules are not follicular and do not coalesce. They can be acneiform, vesicular, pustular and, rarely, even bullous. The condition can be pruritic, even extremely pruritic.
Grover disease typically occurs on the back and abdomen, although unusual distributions and locations may be noted. It does not occur on the face, palms, or soles.
The differential diagnosis includes insect bites, folliculitis, cutaneous candidiasis, drug eruptions, disseminated herpes, and miliaria.