History
Because of the late onset of acrodermatitis chronica atrophicans (ACA), patients rarely remember a tick bite. Instead, they recall having been in the woods or grassy areas a few months or years previously, especially in a geographically endemic region. ACA tends to be diagnosed in older individuals, localized anatomically to the extremities, perhaps predisposed to by age-related anatomic or physiologic changes. [19]
About 20% of patients report a history of erythema migrans. ACA can develop directly from EM or can arise after 6-36 months, often involving the same region of the body. Sometimes, the disease may be preceded by a latent phase (lasting up to several years) or by other manifestations of Lyme borreliosis; the latter can also develop simultaneously.
Commonly, the patient notices localized cutaneous swelling on the distal extremity or on a single digit; sometimes, he or she discovers that one foot is larger than the other when buying shoes. ACA is most often unilateral, although bilateral ACA is also common. However, since lesions may not be symptomatic, patients may not seek medical assistance. [9] Clinical suspicion may be necessary for the diagnosis. [20]
Progressive allodynia (exaggerated reaction to pain) is a characteristic symptom and thus may be a clue to the diagnosis of ACA. Patients commonly report spontaneous acral pain and paresthesia or dysesthesia or cognitive dysfunction.
ACA starts with an inflammatory phase, characterized by multiple soft, erythematous, slowly enlarging cutaneous swellings or flat infiltrations of various sizes or by diffuse bluish red discoloration and edema of the skin. As noted, it usually appears on the distal part of at least one extremity, predominantly on the extensor surfaces of the bony prominences (see the image below).
The typical inflammatory phase patches are seen on the right hand bone prominences.
Common sites of ACA are the foot, the lower leg or the hand, the forearm, and the olecranon area (see the image below); proximal locations on the upper arm and the shoulder or the thigh and the buttock may be seen but are uncommon. Sometimes, the erythema is slight and swelling may dominate, or the signs may be very subtle and go unrecognized by the patient or the physician. Lymphadenopathy may be noticed.
The most common localization of the skin lesions in 12 patients with acrodermatitis chronica atrophicans (ACA). The number of ACA lesions in the particular body region is shown.
For many months or years, only a single part of an extremity may be affected. With time, the skin lesions may extend on 1 extremity or may appear on additional extremities and also involve other parts of the body.
Fibrotic nodules (often multiple, localized linearly in the vicinity of joints) are typical. They can precede ACA or develop simultaneously with it. The most common sites of these nodules are the elbows and the knees.
ACA does not heal spontaneously; gradual conversion into its atrophic phase may occur over many years of infection. In this later phase, the skin becomes thin, atrophic, wrinkled, dry, and translucent. The hair is lost, and the numbers of sebaceous and sweat glands are decreased. Even minor trauma may produce large, slow-to-heal ulcerations of the affected skin.
About 5-10% of patients with ACA develop sclerodermalike plaques. Anetodermalike skin lesions can be seen concomitant with ACA. Occasionally, numerous small violaceous patches and small spinous papules with a background of faint erythema may be evident. [21]
Frequently, ACA is accompanied by peripheral neuropathy, musculoskeletal pains, and joint damage underneath the cutaneous plaques. Involvement of the small joints of the hands and the feet by the fibrotic reaction is often seen. An uncommon cutaneous manifestation is septolobular panniculitis, which may resemble a cellulitis. [22]
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The most common localization of the skin lesions in 12 patients with acrodermatitis chronica atrophicans (ACA). The number of ACA lesions in the particular body region is shown.
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A 73-year-old female farmer with a cutaneous plaque on the sole of her right foot lasting for 6 months that, in the meantime, had extended onto the dorsum of her foot, her right leg, and the lower part of her right thigh. Infection of Borrelia burgdorferi was diagnosed with enzyme-linked immunosorbent assay, and acrodermatitis chronica atrophicans was confirmed histologically.
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The external part of the right foot.
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A 50-year-old male farmer was examined for cutaneous plaques on the dorsal side of his right hand lasting for 8 months that, in the meantime, had extended onto his right forearm and arm and had also developed on his right thigh. The patient complained of muscular weakness related to his right upper limb and periodic arthralgia. The neurologic examination demonstrated signs of right brachial plexus damage, confirmed by electromyography. Borrelia burgdorferi infection was diagnosed with enzyme-linked immunosorbent assay, indirect immunofluorescence assay (titer: 1:1,024), and Western blot. Histologic examination confirmed the diagnosis of acrodermatitis chronica atrophicans.
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The typical inflammatory phase patches are seen on the right hand bone prominences.
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A 68-year-old woman with a history of untreated erythema migrans on her left thigh 2 years previously. Ten months later, the plaque extended over the skin of her left buttock and became bluish with signs of livedo racemosa. Her forearms and breast were also involved. Borrelia burgdorferi infection was diagnosed with indirect immunofluorescence assay (1:4,096) and Western blot. Acrodermatitis chronica atrophicans was confirmed histologically. Because of intrathecal production of specific antibodies, diagnosis of asymptomatic neuroborreliosis was established.
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After 30 days of treatment with ceftriaxone.
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The livedo racemosa and acrodermatitis chronica atrophicans lesions on the left thigh and buttock before treatment.
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The same patient after treatment.
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A 69-year-old woman. The initial lesion developed on the dorsal side of her left hand 2 years previously and extended onto her left forearm and arm. A new erythematous lesion developed 2 months before on her right cheek beside the rosacea signs. Acrodermatitis chronica atrophicans was confirmed in the biopsy specimen taken from the skin of the forearm, and Borrelia burgdorferi infection was diagnosed with indirect immunofluorescence assay (1:2,048). The atrophic phase of acrodermatitis chronica atrophicans is visible on the hand, and the inflammatory phase is visible on the cheek.
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The inflammatory phase of acrodermatitis chronica atrophicans can be seen with rosacea lesions on the cheek, the forehead, and the nose.
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Fibrotic nodules on the left elbow.
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A 48-year-old woman with a history of frequent tick bites and an initial inflammatory skin lesion on the left medial part of her ankle 2 years previously. The lesion extended onto the left leg and involved the knee. Fibrotic nodules developed in the medial part of the ankle and the knee. Moreover, she complained of balance disturbances and vertigo. Neurologic examination revealed the asymmetry of profound reflexes, bilateral lack of plantar reflexes with a tendency to the extensor plantar response (Babinski sign), ataxia, profound dysesthesia, and muscular atrophy of the left calf. Acrodermatitis chronica atrophicans was confirmed by histologic examination, and Borrelia burgdorferi infection was confirmed by a high specific antibody titer with indirect immunofluorescence assay (1:8,192); cerebrospinal fluid was not tested.
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A 26-year-old female nurse recalled the onset of the disease on her right arm 4 years before. After 6 months, the plaques extended onto the right forearm and hand. The left arm and forearm were also involved 3 years previously. Induration of the skin of the right forearm was noted. Borrelia burgdorferi infection was diagnosed with indirect immunofluorescence assay (1:2,048) and confirmed by positive Western blot for both immunoglobulin M and immunoglobulin G antibodies.
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Atrophic phase of acrodermatitis chronica atrophicans of the right upper limb with induration of the forearm.
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A 68-year-old female jogger frequently exposed to ticks. Cutaneous plaques developed 4 years previously on her right lower limb and the right part of her trunk, including her breast and right upper limb. Typical extensive cigarette paper–like plaques, bluish or brownish red in color were evident. Fibrous nodules were found on her right elbow. Borrelia burgdorferi infection was confirmed with indirect immunofluorescence assay (1:1,024) and Western blot. Acrodermatitis chronica atrophicans was finally diagnosed by using histologic examinations.
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A widespread acrodermatitis chronica atrophicans atrophic plaque on the back.
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The atrophic skin lesions and fibrotic nodules of the right upper limb.
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Biopsy specimen from the wrist of the patient shown in Image 4. The epidermis is slightly flattened. A zone of normal connective tissue can be seen below the epidermis. A patchy infiltrate consisting of lymphocytes and plasma cells is seen throughout the dermis. Telangiectasias are evident in the upper and deeper parts of the dermis.
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A higher magnification. Telangiectatic vessels are surrounded by a cellular infiltrate in the upper dermis.
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A higher magnification of the same biopsy specimen. Note the patchy cell infiltrate around the large vessels in the deep dermis.
