Acrodermatitis Chronica Atrophicans Clinical Presentation

Updated: Dec 14, 2022
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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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 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 i 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]