Chronic atrophic acrodermatitis is a skin condition caused by Lyme disease, characterized by skin atrophy, fibrosis, and peripheral neuropathies. Diagnosis requires serologic testing and skin biopsy, and treatment includes antibiotics and rehabilitation therapy.
Chronic atrophic acrodermatitis (ACA) is a skin condition during the latter stages of Lyme disease, or Lyme borreliosis, caused by the infectious organism called Borrelia burgdorferi or Borrelia afzelii. In chronic atrophic acrodermatitis, the skin undergoes gradual atrophy, a reduction in size due to a decrease in both the size and number of cells. This disease, which is also called Herxheimer’s disease or primary diffuse atrophy, has an early inflammatory stage, in which widespread or localized redness or bluish-red discoloration can be observed, and swelling of the skin can be observed. When it spreads, the extensor and articular surfaces of the extremities are involved. The spreading nature of skin lesions is why they are called erythema migrans.
During the advanced stage of chronic atrophic acrodermatitis, there is marked fibrosis, sclerosis, and atrophy. As a result, the skin becomes prominently wrinkled and loose, causing hair loss. Some people may find prominent sclerotic patches and fibrotic bands over the tibia or ulna, called pretibial bands and ulnar bands, respectively. ACA is known to cause problems in the peripheral nervous system, such as allodynia, the experience of pain with normal, non-painful stimuli. Other people experience persistent or intermittent pain in their extremities, and these problems are classified as peripheral neuropathies.
To diagnose chronic atrophic acrodermatitis, confirmation of the presence of skin lesions and tissue-paper-like skin is needed, along with serologic testing and a skin biopsy. Blood drawing is needed to determine the serum immunoglobulin G (IgG) level, which is elevated most of the time. Biopsy of early skin lesions of ACA shows the presence of inflammatory cells in the dermis along with lymphocytes and plasma cells, a loss of rete ridges, and a reduction in elastic fibers, as well as cell size and number. Dilation or enlargement of blood vessels and formation of vacuoles that resemble fat cells may occur. There is also fibrosis, indicated by an increase in the number of collagen-producing cells called fibroblasts, collagen bundles, and sclerosis, or the formation of thick bundles of collagen.
Chronic atrophic acrodermatitis is best treated in its early inflammatory phase. The agent is an infectious spirochete, so the primary treatment of this condition includes doxycycline or penicillin for four weeks. When chronic atrophic acrodermatitis is developed in its advanced stage, it can be difficult to reverse the atrophy and limitation of movement of the upper and lower extremities. In addition to antibiotic treatment, people with chronic atrophy should undergo rehabilitation therapy.
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