While few Lyme Disease symptoms are as slam-dunk diagnostic as an erythema migrans (EM) rash, there are a couple that should tip any doctor off when they appear: acrodermatitis chronica atrophicans (or ACA), which was one of the earliest recognized symptoms in Europe, and borrelial lymphocytoma (BL).
Like EM, both issues affect the skin, though the results are quite different. Now, admittedly, these particular Lyme symptoms mostly appear in European patients; but that’s not always the case. And besides, it’s not impossible to acquire LD during a European visit, so it’s worth knowing the symptomology.
In addition to the infamous Borrelia burgdorferi spirochetes that cause almost all American Lyme Disease cases (as well as many in Europe and elsewhere), the Borrelia genus includes about three dozen other member species, at least 11 of which cause LD in some form or another.
The species implicated in causing both ACA and BL is almost exclusively B. afzerii, though B. garinii may also be involved, and it’s possible that other Borrelia species can contribute to both conditions as well.
A number of European physicians described ACA in the medical literature as early as the 1880s. It’s a chronic Lyme symptom, presenting first as a bluish-red discoloration and swelling that persists for months. It causes the skin to permanently wrinkle as it progresses.
ACA is most common in the elderly. Infected skin comes to resemble thin, crumpled tissue or cigarette paper. The skin basically wastes away as the disease progresses, becoming dry and hairless, and often developing stiff (sclerotic) plaques in which connective tissue replaces the dermis.
Usually, ACA first appears on the backs of the hands or feet, although it can appear elsewhere and may spread to other parts of the body if left untreated. In extreme cases, the patient loses some mobility in their joints. ACA may also affect the peripheral nervous system, causing nerves to misfire painfully.
ACA requires treatment with intravenous antibiotics such as ceftriaxone, cefotaxime, or penicillin G for 12-28 days.
This condition results in the appearance of an ugly red or purplish lump somewhere on the body, most often on a sensitive area such as the earlobe, scrotum, or nipple. The condition is benign, in that it causes no direct damage to the body, unlike ACA.
BL is a form of cutaneous lymphoid hyperplasia, of which there are several types; most are not indicative of a Borrelia infection. However, if you suspect you have LD, you should take the sudden appearance of a lymphocytoma very seriously. It certainly warrants further testing.
A quick response may, in fact, be sufficient to destroy the actual Borrelia infection, since BL appears during the early disseminated stage of Lyme Disease. Most Lyme-literate doctors treat BL with an ordinary course of an antibiotic such as doxycycline.
Are You At Risk?
Yes, though the risk may be minimal. American strains of Borrelia usually don’t produce either ACA or BL as symptoms; however, you should never forget that Lyme is an international disease.
Not only is LD also common in Europe (where medical science first recognized it, actually), it’s starting to appear in Australia and elsewhere. The explanation for that is simple: when we humans shuffle ourselves and our goods around the globe, we take our pests and diseases with us.
Borrelia burgdorferi is already beginning to appear more often in Europe, possibly because of the accidental importation of infected ticks. B. afzerii and B. garinii may very well someday hitch a ride here and start spreading… in which case these unusual Lyme Disease symptoms won’t be unusual anymore.