Acrodermatitis chronica atrophicans: clinical and microbiological characteristics of a cohort of 693 Slovenian patients

Abstract

Background. Information on large groups of patients with acrodermatitis chronica atrophicans (ACA) is limited.Methods. We assessed clinical and microbiological characteristics of patients with ACA diagnosed at a single medical centre and compared findings in periods 1991-2004 vs. 2005-2018. The cohort is representative of Slovenian ACA patients.Results. We assessed 693 patients: 461 females and 232 males, with median age of 64 years. Median duration of ACA before diagnosis was 12 months. In all but 2 patients, the skin lesions were located on extremities, more often on the lower (70.0%) than the upper (45.2%), bilaterally in 42.4%. Reddish-blue discoloration, swelling, thinning and wrinkling of skin were present in 95.2%, 28.1%, 46.4% and 20.5% of patients, respectively. Overall, 64.4% of patients reported constitutional symptoms, 23.1% had local symptoms, and 20.8% had symptoms/signs of peripheral neuropathy. Nodules, arthritis, joint deformity, muscle atrophy and paresis were rare (<3%). Borreliae were isolated from 200/664 (30.1%) skin samples; 92.8% were Borrelia afzelii. B. garinii and B. burgdorferi s.s. were more often isolated from the skin of male patients (OR = 4.17) and from those with arthropathy (OR = 11.74). Patients included in the more recent period were older, complained less often of constitutional symptoms but more often of local symptoms, and more often had local swelling but less often skin atrophy and bilateral involvement, probably as a consequence of earlier diagnosis.Conclusions. ACA, typically caused by B. afzelii, usually affects older women. Clinical presentation depends on the duration of illness and probably on the Borrelia species causing the disease.

Comparison of Lyme Disease in the United States and Europe

“…It starts with reddish-blue discoloration and swelling of the skin (an inflammatory phase), which slowly enlarges and, if untreated, is followed by atrophic changes several months to years later. For some patients, ACA was known to have been preceded by an earlier manifestation of Lyme disease, such as erythema Risk depends on the geographic area; the most common co-infection is tick-borne encephalitis migrans (18). The apparent explanation for the absence of these manifestations in the United States is that these skin infections are principally caused by B. afzelii (Table 3).…”

Discrepancy between IDSA and ESGBOR in Lyme disease: Individual participant meta‐analysis in Türkiye

“…For example, although ACA is described as the most common late disease manifestation (Mullegger, 2004; Steere et al., 2016), it is rare in the US and merely endemic in certain parts of Europe (DiCaudo et al., 1994). B. afzelii has been found to be most abundant in biopsied patients with ACA (Brandt et al., 2015; Ogrinc et al., 2021; Rijpkema et al., 1997), but the exact prevalence of B. afzelii is not known in Türkiye. In this study, we could only identify one reported case of ACA.…”

Primary perineuritis, a rare but treatable neuropathy: Review of perineurial anatomy, clinicopathological features, and differential diagnosis

“…131 Some 21%-64% of patients with ACA have a polyneuropathy, which is distal-predominant, symmetric or asymmetric, and primarily sensory. [132][133][134][135] This neuropathy is the only manifestation of neuroborreliosis unassociated with meningitis. Nerve biopsies in neuroborreliosis-related neuropathies reveal mononuclear perivascular inflammation in the epineurium, perineurium, and endoneurium.…”

“…In Europe, 1% of patients develop a late cutaneous manifestation: acrodermatitis chronica atrophicans (ACA) 131 . Some 21%–64% of patients with ACA have a polyneuropathy, which is distal‐predominant, symmetric or asymmetric, and primarily sensory 132–135 . This neuropathy is the only manifestation of neuroborreliosis unassociated with meningitis.…”