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Keywords = acrodermatitis chronica atrophicans

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12 pages, 491 KB  
Article
Edema as a Key Presentation of Acrodermatitis Chronica Atrophicans: A Retrospective Cohort Study from a Tertiary Setting in Denmark 2017–2025
by Otto Scharff El-Nasser, Helene Mens, Nanna Skaarup Andersen, Christoffer Valdemar Nissen and Anne-Mette Lebech
Diagnostics 2026, 16(9), 1394; https://doi.org/10.3390/diagnostics16091394 - 5 May 2026
Viewed by 303
Abstract
Background/Objectives: Acrodermatitis chronica atrophicans (ACA), a late cutaneous manifestation of Lyme borreliosis, presents with a broad clinical spectrum. Most commonly, a characteristic bluish-red patchy rash, but it can also appear as unilateral limb swelling. This study aimed to characterize the clinical manifestations, diagnostic [...] Read more.
Background/Objectives: Acrodermatitis chronica atrophicans (ACA), a late cutaneous manifestation of Lyme borreliosis, presents with a broad clinical spectrum. Most commonly, a characteristic bluish-red patchy rash, but it can also appear as unilateral limb swelling. This study aimed to characterize the clinical manifestations, diagnostic workup, and outcomes of patients with ACA in a tertiary setting in Denmark. Methods: Retrospective cohort study including all patients diagnosed with ACA at Copenhagen University Hospital-Rigshospitalet between 2017 and 2025. Results: Forty patients were included (median age 57 years; 63% female), with a median BMI of 24.5 [range 15.6–36.3]. Symptom duration was long (median 1 year). All patients presented with a skin rash. The most common location was the lower extremity, 26/40 (65%). Local edema and neuropathic pain were common (20/40) 50% and (23/40) 55%, respectively. A total of 13/40 patients underwent lymphoscintigraphy, which was deemed pathological in 7/13 (54%). The patients presenting with edema underwent significantly more imaging procedures, median 3 (range 1–5) vs. 0 (range 0–2), p < 0.005; they were younger, median age 49 years (range 17–76) vs. median 65 (range 30–81), p = 0.03; but did not differ in BMI, median 26.6 (range 19.0–36.2) versus median 23.8 (range 15.6–36.3), p = 0.48. All patients were Borrelia burgdorferi (Bb) IgG seropositive. Borrelia-specific PCR was positive in 6/13 (46%). Histopathology supported the diagnosis in 19/20 (95%). Clinical evaluation of the treatment response at 3 months was good in 33/40 (83%). Conclusions: Edema/swelling due to lymphatic obstruction is a common presentation of ACA in the tertiary setting, resulting in extensive diagnostic workup. The condition is associated with younger age but not BMI, sex, or immunodeficiency. Raised awareness and earlier testing for Bb IgG in serum seem warranted. Full article
(This article belongs to the Section Clinical Diagnosis and Prognosis)
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10 pages, 1421 KB  
Case Report
Acrodermatitis Chronica Atrophicans in a Patient with Pulmonary Sarcoidosis: Case Report and Literature Review
by Simona Roxana Georgescu, Alexandra Florentina Dobrescu, Ela Ghiță, Iulia Maria Teodora Leulescu and Mircea Tampa
J. Mind Med. Sci. 2025, 12(1), 27; https://doi.org/10.3390/jmms12010027 - 3 May 2025
Viewed by 3651
Abstract
Introduction: Acrodermatitis chronica atrophicans (ACA) is a late-stage cutaneous manifestation of Lyme borreliosis, primarily caused by Borrelia afzelii. It mainly affects the distal limbs and leads to progressive skin atrophy. Unlike other Lyme disease rashes, ACA does not resolve on its own [...] Read more.
Introduction: Acrodermatitis chronica atrophicans (ACA) is a late-stage cutaneous manifestation of Lyme borreliosis, primarily caused by Borrelia afzelii. It mainly affects the distal limbs and leads to progressive skin atrophy. Unlike other Lyme disease rashes, ACA does not resolve on its own and can worsen into severe atrophy and fibrosis if left untreated. Diagnosing ACA can be difficult due to its delayed onset and subtle symptoms, requiring clinical evaluation, multiple blood tests, and skin biopsy. Case presentation: We present the case of a 48-year-old female with a history of pulmonary sarcoidosis who presented to our clinic with multiple erythemato-violaceous patches over her left lower leg and was initially misdiagnosed with venous insufficiency. Histopathological and serological analyses confirmed ACA in its inflammatory phase. The patient responded well to a 30-day course of doxycycline, achieving complete resolution. This report underscores the importance of considering ACA in differential diagnoses and provides a comprehensive review of its pathogenesis, clinical progression, histopathological features, and epidemiology. Conclusions: This case emphasizes the need to consider acrodermatitis chronica atrophicans (ACA) in the differential diagnosis of chronic skin lesions. Clinicians should maintain a high index of suspicion for ACA, particularly in atypical presentations. When the diagnosis is uncertain but clinical suspicion persists, skin biopsy is recommended for histopathologic confirmation. Early diagnosis and appropriate antibiotic therapy are essential to prevent disease progression and irreversible cutaneous atrophy. Accurate diagnosis and effective management require a multidisciplinary approach, involving close collaboration between dermatologists, pathologists, and infectious disease specialists. Full article
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9 pages, 2639 KB  
Case Report
Unusual Presentation of Acrodermatitis Chronica Atrophicans Resulting in Delay of Diagnosis and Inappropriate Treatment in Three Cases
by Thilo Gambichler, Rim Jridi, Heinz-Wolfram Bernd, Andrea von Stemm and Stefanie Boms
Dermato 2024, 4(2), 37-45; https://doi.org/10.3390/dermato4020005 - 2 May 2024
Cited by 6 | Viewed by 5554
Abstract
Acrodermatitis chronica atrophicans (ACA) is not an infrequent condition in Europe. However, the characteristic skin lesions are often confused by non-dermatologists with other conditions. We report three unusual cases in which we made a definitive diagnosis of ACA complicated by cutaneous marginal zone [...] Read more.
Acrodermatitis chronica atrophicans (ACA) is not an infrequent condition in Europe. However, the characteristic skin lesions are often confused by non-dermatologists with other conditions. We report three unusual cases in which we made a definitive diagnosis of ACA complicated by cutaneous marginal zone lymphoma, juxta-articular fibrotic nodules, or bilateral sensory polyneuropathy. In all cases, correct diagnosis and adequate treatment was delayed over a period of at least 12 months. We initiated systemic antibiotics resulting in full recovery in these patients. The present case reports underscore that ACA may be associated with unusual clinical presentation which potentially result in delay of correct diagnosis and treatment. Hence, ACA diagnosis may be considerably delayed leading to inappropriate therapy exposure, prolonged patients’ suffering, and causing unnecessary cost. Thus, physicians who are not familiar with skin conditions should seek a timely dermatologist consultation. Full article
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21 pages, 853 KB  
Review
Post-Inflammatory Hypopigmentation: Review of the Etiology, Clinical Manifestations, and Treatment Options
by Medha Rao, Katherine Young, Ladonya Jackson-Cowan, Arianne Kourosh and Nicholas Theodosakis
J. Clin. Med. 2023, 12(3), 1243; https://doi.org/10.3390/jcm12031243 - 3 Feb 2023
Cited by 31 | Viewed by 24543
Abstract
Post-inflammatory hypopigmentation is a common acquired pigmentary disorder that is more prominent in skin of color, leading to great cosmetic and psychosocial implications. Often, a diagnosis with a pigmentary disorder can negatively impact an individual’s health-related quality of life and may result in [...] Read more.
Post-inflammatory hypopigmentation is a common acquired pigmentary disorder that is more prominent in skin of color, leading to great cosmetic and psychosocial implications. Often, a diagnosis with a pigmentary disorder can negatively impact an individual’s health-related quality of life and may result in stigma. Although most cases of post-inflammatory hypopigmentation resolve spontaneously over time, a systematic diagnostic approach can help with identifying the underlying etiology and informing treatment strategies. It can be due to cutaneous inflammation, sequelae of inflammatory or infectious dermatoses, or dermatologic procedures. Therefore, a thorough understanding of the epidemiology, patient history, physical exam findings, and clinical features of post-inflammatory hypopigmentation phenomenon can explain the primary cause to providers and allow for patient education. It is also important to understand the various therapeutic approaches available and the efficacy of these options, which will inform providers to choose the appropriate therapy for patients. Although algorithms exist for classifying acquired disorders of hypopigmentation, there are no established algorithms for the diagnosis and treatment of post-inflammatory hypopigmentation, which warrants further exploration and discourse. Full article
(This article belongs to the Special Issue Skin Pigmentation Disorders: Clinical Management and Treatment)
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11 pages, 1849 KB  
Case Report
Cutaneous Manifestations of Lyme Borreliosis in Children—A Case Series and Review
by Agnieszka Myszkowska-Torz, Mateusz Tomaszewski, Michał Kotowski, Cezary Witczak, Magdalena Figlerowicz and Katarzyna Mazur-Melewska
Life 2023, 13(1), 72; https://doi.org/10.3390/life13010072 - 27 Dec 2022
Cited by 8 | Viewed by 19552
Abstract
The occurrence of skin lesions is the earliest symptom of Lyme disease, and the diagnosis of these lesions and appropriate treatment may prevent complications of the disease, which are mainly neurological. The cutaneous presentation in borreliosis is heterogeneous. There are typical lesions that [...] Read more.
The occurrence of skin lesions is the earliest symptom of Lyme disease, and the diagnosis of these lesions and appropriate treatment may prevent complications of the disease, which are mainly neurological. The cutaneous presentation in borreliosis is heterogeneous. There are typical lesions that constitute the basis for the diagnosis of Lyme disease, and atypical ones, which cause significant diagnostic difficulties especially when the patient does not remember the tick bite. This study aims to describe the heterogeneous skin symptoms of Lyme borreliosis, as well as offer a practical approach for the recognition of the disease. Based on pediatric cases from clinical practice, rare cutaneous presentations of Lyme disease at various stages of illness and therapy are presented. Diagnostic recommendations for recognizing individual forms are discussed. Full article
(This article belongs to the Special Issue Lyme Disease—An Incompletely Solved Puzzle)
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17 pages, 2150 KB  
Article
Presentation of Acrodermatitis Chronica Atrophicans Rashes on Lyme Disease Patients in Canada
by John D. Scott
Healthcare 2020, 8(2), 157; https://doi.org/10.3390/healthcare8020157 - 4 Jun 2020
Cited by 10 | Viewed by 31024
Abstract
Lyme disease (Lyme borreliosis) is a complex multisystem illness with varying clinical manifestations. This tick-borne zoonosis is caused by the spirochetal bacterium, Borrelia burgdorferi sensu lato (Bbsl) and, worldwide, presents with at least 20 different types of rashes. Certain cutaneous rashes are inherently [...] Read more.
Lyme disease (Lyme borreliosis) is a complex multisystem illness with varying clinical manifestations. This tick-borne zoonosis is caused by the spirochetal bacterium, Borrelia burgdorferi sensu lato (Bbsl) and, worldwide, presents with at least 20 different types of rashes. Certain cutaneous rashes are inherently interconnected to various stages of Lyme disease. In this study, five Canadian Lyme disease patients from a multi-age range presented various phases of the acrodermatitis chronica atrophicans (ACA) rash. In each case of ACA, the underlying etiological pathogen was the Lyme disease spirochete. Although ACA rashes are normally found on the lower extremities, this study illustrates that ACA rashes are not directly correlated with a tick bite, geographic area, age, Bbsl genospecies, exercise, or any given surface area of the body. Case 4 provides confirmation for an ACA rash and gestational Lyme disease. One patient (Case 5) puts forth a Bbsl and Bartonella sp. co-infection with a complex ACA rash. This study documents ACA rashes on Lyme disease patients for the first time in Canada. Full article
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