Acrodermatitis chronica atrophicans overview: Difference between revisions

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{{CMG}}; {{AE}} {{RT}}
 
{{CMG}}; {{AE}} {{Anahita}} ; {{RT}}
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{{ACA}}


==Overview==
==Overview==
Acrodermatitis chronica atrophicans is a [[skin rash]] indicative of the third or late stage of European [[Lyme Disease|Lyme borreliosis]]. It is a [[dermatology|dermatological]] condition that takes a chronically progressive course and finally leads to a widespread [[atrophy]] of the [[skin]]. Involvement of the [[peripheral nervous system]] is often observed, specifically [[polyneuropathy]].
First record of [[acrodermatitis chronica atrophicans]] was made in 1883 in Breslau, Germany, where a [[physician]] named Alfred Buchwald first delineated it. [[Acrodermatitis chronica atrophicans]] is one of the tertiary presentations of [[Lyme disease|European lyme borreliosis]] with [[Borrelia|Borrelia afzelii]] known as the most predominant responsible [[microorganism]]. [[Transmission (medicine)|Transmission]] of this [[infection]] probably occur via [[Ixodes scapularis|ixodes tick]] (such as [[Ixodes scapularis|Ixodes ricinus]]), [[mosquito]] and [[fly|horsefly]] [[bite]]. These [[vectors]] themselves get [[infection|infected]] by feeding on an [[infection|infected]] animal reservoir. [[immune system|Immune reaction]] against [[borrelia]] leads to infiltration of [[T cell|CD3+]] and [[T cell|CD4+ cells]] in the [[dermis]]. Some conditions such as [[Lyme disease|lymphocytic meningoradiculitis]], [[Lichen sclerosus|lichen sclerosus et atrophicus]], [[morphea]] and other [[tick]] borne [[diseases]] have been associated with [[acrodermatitis chronica atrophicans]]. Thinning of [[skin]], visible [[vein|veins]], [[edema|swelling]] and [[wrinkles]] are some of the features can be noticed on [[gross pathology]]. [[Microscopy|Light]] and [[Electron microscope|electron microscopic study]] of the [[skin]] [[biopsy]] shows degeneration of the [[Descemet's membrane|elastica]] and [[collagen]] fibers. [[Acrodermatitis chronica atrophicans]] must be differentiated from [[Chronic (medical)|chronic]] [[venous insufficiency]], [[Chronic (medical)|chronic]] [[Ischemia|arterial insufficiency]], [[Superficial (human anatomy)|superficial]] [[thrombophlebitis]], [[frostbite]], [[morphea]], and [[granuloma annulare]]. [[Acrodermatitis chronica atrophicans]] is a rare [[disease]]. The [[prevalence]] of [[acrodermatitis chronica atrophicans]] is estimated to include 10% of cases with [[lyme disease]] in Europe. The [[incidence]] of [[acrodermatitis chronica atrophicans]] increases with [[aging|age]]. [[Acrodermatitis chronica atrophicans]] affects [[female|women]] more than [[male|men]] and the majority of [[acrodermatitis chronica atrophicans]] cases are reported in northern, central and eastern Europe. Common [[risk factors]] in the development of [[acrodermatitis chronica atrophicans]] include [[tick]] exposure, [[female]] gender and residents of northern, central and eastern Europe. The course of [[acrodermatitis chronica atrophicans]] is chronic and could lasts for several years and it can progress slowly overtime. In first phase ([[inflammation|the inflammatory phase]]) [[skin]] changes appear as blue and red discoloration with boggy infiltration. These [[inflammation|inflammatory]] [[skin]] lesions can become [[atrophy|atrophic]] without [[treatment]] ([[atrophy|atrophic phase]]). Superimposed [[Bacteria|bacterial]] [[infection]], sclerotic [[skin]] changes, [[Cancer|malignancies]], [[arthropathy]] and [[peripheral neuropathy]] are some of the common [[Complication (medicine)|complications]] of [[acrodermatitis chronica atrophicansis]]. The general [[pognosis]] is good with proper and rapid [[treatment]] in [[inflammation#Acute inflammation|acute inflammatory]] stage of [[acrodermatitis chronica atrophicans]], nevertheless late [[treatment]] can cause some irreversible changes. [[Skin]] [[physical examination|examination]] of [[acrodermatitis chronica atrophicans]]'s [[patients]] include blue, [[Erythema|red]] or brown discoloration, [[hypopigmentation]], [[Induration|indurated]] [[Plaque|plaques]] and [[wrinkles]]. High anti-[[Spirochaete|spirochetal]] [[antibody]] levels (such as [[Immunoglobulin G|IgG]], [[Immunoglobulin G|IgM]] and [[Immunoglobulin A|IgA]]) has been detected at indirect [[immunofluorescence]] and [[enzyme linked immunosorbent assay (ELISA)]]. [[Antibiotic]] [[therapy]] is recommended in [[patients]] with [[acrodermatitis chronica atrophicans]]. Up to four weeks [[treatment]] with [[antibiotics]] such as [[amoxicillin]], [[doxycycline]], [[ceftriaxone]], [[cefotaxime]] and [[Penicillin|penicillin G]] has been recommended for [[acrodermatitis chronica atrophicans]]'s [[treatment]]. Since [[Transmission (medicine)|transmission]] of [[borrelia]] [[infection]] occurs by [[ticks]], [[mosquitos]] and [[fly|horse flies]] [[bites]], [[Prevention (medical)|primary prevention]] could be achieved by [[bite]] avoidance.


==Historical Perspective==
==Historical Perspective==
The first record of ACA was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it.  [[Herxheimer reaction|Herxheimer]] and Hartmann described it in 1902 as a "tissue paper" like [[cutaneous]] atrophy.
 
*The First record of [[acrodermatitis chronica atrophicans]] was made in 1883 in Breslau, Germany, where a [[physician]] named Alfred Buchwald first delineated it.<ref name="pmid33085436">{{cite journal| author=| title=StatPearls | journal= | year= 2021 | volume= | issue=  | pages=  | pmid=33085436 | doi= | pmc= | url= }}</ref>
*Later in 1902 Herxheimer and Hartmann described it as a "tissue paper" like [[cutaneous]] [[atrophy]] and there were first [[physicians]] that came up with [[acrodermatitis chronica atrophicans]]'s name. They described the biphasic manner of this [[disease]] by demonstrating both [[inflammation|inflammatory]] and [[atrophy|atrophic phases]] of it.<ref name="Buchwald1883">{{cite journal|last1=Buchwald|first1=A.|title=Ein Fall von diffuser idiopathischer Haut-Atrophie|journal=Vierteljahresschrift für Dermatologie und Syphilis|volume=10|issue=1|year=1883|pages=553–556|issn=0340-3696|doi=10.1007/BF01833474}}</ref>
*In 1950s the possibility of [[human]] to [[human]] [[Transmission (medicine)|transmission]] was discussed. For the first time in 1984, [[borrelia]] was discovered as the responsible [[etiology]] of [[acrodermatitis chronica atrophicans]].
 
==Pathophysiology==
 
*[[Acrodermatitis chronica atrophicans]] is one of the tertiary presentations of [[Lyme disease|European Lyme borreliosis]] with [[Borrelia|Borrelia afzelii]] known as the most predominant responsible [[microorganism]].
*Nevertheless other [[borrelia]] species such as [[Borrelia|borrelia garinii]] and [[borrelia burgdorferi]] ([[Borrelia burgdorferi|B. burgdorferi sensu lato]]) have been also detected in [[acrodermatitis chronica atrophicans]] [[patients]].
*[[Transmission (medicine)|Transmission]] of this [[infection]] probably occur via [[Ixodes scapularis|ixodes tick]] (such as [[Ixodes scapularis|Ixodes ricinus]]), [[mosquito]] and [[fly|horsefly]] [[bite]]. These [[vectors]] themselves get [[infection|infected]] by feeding on an [[infection|infected]] animal reservoir.
*Development of various [[symptoms]] in this [[disease]] is a result of [[Chronic (medical)|chronic]] [[T cell]] mediated reaction of [[immune system]] against [[borrelia]].<ref name="pmid26156537">{{cite journal| author=Brandt FC, Ertas B, Falk TM, Metze D, Böer-Auer A| title=Histopathology and immunophenotype of acrodermatitis chronica atrophicans correlated with ospA and ospC genotypes of Borrelia species. | journal=J Cutan Pathol | year= 2015 | volume= 42 | issue= 10 | pages= 674-92 | pmid=26156537 | doi=10.1111/cup.12550 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26156537  }}</ref>
*This [[immune system|immune reaction]] leads to infiltration of [[T cell|CD3+]] and [[T cell|CD4+ cells]] in the [[dermis]]. [[Borrelia]] is capable of attaching to the [[extracellular matrix]] proteins (such as [[glycosaminoglycan]], [[fibronectin]] and [[decorin]] proteoglycan) which eventually leads to [[Metalloproteinases (MMPs)|metalloproteases]] activation and [[extracellular matrix]] degradation. [[inflammation|Pro-inflammatory]] [[cytokine|cytokines]], such as [[tumor necrosis factor alpha]] and [[interleukin-4]], have been detected in [[skin]] [[biopsy|biopsies]].<ref name="BrandtErtas2015">{{cite journal|last1=Brandt|first1=Friederike C.|last2=Ertas|first2=Beyhan|last3=Falk|first3=Thomas M.|last4=Metze|first4=Dieter|last5=Böer-Auer|first5=Almut|title=Histopathology and immunophenotype of acrodermatitis chronica atrophicans correlated withospAandospCgenotypes ofBorreliaspecies|journal=Journal of Cutaneous Pathology|volume=42|issue=10|year=2015|pages=674–692|issn=03036987|doi=10.1111/cup.12550}}</ref>
*There is no known [[gene]] responsible in [[pathophysiology]] of [[acrodermatitis chronica atrophicans]] [[disease]]. Some conditions such as [[Lyme disease|lymphocytic meningoradiculitis]], [[Lichen sclerosus|lichen sclerosus et atrophicus]], [[morphea]] and other [[tick]] borne [[diseases]] have been associated with [[acrodermatitis chronica atrophicans]]. Thinning of [[skin]], visible [[vein|veins]], [[edema|swelling]] and [[wrinkles]] are some of the features can be noticed on [[gross pathology]].
*[[Microscopy|Light]] and [[Electron microscope|electron microscopic study]] of the [[skin]] [[biopsy]] shows degeneration of the [[Descemet's membrane|elastica]] and [[collagen]] fibers. Thinning of [[dermis]] and [[epidermis]], [[Pigment|pigmented]] [[stratum germinativum]], [[dermis|dermal]] [[blood vessels]] dilation and perivascular [[plasma cell]] infiltration are some of the findings on [[pathology|microscopic pathology]].<ref name="pmid9602229">{{cite journal| author=Brehmer-Andersson E, Hovmark A, Asbrink E| title=Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases. | journal=Acta Derm Venereol | year= 1998 | volume= 78 | issue= 3 | pages= 207-13 | pmid=9602229 | doi=10.1080/000155598441558 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9602229  }}</ref>
 
==Causes==
 
*This progressive [[skin]] disorder is due to the effect of [[Chronic (medical)|chronic]] [[infection]] with the [[spirochete]] [[borrelia|borrelia afzelii]], which is the predominant cause of [[acrodermatitis chronica atrophicans]].
*However [[borrelia|borrelia afzelii]] is not the exclusive [[etiology|etiologic]] agent of [[acrodermatitis chronica atrophicans]] and other [[microorganisms]] such as [[borrelia|borrelia garinii]] and [[borrelia|borrelia burgdorferi]] have also been detected.
 
==Differentiating Acrodermatitis Chronica Atrophicans from Other Diseases==
 
*[[Acrodermatitis chronica atrophicans]] must be differentiated from [[Chronic (medical)|chronic]] [[venous insufficiency]], [[Chronic (medical)|chronic]] [[Ischemia|arterial insufficiency]], [[Superficial (human anatomy)|superficial]] [[thrombophlebitis]], [[frostbite]], [[morphea]], [[erysipelas]], [[acrocyanosis]] and [[granuloma annulare]].
 
==Epidemiology and Demographics==
 
*[[Acrodermatitis chronica atrophicans]] is a rare [[disease]]. The [[prevalence]] of [[acrodermatitis chronica atrophicans]] is estimated to include 10% of cases with [[lyme disease]] in Europe.<ref name="pmid15030123">{{cite journal| author=Christova I, Komitova R| title=Clinical and epidemiological features of Lyme borreliosis in Bulgaria. | journal=Wien Klin Wochenschr | year= 2004 | volume= 116 | issue= 1-2 | pages= 42-6 | pmid=15030123 | doi=10.1007/BF03040423 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15030123  }}</ref><ref name="pmid24550705">{{cite journal| author=Stinco G, Ruscio M, Bergamo S, Trotter D, Patrone P| title=Clinical features of 705 Borrelia burgdorferi seropositive patients in an endemic area of northern Italy. | journal=ScientificWorldJournal | year= 2014 | volume= 2014 | issue=  | pages= 414505 | pmid=24550705 | doi=10.1155/2014/414505 | pmc=3914583 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24550705  }}</ref>
*The [[incidence]] of [[acrodermatitis chronica atrophicans]] increases with [[aging|age]] and commonly affects individuals in range of 40 to 70 years old with a [[median]] of 64 years old. However there are few [[case report|case reports]] on [[child|children]] who are [[diagnosis|diagnosed]] with [[acrodermatitis chronica atrophicans]].
*[[Acrodermatitis chronica atrophicans]] affects [[female|women]] more than [[male|men]].
*The majority of [[acrodermatitis chronica atrophicans]] cases are reported in northern, central and eastern Europe (most commonly in countries bordering the Baltic Sea). Lately few cases of [[acrodermatitis chronica atrophicans]] have been reported in the United States and Canada.<ref name="pmid16282646">{{cite journal| author=Nygård K, Brantsaeter AB, Mehl R| title=Disseminated and chronic Lyme borreliosis in Norway, 1995 - 2004. | journal=Euro Surveill | year= 2005 | volume= 10 | issue= 10 | pages= 235-8 | pmid=16282646 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16282646  }}</ref>
 
==Risk Factors==
 
*Common [[risk factors]] in the development of [[acrodermatitis chronica atrophicans]] include:<ref name="pmid245507052">{{cite journal| author=Stinco G, Ruscio M, Bergamo S, Trotter D, Patrone P| title=Clinical features of 705 Borrelia burgdorferi seropositive patients in an endemic area of northern Italy. | journal=ScientificWorldJournal | year= 2014 | volume= 2014 | issue=  | pages= 414505 | pmid=24550705 | doi=10.1155/2014/414505 | pmc=3914583 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24550705  }}</ref>
**[[Tick]] exposure
**Female gender and residents of northern, central and eastern Europe.
 
==Natural History, Complications, and Prognosis==
 
*The course of [[acrodermatitis chronica atrophicans]] is chronic and could last for several years and it can progress slowly over time. It has been estimated that [[mean]] duration of [[acrodermatitis chronica atrophicans]] before [[diagnosis]] is approximately 12 months, based on a study. It usually start on one [[Limb (anatomy)|extremity]] and can spread and involve [[Extension (kinesiology)|extensor surfaces]] of the [[Limb (anatomy)|acral regions of limbs]]<ref name="pmid30429707">{{cite journal| author=Moniuszko-Malinowska A, Czupryna P, Dunaj J, Pancewicz S, Garkowski A, Kondrusik M | display-authors=etal| title=Acrodermatitis chronica atrophicans: various faces of the late form of Lyme borreliosis. | journal=Postepy Dermatol Alergol | year= 2018 | volume= 35 | issue= 5 | pages= 490-494 | pmid=30429707 | doi=10.5114/ada.2018.77240 | pmc=6232541 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30429707  }}</ref>.
*[[Acrodermatitis chronica atrophicans]] has a biphasic manner. In the first phase ([[inflammation|the inflammatory phase]]) [[skin]] changes appear as blue and red discoloration with boggy infiltration. These [[inflammation|inflammatory]] [[skin]] lesions can become [[atrophy|atrophic]] without [[treatment]] ([[atrophy|atrophic phase]]).
*Based on two studies, 55% and 66% of [[patients]] with [[acrodermatitis chronica atrophicans]] have at least one history of [[tick]] [[bite]], while others may never remember such an accident. One fifth of [[patients]] in a study experienced [[Lyme disease history and symptoms|erythema migrans]] 6 months to 8 years before [[acrodermatitis chronica atrophicans]] development.
*Superimposed [[Bacteria|bacterial]] [[infection]], sclerotic [[skin]] changes, [[Cancer|malignancies]], [[arthropathy]] and [[peripheral neuropathy]] are some of the common [[Complication (medicine)|complications]] of [[acrodermatitis chronica atrophicansis]]. In contrast to other [[skin]] manifestations of [[borrelia]] [[infection]], [[acrodermatitis chronica atrophicans]] doesn't heal without [[treatment]] and can lead to extensive [[atrophy]] of [[skin]] and limitations of [[Upper limb|upper]] and [[Leg|lower limb]] [[joint]] mobility.
*The general [[pognosis]] is good with proper and rapid [[treatment]] in [[inflammation#Acute inflammation|acute inflammatory]] stage of [[acrodermatitis chronica atrophicans]]. Nevertheless late [[treatment]] can cause some irreversible changes.<ref name="pmid3728879">{{cite journal| author=Asbrink E, Brehmer-Andersson E, Hovmark A| title=Acrodermatitis chronica atrophicans--a spirochetosis. Clinical and histopathological picture based on 32 patients; course and relationship to erythema chronicum migrans Afzelius. | journal=Am J Dermatopathol | year= 1986 | volume= 8 | issue= 3 | pages= 209-19 | pmid=3728879 | doi=10.1097/00000372-198606000-00005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3728879  }}</ref>
 
==Diagnosis==
===History and Symptoms===
 
*History of [[tick bite]], [[Lyme disease|erythema migrans]] or other [[symptoms]] of [[lyme disease]], and [[rheumatology|rheumatological]] [[symptoms]] have been presented in [[patients]] with [[acrodermatitis chronica atrophicans]]. Since there could be several years between the [[tick]] [[bite]] and development of [[skin]] lesions, absence of [[tick]] [[bite]] in [[patients]]' history never exclude the [[diagnosis]].
*[[Symptoms]] and different forms of [[skin]] involvement in [[acrodermatitis chronica atrophicans]] are dependent to duration of the [[disease]]. [[Symptoms]], such as sclerotic [[skin]] changes, [[pain]] and burning, [[edema]] and [[B symptoms|constitutional symptoms]] have been observed in [[acrodermatitis chronica atrophicans]].<ref name="pmid304297072">{{cite journal| author=Moniuszko-Malinowska A, Czupryna P, Dunaj J, Pancewicz S, Garkowski A, Kondrusik M | display-authors=etal| title=Acrodermatitis chronica atrophicans: various faces of the late form of Lyme borreliosis. | journal=Postepy Dermatol Alergol | year= 2018 | volume= 35 | issue= 5 | pages= 490-494 | pmid=30429707 | doi=10.5114/ada.2018.77240 | pmc=6232541 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30429707  }}</ref>
*Half of [[patients]] with [[acrodermatitis chronica atrophicans]] experience [[symptoms]] of [[peripheral neuropathy]], such as [[paresthesia]] and [[hypesthesia]]. [[Symptoms]] of [[peripheral neuropathy]] can occur at the exact site of [[acrodermatitis chronica atrophicans]]'s lesion or at other sites.<ref name="pmid2847621">{{cite journal| author=Kristoferitsch W, Sluga E, Graf M, Partsch H, Neumann R, Stanek G | display-authors=etal| title=Neuropathy associated with acrodermatitis chronica atrophicans. Clinical and morphological features. | journal=Ann N Y Acad Sci | year= 1988 | volume= 539 | issue=  | pages= 35-45 | pmid=2847621 | doi=10.1111/j.1749-6632.1988.tb31836.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2847621  }}</ref>
*Involvement of [[leg|lower limb]] is more common compared to the [[upper limb]]. In some cases episodic [[knee]] [[joint]] [[effusion]] has been observed.
 
===Physical Examination===
 
*[[Skin]] [[physical examination|examination]] of [[acrodermatitis chronica atrophicans]]'s [[patients]] include blue, [[Erythema|red]] or brown discoloration, [[hypopigmentation]], [[Induration|indurated]] [[Plaque|plaques]] and [[wrinkles]], thinning and shining of involved [[skin]].<ref name="pmid7873838">{{cite journal| author=Müller DE, Itin PH, Büchner SA, Rufli T| title=Acrodermatitis chronica atrophicans involving the face. Evidence for Borrelia burgdorferi infection confirmed by DNA amplification. | journal=Dermatology | year= 1994 | volume= 189 | issue= 4 | pages= 430-1 | pmid=7873838 | doi=10.1159/000246901 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7873838  }}</ref>
*Readily visible [[veins]], [[edema]], [[ulcers]] and peeling are usually found. Although the most common location of these [[skin]] changes are observed on [[Limb (anatomy)|limbs]], there are some cases with [[face|facial]] and [[abdomen|abdominal]] involvement.
*[[neuropathy|Peripheral neuropathy]] develops in 50% of [[patients]]. [[Physical examination]] of some [[patients]] may reveal [[Ulna|ulnar bands]]. Moreover [[Fibrosis|fibrotic]] [[Nodule (medicine)|nodules]] could be seen on [[bone|bony]] prominences, such as [[tibia]] or [[ulna]].
 
===Laboratory Findings===
 
*High anti-[[Spirochaete|spirochetal]] [[antibody]] levels (such as [[Immunoglobulin G|IgG]], [[Immunoglobulin G|IgM]] and [[Immunoglobulin A|IgA]]) has been detected at indirect [[immunofluorescence]] and [[enzyme linked immunosorbent assay (ELISA)]].<ref name="pmid27976670">{{cite journal| author=Steere AC, Strle F, Wormser GP, Hu LT, Branda JA, Hovius JW | display-authors=etal| title=Lyme borreliosis. | journal=Nat Rev Dis Primers | year= 2016 | volume= 2 | issue=  | pages= 16090 | pmid=27976670 | doi=10.1038/nrdp.2016.90 | pmc=5539539 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27976670  }}</ref>
*Among various [[antigens]] in [[borrelia burgdorferi]], [[flagellum]] [[antigen]] is one of the recommended [[serology|serologic evaluation]] in [[Acrodermatitis chronica atrophicans|acrodermatitis chronica atrophicans]] [[patients]]. [[Diagnosis]] of [[Acrodermatitis chronica atrophicans|acrodermatitis chronica atrophicans]] can be excluded if the [[serology|serologic]] evaluataion is negative.<ref name="pmid23190290">{{cite journal| author=Ljøstad U, Mygland Å| title=Chronic Lyme; diagnostic and therapeutic challenges. | journal=Acta Neurol Scand Suppl | year= 2013 | volume=  | issue= 196 | pages= 38-47 | pmid=23190290 | doi=10.1111/ane.12048 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23190290  }}</ref><ref name="pmid20132258">{{cite journal| author=Stanek G, Fingerle V, Hunfeld KP, Jaulhac B, Kaiser R, Krause A | display-authors=etal| title=Lyme borreliosis: clinical case definitions for diagnosis and management in Europe. | journal=Clin Microbiol Infect | year= 2011 | volume= 17 | issue= 1 | pages= 69-79 | pmid=20132258 | doi=10.1111/j.1469-0691.2010.03175.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20132258  }}</ref>
*[[Borrelia]] itself has been found in some of the [[skin]] samples. When clinical presentations are not clear enough, [[biopsy]] and [[histology|histological]] evaluation can assist. Findings such as [[Plasma cell|plasma cells]], [[histiocytes]] and [[Lymphocyte|lymphocytic infiltration]] plus [[telangiectasia]] and thinning of [[dermis]] and [[epidermis]] are commonly found in [[skin]] [[biopsy|biopsies]].<ref name="Steere2001">{{cite journal|last1=Steere|first1=Allen C.|title=Lyme Disease|journal=New England Journal of Medicine|volume=345|issue=2|year=2001|pages=115–125|issn=0028-4793|doi=10.1056/NEJM200107123450207}}</ref>
 
===Other Diagnostic Studies===
 
*There are no other [[diagnosis|diagnostic studies]] associated with [[acrodermatitis chronica atrophicans]].


==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
Treatment of ACA consists of antibiotics including [[doxycycline]] and [[penicillin]] for up to four weeks in the acute case.
 
*[[Antibiotic]] [[therapy]] is recommended in [[patients]] with [[acrodermatitis chronica atrophicans]].
*Up to four weeks [[treatment]] with [[antibiotics]] such as [[amoxicillin]], [[doxycycline]], [[ceftriaxone]], [[cefotaxime]] and [[Penicillin|penicillin G]] has been recommended for [[acrodermatitis chronica atrophicans]]'s [[treatment]].
**Preferred regimen (1): [[Amoxicillin]] 500 to 1000 mg three times daily for 14 to 28 days<ref name="pmid18536243">{{cite journal| author=Flisiak R, Pancewicz S, Polish Society of Epidemiology and Infectious Diseases| title=[Diagnostics and treatment of Lyme borreliosis. Recommendations of Polish Society of Epidemiology and Infectious Diseases]. | journal=Przegl Epidemiol | year= 2008 | volume= 62 | issue= 1 | pages= 193-9 | pmid=18536243 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18536243  }}</ref><ref name="pmid26233093">{{cite journal| author=Pancewicz SA, Garlicki AM, Moniuszko-Malinowska A, Zajkowska J, Kondrusik M, Grygorczuk S | display-authors=etal| title=Diagnosis and treatment of tick-borne diseases recommendations of the Polish Society of Epidemiology and Infectious Diseases. | journal=Przegl Epidemiol | year= 2015 | volume= 69 | issue= 2 | pages= 309-16, 421-8 | pmid=26233093 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26233093  }}</ref>
**Preferred regimen (2): [[Doxycycline]] 100 mg twice daily or 200 mg once daily for 14 to 28 days
**Preferred regimen (3): [[Ceftriaxone]] (IV) 2000 mg every 24 hours for 14 to 28 days
**Preferred regimen (4): [[Cefotaxime]] (IV) 2000 mg every 8 hours for 14 to 28 days
**Preferred regimen (5): [[Penicillin G benzathine|Penicillin]] G (IV) 3 to 4 MU every 4 hours for 14 to 28 days
 
===Primary Prevention===
 
*Since [[Transmission (medicine)|transmission]] of [[borrelia]] [[infection]] occurs by [[ticks]], [[mosquitos]] and [[fly|horse flies]] [[bites]], [[Prevention (medical)|primary prevention]] could be achieved by [[bite]] avoidance.
*Instructions such as using insect repellants, avoiding [[tick]]-infested regions or wearing long sleeves and pants when necessary can help.
 
===Secondary Prevention===
 
*Proper [[antibiotic]] [[treatment]] of a [[patient]] who has been [[diagnosis|diagnosed]] with [[lyme disease]] can reduce the probability of [[acrodermatitis chronica atrophicans]].


==References==
==References==
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[[Category:Spirochaetes]]
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Latest revision as of 18:03, 23 June 2021


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2] ; Raviteja Guddeti, M.B.B.S. [3]

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Overview

First record of acrodermatitis chronica atrophicans was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it. Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis with Borrelia afzelii known as the most predominant responsible microorganism. Transmission of this infection probably occur via ixodes tick (such as Ixodes ricinus), mosquito and horsefly bite. These vectors themselves get infected by feeding on an infected animal reservoir. Immune reaction against borrelia leads to infiltration of CD3+ and CD4+ cells in the dermis. Some conditions such as lymphocytic meningoradiculitis, lichen sclerosus et atrophicus, morphea and other tick borne diseases have been associated with acrodermatitis chronica atrophicans. Thinning of skin, visible veins, swelling and wrinkles are some of the features can be noticed on gross pathology. Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers. Acrodermatitis chronica atrophicans must be differentiated from chronic venous insufficiency, chronic arterial insufficiency, superficial thrombophlebitis, frostbite, morphea, and granuloma annulare. Acrodermatitis chronica atrophicans is a rare disease. The prevalence of acrodermatitis chronica atrophicans is estimated to include 10% of cases with lyme disease in Europe. The incidence of acrodermatitis chronica atrophicans increases with age. Acrodermatitis chronica atrophicans affects women more than men and the majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe. Common risk factors in the development of acrodermatitis chronica atrophicans include tick exposure, female gender and residents of northern, central and eastern Europe. The course of acrodermatitis chronica atrophicans is chronic and could lasts for several years and it can progress slowly overtime. In first phase (the inflammatory phase) skin changes appear as blue and red discoloration with boggy infiltration. These inflammatory skin lesions can become atrophic without treatment (atrophic phase). Superimposed bacterial infection, sclerotic skin changes, malignancies, arthropathy and peripheral neuropathy are some of the common complications of acrodermatitis chronica atrophicansis. The general pognosis is good with proper and rapid treatment in acute inflammatory stage of acrodermatitis chronica atrophicans, nevertheless late treatment can cause some irreversible changes. Skin examination of acrodermatitis chronica atrophicans's patients include blue, red or brown discoloration, hypopigmentation, indurated plaques and wrinkles. High anti-spirochetal antibody levels (such as IgG, IgM and IgA) has been detected at indirect immunofluorescence and enzyme linked immunosorbent assay (ELISA). Antibiotic therapy is recommended in patients with acrodermatitis chronica atrophicans. Up to four weeks treatment with antibiotics such as amoxicillin, doxycycline, ceftriaxone, cefotaxime and penicillin G has been recommended for acrodermatitis chronica atrophicans's treatment. Since transmission of borrelia infection occurs by ticks, mosquitos and horse flies bites, primary prevention could be achieved by bite avoidance.

Historical Perspective

Pathophysiology

Causes

Differentiating Acrodermatitis Chronica Atrophicans from Other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Primary Prevention

Secondary Prevention

References

  1. ↑ "StatPearls". 2021. PMID 33085436 Check |pmid= value (help).
  2. ↑ Buchwald, A. (1883). "Ein Fall von diffuser idiopathischer Haut-Atrophie". Vierteljahresschrift für Dermatologie und Syphilis. 10 (1): 553–556. doi:10.1007/BF01833474. ISSN 0340-3696.
  3. ↑ Brandt FC, Ertas B, Falk TM, Metze D, Böer-Auer A (2015). "Histopathology and immunophenotype of acrodermatitis chronica atrophicans correlated with ospA and ospC genotypes of Borrelia species". J Cutan Pathol. 42 (10): 674–92. doi:10.1111/cup.12550. PMID 26156537.
  4. ↑ Brandt, Friederike C.; Ertas, Beyhan; Falk, Thomas M.; Metze, Dieter; Böer-Auer, Almut (2015). "Histopathology and immunophenotype of acrodermatitis chronica atrophicans correlated withospAandospCgenotypes ofBorreliaspecies". Journal of Cutaneous Pathology. 42 (10): 674–692. doi:10.1111/cup.12550. ISSN 0303-6987.
  5. ↑ Brehmer-Andersson E, Hovmark A, Asbrink E (1998). "Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases". Acta Derm Venereol. 78 (3): 207–13. doi:10.1080/000155598441558. PMID 9602229.
  6. ↑ Christova I, Komitova R (2004). "Clinical and epidemiological features of Lyme borreliosis in Bulgaria". Wien Klin Wochenschr. 116 (1–2): 42–6. doi:10.1007/BF03040423. PMID 15030123.
  7. ↑ Stinco G, Ruscio M, Bergamo S, Trotter D, Patrone P (2014). "Clinical features of 705 Borrelia burgdorferi seropositive patients in an endemic area of northern Italy". ScientificWorldJournal. 2014: 414505. doi:10.1155/2014/414505. PMC 3914583. PMID 24550705.
  8. ↑ Nygård K, Brantsaeter AB, Mehl R (2005). "Disseminated and chronic Lyme borreliosis in Norway, 1995 - 2004". Euro Surveill. 10 (10): 235–8. PMID 16282646.
  9. ↑ Stinco G, Ruscio M, Bergamo S, Trotter D, Patrone P (2014). "Clinical features of 705 Borrelia burgdorferi seropositive patients in an endemic area of northern Italy". ScientificWorldJournal. 2014: 414505. doi:10.1155/2014/414505. PMC 3914583. PMID 24550705.
  10. ↑ Moniuszko-Malinowska A, Czupryna P, Dunaj J, Pancewicz S, Garkowski A, Kondrusik M; et al. (2018). "Acrodermatitis chronica atrophicans: various faces of the late form of Lyme borreliosis". Postepy Dermatol Alergol. 35 (5): 490–494. doi:10.5114/ada.2018.77240. PMC 6232541. PMID 30429707.
  11. ↑ Asbrink E, Brehmer-Andersson E, Hovmark A (1986). "Acrodermatitis chronica atrophicans--a spirochetosis. Clinical and histopathological picture based on 32 patients; course and relationship to erythema chronicum migrans Afzelius". Am J Dermatopathol. 8 (3): 209–19. doi:10.1097/00000372-198606000-00005. PMID 3728879.
  12. ↑ Moniuszko-Malinowska A, Czupryna P, Dunaj J, Pancewicz S, Garkowski A, Kondrusik M; et al. (2018). "Acrodermatitis chronica atrophicans: various faces of the late form of Lyme borreliosis". Postepy Dermatol Alergol. 35 (5): 490–494. doi:10.5114/ada.2018.77240. PMC 6232541. PMID 30429707.
  13. ↑ Kristoferitsch W, Sluga E, Graf M, Partsch H, Neumann R, Stanek G; et al. (1988). "Neuropathy associated with acrodermatitis chronica atrophicans. Clinical and morphological features". Ann N Y Acad Sci. 539: 35–45. doi:10.1111/j.1749-6632.1988.tb31836.x. PMID 2847621.
  14. ↑ Müller DE, Itin PH, Büchner SA, Rufli T (1994). "Acrodermatitis chronica atrophicans involving the face. Evidence for Borrelia burgdorferi infection confirmed by DNA amplification". Dermatology. 189 (4): 430–1. doi:10.1159/000246901. PMID 7873838.
  15. ↑ Steere AC, Strle F, Wormser GP, Hu LT, Branda JA, Hovius JW; et al. (2016). "Lyme borreliosis". Nat Rev Dis Primers. 2: 16090. doi:10.1038/nrdp.2016.90. PMC 5539539. PMID 27976670.
  16. ↑ Ljøstad U, Mygland Å (2013). "Chronic Lyme; diagnostic and therapeutic challenges". Acta Neurol Scand Suppl (196): 38–47. doi:10.1111/ane.12048. PMID 23190290.
  17. ↑ Stanek G, Fingerle V, Hunfeld KP, Jaulhac B, Kaiser R, Krause A; et al. (2011). "Lyme borreliosis: clinical case definitions for diagnosis and management in Europe". Clin Microbiol Infect. 17 (1): 69–79. doi:10.1111/j.1469-0691.2010.03175.x. PMID 20132258.
  18. ↑ Steere, Allen C. (2001). "Lyme Disease". New England Journal of Medicine. 345 (2): 115–125. doi:10.1056/NEJM200107123450207. ISSN 0028-4793.
  19. ↑ Flisiak R, Pancewicz S, Polish Society of Epidemiology and Infectious Diseases (2008). "[Diagnostics and treatment of Lyme borreliosis. Recommendations of Polish Society of Epidemiology and Infectious Diseases]". Przegl Epidemiol. 62 (1): 193–9. PMID 18536243.
  20. ↑ Pancewicz SA, Garlicki AM, Moniuszko-Malinowska A, Zajkowska J, Kondrusik M, Grygorczuk S; et al. (2015). "Diagnosis and treatment of tick-borne diseases recommendations of the Polish Society of Epidemiology and Infectious Diseases". Przegl Epidemiol. 69 (2): 309–16, 421–8. PMID 26233093.

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