Acrodermatitis chronica atrophicans pathophysiology
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
Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis. Borrelia afzelii is known as the most predominant responsible microorganism. Nevertheless, other borrelia species such as borrelia garinii and borrelia burgdorferi (B. burgdorferi sensu lato) have been also detected in acrodermatitis chronica atrophicans patients. 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. Development of various symptoms in this disease is a result of chronic T cell mediated reaction of immune system against borrelia. This immune reaction leads to infiltration of CD3+ and 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 metalloproteases activation and extracellular matrix degradation. Pro-inflammatory cytokines, such as tumor necrosis factor alpha and interleukin-4, have been detected in skin biopsies. There is no known gene responsible in pathophysiology of acrodermatitis chronica atrophicans disease. 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 that can be noticed on gross pathology. Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers. Thinning of dermis and epidermis, pigmented stratum germinativum, dermal blood vessels dilation and perivascular plasma cell infiltration are some of the findings on microscopic pathology.
Pathophysiology
Pathogenesis
- Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis.[1][2]
- This progressive skin process is due to the effect of chronic infection with the spirochete borrelia burgdorferi. Borrelia afzelii is the predominant pathophysiology, but may not be the exclusive, etiology of acrodermatitis chronica atrophicans.[3][4][5][6][1][2]
- Borrelia garinii, borrelia afzelii and borrelia burgdorferi (B. burgdorferi sensu lato), are all responsible and have been detected in acrodermatitis chronica atrophicans patients.[7][8]
- Based on numerous studies, majority of skin biopsies from acrodermatitis chronica atrophicans patients demonstrated borrelia afzelii.[8][7]
- 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.[9][3]
- Acrodermatitis chronica atrophicans development is the result of chronic T cell mediated reaction of immune system against borrelia. This immune reaction leads to infiltration of CD3+ and CD4+ cells in the dermis.[9]
- Borrelia is capable of attaching to the extracellular matrix proteins (such as glycosaminoglycan, fibronectin and decorin proteoglycan) which eventually leads to metalloproteases activation. When metalloproteases activate they degrade extracellular matrix.[9][10]
- High affinity of borrelia to collagen fibers explains how damage of connective tissue, fibrosis and dermal atrophy occur.[9][11][12]
- Fibrosis and collagen accumulation lead to formation of band-like hardness on extremities and may cause joint movement reduction as a consequent.[12]
- Based on a study which included acrodermatitis chronica atrophicans patients, expression of pro-inflammatory cytokines (such as tumor necrosis factor alpha and interleukin-4) have been detected in skin biopsies. In contrast to skin biopsies of erythema migrans which demonstrate high expression of interferon-gamma, skin biopsies of acrodermatitis chronica atrophicans lack this interferon. Since presence of interferon-gamma is related to control of the spirochetal infection, it's absence in acrodermatitis chronica atrophicans could explain the chronic manner of this disorder.[13]
Genetics
There is no known gene responsible in pathophysiology of acrodermatitis chronica atrophicans disease.
Associated Conditions
Conditions associated include:[14][4][15][16][17]
- Lymphocytic meningoradiculitis:
- Also known as bannwarth syndrome.
- Lymphocytic meningoradiculitis is a neurological disease which is also due to Borrelia burgdorferi infection and subsequent lyme disease.
- Symptoms such as radicular pain in cervical or lumbar regions and cranial nerve palsy (such as facial palsy) are common among patients.
- Lichen sclerosus et atrophicus
- Also called Lichen sclerosus.
- It appears as scleroderma-like skin lesions.
- It has been reported in 12% patients of a study done on 50 patients with acrodermatitis chronica atrophicans.
- Morphea
- Other tick borne diseases
Gross Pathology
- In the atrophy phase of the acrodermatitis chronica atrophicans skin appears transparent with easily seen veins on gross pathology.[8][18]
- The following list are some of the findings on gross pathology:[2]
Microscopic Pathology
- Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers.[19]
- A study that investigated skin biopsies under light and electron microscopes demonstrated osmiophilic materials around collagen fibres.[19]
- Findings from biopsies exhibit leukocytic infiltrations, plasma cells, histiocytes and telangiectasia. [20][4][9]
- Thinning of skin layers such as dermis and epidermis has been seen in atrophic phase. [8][20][9]
- Pigmented stratum germinativum also has been reported in some biopsies.[20]
- The following are list of pathognomonic microscopic findings when atrophic phase starts:[20][9][19]
- Epidermal atrophy
- Elastin and collagen damage
- Dermal blood vessels dilation
- Perivascular plasma cell infiltration
- Particles of elastic and oxytalan fibres
References
- ↑ 1.0 1.1 Smetanick MT, Zellis SL, Ermolovich T (2010). "Acrodermatitis chronica atrophicans: a case report and review of the literature". Cutis. 85 (5): 247–52. PMID 20540415.
- ↑ 2.0 2.1 2.2 Ogrinc K, Maraspin V, Lusa L, Cerar Kišek T, Ružić-Sabljić E, Strle F (2021). "Acrodermatitis chronica atrophicans: clinical and microbiological characteristics of a cohort of 693 Slovenian patients". J Intern Med. doi:10.1111/joim.13266. PMID 33550695 Check
|pmid=value (help). - ↑ 3.0 3.1 Scott JD (2020). "Presentation of Acrodermatitis Chronica Atrophicans Rashes on Lyme Disease Patients in Canada". Healthcare (Basel). 8 (2). doi:10.3390/healthcare8020157. PMC 7349802 Check
|pmc=value (help). PMID 32512846 Check|pmid=value (help). - ↑ 4.0 4.1 4.2 Asbrink E, Hovmark A, Olsson I (1986). "Clinical manifestations of acrodermatitis chronica atrophicans in 50 Swedish patients". Zentralbl Bakteriol Mikrobiol Hyg A. 263 (1–2): 253–61. doi:10.1016/s0176-6724(86)80128-6. PMID 3577484.
- ↑ Hansen K, Asbrink E (1989). "Serodiagnosis of erythema migrans and acrodermatitis chronica atrophicans by the Borrelia burgdorferi flagellum enzyme-linked immunosorbent assay". J Clin Microbiol. 27 (3): 545–51. doi:10.1128/jcm.27.3.545-551.1989. PMC 267355. PMID 2715325.
- ↑ Rudenko N, Golovchenko M (2021). "Sexual Transmission of Lyme Borreliosis? The Question That Calls for an Answer". Trop Med Infect Dis. 6 (2). doi:10.3390/tropicalmed6020087. PMID 34074046 Check
|pmid=value (help). - ↑ 7.0 7.1 Rijpkema SG, Tazelaar DJ, Molkenboer MJ, Noordhoek GT, Plantinga G, Schouls LM; et al. (1997). "Detection of Borrelia afzelii, Borrelia burgdorferi sensu stricto, Borrelia garinii and group VS116 by PCR in skin biopsies of patients with erythema migrans and acrodermatitis chronica atrophicans". Clin Microbiol Infect. 3 (1): 109–116. doi:10.1111/j.1469-0691.1997.tb00259.x. PMID 11864084.
- ↑ 8.0 8.1 8.2 8.3 Picken RN, Strle F, Picken MM, Ruzic-Sabljic E, Maraspin V, Lotric-Furlan S; et al. (1998). "Identification of three species of Borrelia burgdorferi sensu lato (B. burgdorferi sensu stricto, B. garinii, and B. afzelii) among isolates from acrodermatitis chronica atrophicans lesions". J Invest Dermatol. 110 (3): 211–4. doi:10.1046/j.1523-1747.1998.00130.x. PMID 9506437.
- ↑ 9.0 9.1 9.2 9.3 9.4 9.5 9.6 "StatPearls". 2021. PMID 33085436 Check
|pmid=value (help). - ↑ Guo, B P; Norris, S J; Rosenberg, L C; Höök, M (1995). "Adherence of Borrelia burgdorferi to the proteoglycan decorin". Infection and Immunity. 63 (9): 3467–3472. doi:10.1128/iai.63.9.3467-3472.1995. ISSN 0019-9567.
- ↑ Koning, J.; Tazelaar, D. J.; Hoogkamp-Korstanje, J. A. A.; Elema, J. D. (1995). "Acrodermatitis chronica atrophicans: A light and electron microscopic study". Journal of Cutaneous Pathology. 22 (1): 23–32. doi:10.1111/j.1600-0560.1995.tb00735.x. ISSN 0303-6987.
- ↑ 12.0 12.1 Muller, Kurt E. (2012). "Damage of Collagen and Elastic Fibres by Borrelia Burgdorferi – Known and New Clinical and Histopathological Aspects". The Open Neurology Journal. 6 (1): 179–186. doi:10.2174/1874205X01206010179. ISSN 1874-205X.
- ↑ Müllegger RR, McHugh G, Ruthazer R, Binder B, Kerl H, Steere AC (2000). "Differential expression of cytokine mRNA in skin specimens from patients with erythema migrans or acrodermatitis chronica atrophicans". J Invest Dermatol. 115 (6): 1115–23. doi:10.1046/j.1523-1747.2000.00198.x. PMID 11121150.
- ↑ Khalili M, Wong RJ (2018). "Underserved Does Not Mean Undeserved: Unfurling the HCV Care in the Safety Net". Dig Dis Sci. 63 (12): 3250–3252. doi:10.1007/s10620-018-5316-9. PMC 6436636. PMID 30311153.
- ↑ Kim, MyungHwa; Choi, MiSoo; Seong, GiHyun; Park, MyeongJin; Park, Minkee; Hong, SeungPhil; Park, ByungCheol (2020). "Rapidly progressing generalized morphea with high lyme disease titer". Indian Journal of Dermatology. 65 (5): 432. doi:10.4103/ijd.IJD_279_18. ISSN 0019-5154.
- ↑ 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.
- ↑ Aberer E, Klade H, Hobisch G (1991). "A clinical, histological, and immunohistochemical comparison of acrodermatitis chronica atrophicans and morphea". Am J Dermatopathol. 13 (4): 334–41. doi:10.1097/00000372-199108000-00003. PMID 1928618.
- ↑ Abele DC, Anders KH (1990). "The many faces and phases of borreliosis II". J Am Acad Dermatol. 23 (3 Pt 1): 401–10. doi:10.1016/0190-9622(90)70233-8. PMID 2212138.
- ↑ 19.0 19.1 19.2 de Koning J, Tazelaar DJ, Hoogkamp-Korstanje JA, Elema JD (1995). "Acrodermatitis chronica atrophicans: a light and electron microscopic study". J. Cutan. Pathol. 22 (1): 23–32. PMID 7751475. Unknown parameter
|month=ignored (help) - ↑ 20.0 20.1 20.2 20.3 Nadal, D; Gundelfinger, R; Flueler, U; Boltshauser, E (1988). "Acrodermatitis chronica atrophicans". Archives of Disease in Childhood. 63 (1): 72–74. doi:10.1136/adc.63.1.72. ISSN 0003-9888.