Acral necrosis

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	Acral necrosis;
	A hand with acral gangrene due to plague

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ogheneochuko Ajari, MB.BS, MS [2]

Overview

Acral necrosis is the black discoloration of the skin of the extremities due to decreased blood supply to the afftected areas. Acral necrosis is a common symptom of bubonic plague.

Historical Perspective

The striking black discoloration of skin and tissue of the extremities is commonly thought to have given rise to the name Black Death, associated both with the disease and the pandemic which occurred in the 14th century. The term in fact came from the figural sense of "black", that is glum, lugubrious or dreadful.

Pathophysiology

Clotting and bleeding beneath the skin causes an area of hemorrhage, the presence of red blood cells lying outside of capillaries, into the skin and subcutaneous tissue. In isolation, this is called an ecchymosis or bruise and may be the result of injury or illness. However, acral necrosis occurs when blood supply is disrupted for prolonged periods, blackening and damaging the affected area and surrounding tissue.

Gross Pathology

Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Hand: Gangrene: Gross excellent demonstration of gangrenous necrosis of fingers
Hand: Gangrene: Gross excellent demonstration of gangrenous necrosis of fingers

Hand: Gangrene: Gross excellent demonstration of gangrenous necrosis of fingers
Necrosis of distal finger in a patient with panniculitis and fascitis, streptococcus A septicemia in a patient with Scleroderma who was on high dose steroids

Causes

Common Causes

Causes by Organ System

Cardiovascular	Arteriovenous fistula, atherosclerosis, deep vein thrombosis, cutaneous polyarteritis nodosa, peripheral vascular disease, vasculitis
Chemical/Poisoning	Biological warefare agents, carbon monoxide poisoning
Dental	No underlying causes
Dermatologic	Calcific azotemic arteriopathy, deep vein thrombosis, erythromelagia, fasciitis, frostbite, panniculitis, polyangiitis overlap syndrome, purpura fulminans, Raynaud's disease, scleroderma, Sharp syndrome, T-cell lymphoma, vasculitis
Drug Side Effect	Bleomycin, capecitabine, coumarin, doxorubicin, gemcitabine, heparin induced thrombocytopenia, quinine, vinblastine, vincristine, warfarin
Ear Nose Throat	Vasculitis, Waldenstrom's macroglobulinemia
Endocrine	Diabetes mellitus
Environmental	No underlying causes
Gastroenterologic	No underlying causes
Genetic	Primary hyperoxaluria type 1
Hematologic	Cryofibrinogenemia, heparin induced thrombocytopenia, hypereosinophilic syndrome, polyangiitis overlap syndrome, primary hyperoxaluria type 1, purpura fulminans, Waldenstrom's macroglobulinemia
Iatrogenic	No underlying causes
Infectious Disease	Borrelia infection, bubonic plague, fasciitis, panniculitis, pneumonic plague, stenotrophomonas maltophilia
Musculoskeletal/Orthopedic	No underlying causes
Neurologic	No underlying causes
Nutritional/Metabolic	Diabetes mellitus, primary hyperoxaluria type 1
Obstetric/Gynecologic	Antiphospholipid antibody syndrome, chronic renal failure
Oncologic	T-cell lymphoma
Ophthalmologic	Waldenstrom's macroglobulinemia
Overdose/Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	No underlying causes
Renal/Electrolyte	Calcific azotemic arteriopathy, chronic renal failure, polyangiitis overlap syndrome, primary hyperoxaluria type 1, vasculitis
Rheumatology/Immunology/Allergy	Antiphospholipid antibody syndrome, cutaneous polyarteritis nodosa, peripheral vascular disease, polyangiitis overlap syndrome, Raynaud's disease, scleroderma, Sharp syndrome, vasculitis, Waldenstrom's macroglobulinemia
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	Primary hyperoxaluria type 1, stenotrophomonas maltophilia, vasculitis
Miscellaneous	No underlying causes

Causes in Alphabetical Order

Natural History, Complications and Prognosis

Untreated cases can lead to death.

Treatment

With appropriate medical treatment, areas with acral necrosis may be successfully restored to function.

References

↑ Van Beek N, Schumacher N, Haase O, Zillikens D, Kahle B, Schmidt E (2013). "[Primary antiphospholipid syndrome: newly developed leg ulcer and history of stroke.]". Hautarzt. doi:10.1007/s00105-013-2601-6. PMID 23744031.
↑ ^2.0 ^2.1 Mactier RA, Stewart WK, Parham DM, Tainsh JA (1990). "Acral gangrene attributed to calcific azotaemic arteriopathy and the steal effect of an arteriovenous fistula". Nephron. 54 (4): 347–50. PMID 2325802.
↑ Stelzner C, Zimmermann F, Schellong SM (2012). "[Digital necrosis in a patient with atherosclerosis: also a paraneoplasia?]". Dtsch Med Wochenschr. 137 (15): 780–4. doi:10.1055/s-0032-1304914. PMID 22492412.
↑ Grünwald V, Bolte O, Wiebe S, Ganser A, Schöffski P (2005). "Acral necrosis after inadequate excessive administration of bleomycin in a testicular cancer patient". Onkologie. 28 (1): 41–3. doi:10.1159/000082054. PMID 15591723.
↑ Bär H, Pöhlmann G, Figulla HR (2000). "[Acute acral ischemia in all fingers possibly due to a Borrelia infection]". Vasa. 29 (4): 279–81. PMID 11141652.
↑ Sauter C, Saborowski A, Ockenfels HM (2007). "[Unilateral acral necrosis as a minor form of hand-foot syndrome. Patient with unilateral acral necrosis secondary to capecitabine therapy for metastatic breast cancer]". Hautarzt. 58 (7): 619–22. doi:10.1007/s00105-006-1227-3. PMID 17066280.
↑ del Giudice P, Lacour JP, Bahadoran P, Hoff-Bermond C, Ortonne JP (1995). "[Cutaneous necrosis of the extremities during carbon monoxide poisoning]". Ann Dermatol Venereol. 122 (11–12): 780–2. PMID 8729825.
↑ Scheinman PL, Helm KF, Fairley JA (1991). "Acral necrosis in a patient with chronic renal failure. Calciphylaxis". Arch Dermatol. 127 (2): 248–9, 251–2. PMID 1990994.
↑ Jansen TL, Janssen M, Janssens PM (2003). "Transient cryofibrinogenaemia with acral digital necrosis, secondary Raynaud's phenomenon and polyarthritis". Clin Exp Rheumatol. 21 (3): 407–8. PMID 12846071.
↑ Stussi G, Schneider E, Trüeb RM, Seebach JD (2001). "Acral necrosis of the fingers as initial manifestation of cutaneous polyarteritis nodosa--a case report". Angiology. 52 (1): 63–7. PMID 11205933.
↑ Holstein A, Bätge R, Egberts EH (2010). "Gemcitabine induced digital ischaemia and necrosis". Eur J Cancer Care (Engl). 19 (3): 408–9. doi:10.1111/j.1365-2354.2008.01057.x. PMID 19490003.
↑ Weichenhain B, Nerl C, Stiegler H (1994). "[Polyangiitis overlap syndrome]". Dtsch Med Wochenschr. 119 (17): 618–23. doi:10.1055/s-2008-1058738. PMID 8168423.
↑ DE Grip WJ, Bonting SL, Daemen FJ (1975). "Biochemical aspects of the visual process. XXXI. Chemical modification studies on rod outer segment retinol dehydrogenase". Exp Eye Res. 21 (6): 549–55. PMID NBK1283 Check |pmid= value (help).
↑ Süss R, Megahed M, Zumdick M, Glover M, Ruzicka T, Lehmann P (1996). "[Purpura fulminans with extensive skin necroses]". Hautarzt. 47 (7): 541–4. PMID 8926172.
↑ Silbernagel G, Schmalzing M, Riessen R, Koetter I (2010). "[Sepsis with acral necrosis in a patient with Sharp syndrome - case 8/2010]". Dtsch Med Wochenschr. 135 (36): 1741. doi:10.1055/s-0030-1247615. PMID 20812159.
↑ Pereira O, Velho GC, Lopes V, Mota F, Santos C, Massa A (2001). "Acral necrosis by Stenotrophomonas maltophilia". J Eur Acad Dermatol Venereol. 15 (4): 334–6. PMID 11730046.
↑ Distelrath K, Neuber K, Blödorn-Schlicht N, Altenhoff J, Dührsen U, Düring J; et al. (1999). "[Painful ischemia of the extremities with T-cell lymphoma]". Hautarzt. 50 (4): 284–7. PMID 10354922.

Template:WH Template:WS

[pmid23744031-1] Van Beek N, Schumacher N, Haase O, Zillikens D, Kahle B, Schmidt E (2013). "[Primary antiphospholipid syndrome: newly developed leg ulcer and history of stroke.]". Hautarzt. doi:10.1007/s00105-013-2601-6. PMID 23744031.

[pmid2325802-2] 2.0 ^2.1 Mactier RA, Stewart WK, Parham DM, Tainsh JA (1990). "Acral gangrene attributed to calcific azotaemic arteriopathy and the steal effect of an arteriovenous fistula". Nephron. 54 (4): 347–50. PMID 2325802.

[pmid22492412-3] Stelzner C, Zimmermann F, Schellong SM (2012). "[Digital necrosis in a patient with atherosclerosis: also a paraneoplasia?]". Dtsch Med Wochenschr. 137 (15): 780–4. doi:10.1055/s-0032-1304914. PMID 22492412.

[pmid15591723-4] Grünwald V, Bolte O, Wiebe S, Ganser A, Schöffski P (2005). "Acral necrosis after inadequate excessive administration of bleomycin in a testicular cancer patient". Onkologie. 28 (1): 41–3. doi:10.1159/000082054. PMID 15591723.

[pmid11141652-5] Bär H, Pöhlmann G, Figulla HR (2000). "[Acute acral ischemia in all fingers possibly due to a Borrelia infection]". Vasa. 29 (4): 279–81. PMID 11141652.

[pmid17066280-6] Sauter C, Saborowski A, Ockenfels HM (2007). "[Unilateral acral necrosis as a minor form of hand-foot syndrome. Patient with unilateral acral necrosis secondary to capecitabine therapy for metastatic breast cancer]". Hautarzt. 58 (7): 619–22. doi:10.1007/s00105-006-1227-3. PMID 17066280.

[pmid8729825-7] Giudice P, Lacour JP, Bahadoran P, Hoff-Bermond C, Ortonne JP (1995). "[Cutaneous necrosis of the extremities during carbon monoxide poisoning]". Ann Dermatol Venereol. 122 (11–12): 780–2. PMID 8729825.

[pmid1990994-8] Scheinman PL, Helm KF, Fairley JA (1991). "Acral necrosis in a patient with chronic renal failure. Calciphylaxis". Arch Dermatol. 127 (2): 248–9, 251–2. PMID 1990994.

[pmid12846071-9] Jansen TL, Janssen M, Janssens PM (2003). "Transient cryofibrinogenaemia with acral digital necrosis, secondary Raynaud's phenomenon and polyarthritis". Clin Exp Rheumatol. 21 (3): 407–8. PMID 12846071.

[pmid11205933-10] Stussi G, Schneider E, Trüeb RM, Seebach JD (2001). "Acral necrosis of the fingers as initial manifestation of cutaneous polyarteritis nodosa--a case report". Angiology. 52 (1): 63–7. PMID 11205933.

[pmid19490003-11] Holstein A, Bätge R, Egberts EH (2010). "Gemcitabine induced digital ischaemia and necrosis". Eur J Cancer Care (Engl). 19 (3): 408–9. doi:10.1111/j.1365-2354.2008.01057.x. PMID 19490003.

[pmid8168423-12] Weichenhain B, Nerl C, Stiegler H (1994). "[Polyangiitis overlap syndrome]". Dtsch Med Wochenschr. 119 (17): 618–23. doi:10.1055/s-2008-1058738. PMID 8168423.

[pmidNBK1283-13] DE Grip WJ, Bonting SL, Daemen FJ (1975). "Biochemical aspects of the visual process. XXXI. Chemical modification studies on rod outer segment retinol dehydrogenase". Exp Eye Res. 21 (6): 549–55. PMID NBK1283 Check |pmid= value (help).

[pmid8926172-14] Süss R, Megahed M, Zumdick M, Glover M, Ruzicka T, Lehmann P (1996). "[Purpura fulminans with extensive skin necroses]". Hautarzt. 47 (7): 541–4. PMID 8926172.

[pmid20812159-15] Silbernagel G, Schmalzing M, Riessen R, Koetter I (2010). "[Sepsis with acral necrosis in a patient with Sharp syndrome - case 8/2010]". Dtsch Med Wochenschr. 135 (36): 1741. doi:10.1055/s-0030-1247615. PMID 20812159.

[pmid11730046-16] Pereira O, Velho GC, Lopes V, Mota F, Santos C, Massa A (2001). "Acral necrosis by Stenotrophomonas maltophilia". J Eur Acad Dermatol Venereol. 15 (4): 334–6. PMID 11730046.

[pmid10354922-17] Distelrath K, Neuber K, Blödorn-Schlicht N, Altenhoff J, Dührsen U, Düring J; et al. (1999). "[Painful ischemia of the extremities with T-cell lymphoma]". Hautarzt. 50 (4): 284–7. PMID 10354922.

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