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

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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

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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

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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

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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

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### Common Causes

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* Bubonic plague
* Peripheral vascular disease
* Pneumonic plague
* Raynaud's disease

### Causes by Organ System

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Cardiovascular | Arteriovenous fistula, atherosclerosis, deep vein thrombosis, cutaneous polyarteritis nodosa, peripheral vascular disease, vasculitis

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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

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* Antiphospholipid Antibody Syndrome[1]
* Arteriovenous fistula[2]
* Atherosclerosis[3]
* Biological warefare agents
* Bleomycin[4]
* Borrelia infection[5]
* Bubonic plague
* Calcific azotemic arteriopathy[2]
* Capecitabine[6]
* Carbon monoxide poisoning[7]
* Chronic renal failure[8]
* Coumarin
* Cryofibrinogenemia[9]
* Cutaneous polyarteritis nodosa[10]

* Deep vein thrombosis
* Diabetes mellitus
* Doxorubicin
* Erythromelagia
* Fasciitis
* Frostbite
* Gemcitabine[11]
* Heparin induced thrombocytopenia
* Hypereosinophilic syndrome
* Panniculitis
* Peripheral vascular disease
* Pneumonic plague
* Polyangiitis overlap syndrome[12]
* Primary hyperoxaluria type 1[13]
* Purpura fulminans[14]
* Quinine

* Raynaud's disease
* Scleroderma
* Sharp syndrome[15]
* Stenotrophomonas maltophilia[16]
* T-cell lymphoma[17]
* Vasculitis
* Vinblastine
* Vincristine
* Waldenstrom's macroglobulinemia
* Warfarin

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## Natural History, Complications and Prognosis

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Untreated cases can lead to death.

## Treatment

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

## References

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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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
7. ↑ 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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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).CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Multiple names: authors list (link)
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.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)

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