Acral necrosis

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]

Overview

Acral necrosis is a symptom common in bubonic plague.

Historical Perspective

The striking black discoloration of skin and tissue, primarily on 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

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

Causes by Organ System

Cardiovascular	Antiphospholipid Antibody Syndrome, arteriovenous fistula, atherosclerosis, borrelia infection, cutaneous polyarteritis nodosa, diabetes mellitus, heparin induced thrombocytopenia, hypereosinophilic syndrome, scleroderma, Sharp syndrome, vasculitis
Chemical/Poisoning	Biological warefare agents, carbon monoxide poisoning
Dental	No underlying causes
Dermatologic	Antiphospholipid Antibody Syndrome, arteriovenous fistula, borrelia infection, bubonic plague, calcific azotemic arteriopathy, chronic renal failure, cryofibrinogenemia, cutaneous polyarteritis nodosa, deep vein thrombosis, diabetes mellitus, erythromelagia, fasciitis, frostbite, heparin induced thrombocytopenia, hypereosinophilic syndrome, panniculitis, peripheral vascular disease, pneumonic plague, polyangiitis overlap syndrome, primary hyperoxaluria type 1, purpura fulminans, Raynaud's disease, scleroderma, Sharp syndrome, T-cell lymphoma, vasculitis, Waldenstrom's macroglobulinemia
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	Bubonic plague, chronic renal failure, cutaneous polyarteritis nodosa, diabetes mellitus, fasciitis, scleroderma, hypereosinophilic syndrome, vasculitis
Genetic	Primary hyperoxaluria type 1
Hematologic	Antiphospholipid Antibody Syndrome, cryofibrinogenemia, heparin induced thrombocytopenia, polyangiitis overlap syndrome, primary hyperoxaluria type 1, purpura fulminans, Sharp syndrome
Iatrogenic	No underlying causes
Infectious Disease	Borrelia infection, bubonic plague, fasciitis, panniculitis, pneumonic plague, sternotrophomonas maltophilia
Musculoskeletal/Orthopedic	Antiphospholipid Antibody Syndrome, arteriovenous fistula, atherosclerosis, borrelia infection, bubonic plague, chronic renal failure, cutaneous polyarteritis nodosa, deep vein thrombosis, diabetes mellitus, erythromelagia, frostbite, heparin induced thrombocytopenia, peripheral vascular disease, polyangiitis overlap syndrome, Raynaud's disease, scleroderma, Sharp syndrome, T-cell lymphoma, vasculitis, Waldenstrom's macroglobulinemia
Neurologic	Antiphospholipid Antibody Syndrome, arteriovenous fistula, atherosclerosis, borrelia infection, bubonic plague,chronic renal failure, cutaneous polyarteritis nodosa, diabetes mellitus, frostbite, hypereosinophilic syndrome, peripheral vascular disease, pneumonic plague, vasculitis, Waldenstrom's macroglobulinemia
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	Arteriovenous fistula, bubonic plague, chronic renal failure, deep vein thrombosis, heparin induced thrombocytopenia, hypereosinophilic syndrome, pneumonic plague, scleroderma, Sharp syndrome, sternotrophomonas maltophilia, vasculitis
Renal/Electrolyte	No underlying causes
Rheumatology/Immunology/Allergy	No underlying causes
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	No underlying causes

Causes in Alphabetical Order

• Antiphospholipid Antibody Syndrome
• Arteriovenous fistula
• Atherosclerosis
• Biological warefare agents
• Bleomycin
• Borrelia infection
• Bubonic plague
• Calcific azotemic arteriopathy
• Capecitabine
• Carbon monoxide poisoning
• Chronic renal failure
• Coumarin
• Cryofibrinogenemia
• Cutaneous polyarteritis nodosa
• Deep vein thrombosis
• Diabetes mellitus
• Doxorubicin
• Erythromelagia
• Fasciitis
• Frostbite
• Gemcitabine
• Heparin induced thrombocytopenia
• Hypereosinophilic syndrome
• Panniculitis
• Peripheral vascular disease
• Pneumonic plague
• Polyangiitis overlap syndrome
• Primary hyperoxaluria type 1
• Purpura fulminans
• Quinine
• Raynaud's disease
• Scleroderma
• Sharp syndrome
• Sternotrophomonas maltophilia
• T-cell lymphoma
• Vasculitis
• Vinblastine
• Vincristine
• Waldenstrom's macroglobulinemia
• Warfarin

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.