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Acrocyanosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Cassirer syndrome; Crocq disease

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Acrocyanosis refer to a persistent blue or cyanotic discoloration of the digits, most commonly occurring in the hands although also occurring in the face and feet as well. The blue color is typically associated with poor oxygenation such as in asthma or emphysema

References

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Historical Perspective

Historical Perspective

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References

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Classifications

Classifications

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Classification

Acrocyanosis can be placed into two categories. They are:

References

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Gross Pathology

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

  • Acrocyanosis: Gross natural color outstandingly horrible example in infant with apparent gangrene of distal and middle phalanges. Pseudomonas sepsis
    Acrocyanosis: Gross natural color outstandingly horrible example in infant with apparent gangrene of distal and middle phalanges. Pseudomonas sepsis
  • Acrocyanosis: Gross natural color horrible example of gangrene of feet and skin of legs with Pseudomonas sepsis
    Acrocyanosis: Gross natural color horrible example of gangrene of feet and skin of legs with Pseudomonas sepsis
  • Acrocyanosis: Gross, a case of DIC, distal phalangeal cyanosis
    Acrocyanosis: Gross, a case of DIC, distal phalangeal cyanosis
  • Hand: Acrocyanosis: Gross, an excellent example
    Hand: Acrocyanosis: Gross, an excellent example
  • Acrocyanosis: Gross, excellent example of cyanotic nail beds
    Acrocyanosis: Gross, excellent example of cyanotic nail beds

References

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Causes

Causes

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

Overview

Acrocyanosis results from central or local tissue oxygenation defects[1]. Acrocyanosis could be due to both primary and secondary causes, from neurologic, psychiatric, autoimmune, infective, metabolic or other causes and its treatment depends on the underlying cause.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Causes by Organ System

Cardiovascular Atheromatous embolism, Buerger’s disease, chronic orthostatic intolerance, erythromelalgia, myocardial infarction, postural orthostatic tachycardia syndrome, pulmonary arteriovenous malformation, pulmonary embolism, pulmonary hypertension, Sneddon syndrome, Takayasu arteritis
Chemical / poisoning Arsenic poisoning, Butyl nitrate
Dermatologic Acrocyanosis of infancy, black foot disease, Buerger’s disease, ethylmalonic encephalopathy, fucosidosis, livedoid vasculopathy, oxalosis, remittent idiopathic necrotizing acrocyanosis, scleroderma, sulfhemoglobinemia, Van Bogaert-Hozay syndrome, waldenström macroglobulinemia, wegener’s granulomatosis
Drug Side Effect Amiodarone, Amphotericin B deoxycholate, butyl nitrate, butyl nitrite, desipramine, fluoxetine, gemcitabine, imipramine, interferon 2 alpha, metoclopramide, phenazopyridine, tolazoline, tricyclic antidepressants, vasopressors
Ear Nose Throat Wegener’s granulomatosis, Van Bogaert-Hozay syndrome
Endocrine Anorexia nervosa, bulimia nervosa, carcinoid syndrome, POEMS syndrome
Environmental Cold exposure, high altitude
Gastroenterologic Anorexia nervosa, bulimia nervosa, carcinoid syndrome, fucosidosis, Sandifer syndrome, scleroderma, Waldenström macroglobulinemia
Genetic Aicardi-Goutieres syndrome, Asperger disease, DiGeorge syndrome, erythromelalgia, ethylmalonic aciduria, ethylmalonic encephalopathy, familial spastic paraplegia, fucosidosis, genetic, mitochondrial disease
Hematologic Antiphospholipid syndrome, carcinoid syndrome, Castleman’s disease, chronic lymphocytic leukemia, cold agglutinin disease, cryoglobulinemia, essential thrombocythemia, methemoglobinemia, paroxysmal cold hemoglobinuria, polycythemia, pulmonary embolism, secondary polycythemia, sulfhemoglobinemia, Waldenström macroglobulinemia
Iatrogenic No underlying causes
Infectious Disease Congenital syphilis, cytomegalovirus, HIV, infectious mononucleosis, influenza virus infection, listeriosis, mumps, mycoplasma, parvovirus B19 infection, psittacosis, secondary syphilis
Musculoskeletal / Ortho Antiphospholipid syndrome, brachial plexus neuropathy, Buerger’s disease, erythromelalgia, fucosidosis, Klippel-Feil syndrome, puffy hand syndrome, stroke,remittent idiopathic necrotizing acrocyanosis, scalenus anterior syndrome, supernumerary cervical rib, Van Bogaert-Hozay syndrome, Waldenström macroglobulinemia
Neurologic Adolescent chronic fatigue syndrome, Aicardi-Goutieres syndrome, antiphospholipid syndrome, apnea, Asperger disease, brachial plexus neuropathy, cervical plexus compression neuropathy, ergotism, erythromelalgia, ethylmalonic aciduria, ethylmalonic encephalopathy, familial spastic paraplegia, mitochondrial disease, POEMS syndrome, Sandifer syndrome, Sneddon syndrome, spinal cord injury, stroke, thoracic outlet syndrome, tonic-clonic seizure, Van Bogaert-Hozay syndrome, vegetative dystonia
Nutritional / Metabolic Anorexia nervosa, bulimia nervosa, ethylmalonic aciduria, fucosidosis
Obstetric/Gynecologic Antiphospholipid syndrome,ovarian cancer
Oncologic Burkitt’s lymphoma, carcinoid syndrome, chronic lymphocytic leukemia, Hodgkin lymphoma, malignancy, ovarian cancer, paraneoplastic disorder, Waldenström macroglobulinemia
Opthalmologic Atheromatous embolism, Van Bogaert-Hozay syndrome, Waldenström macroglobulinemia, Wegener’s granulomatosis
Overdose / Toxicity Ergotism
Psychiatric Adolescent chronic fatigue syndrome, anorexia nervosa, asperger disease, bulimia nervosa, neurocirculatory asthenia, schizophrenia, Van Bogaert-Hozay syndrome
Pulmonary Pickwickian syndrome, pulmonary alveolar proteinosis, pulmonary arteriovenous malformation, pulmonary embolism, pulmonary hypertension, scleroderma, Waldenström macroglobulinemia, Wegener’s granulomatosis
Renal / Electrolyte Hyperoxaluria type 1, oxalosis, scleroderma, Waldenström macroglobulinemia, Wegener’s granulomatosis
Rheum / Immune / Allergy Adolescent chronic fatigue syndrome, antiphospholipid syndrome, Buerger’s disease, Castleman’s disease, CREST syndrome, cryoglobulinemia, DiGeorge syndrome, livedoid vasculopathy, mixed connective tissue disorder, paraneoplastic disorder, paraproteinemia, Raynaud’s phenomenon, rheumatoid arthritis, scleroderma, Sneddon syndrome, systemic lupus erythematosus, systemic sclerosis, Wegener’s granulomatosis
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Dental Wegener’s granulomatosis
Miscellaneous Black foot disease, high altitude, idiopathic

Causes in Alphabetical Order

References

  1. ↑ Das S, Maiti A (2013). “Acrocyanosis: an overview”. Indian J Dermatol. 58 (6): 417–20. doi:10.4103/0019-5154.119946. PMC 3827510. PMID 24249890.
  2. ↑ Pavlou E, Augoustides-Savvopoulou P, Gregersen N, Haas D, Gkampeta A, Athanassiadou-Piperopoulou F (2013). “An infant with ethylmalonic encephalopathy masquerading as a hematologic disorder”. J Child Neurol. 28 (5): 668–71. doi:10.1177/0883073812449070. PMID 22805253.
  3. ↑ Gayraud M (2007). “Raynaud’s phenomenon”. Joint Bone Spine. 74 (1): e1–8. doi:10.1016/j.jbspin.2006.07.002. PMID 17218139.
  4. ↑ 4.0 4.1 Stewart JM, Gewitz MH, Weldon A, Arlievsky N, Li K, Munoz J (1999). “Orthostatic intolerance in adolescent chronic fatigue syndrome”. Pediatrics. 103 (1): 116–21. PMID 9917448.
  5. ↑ Abdel-Salam GM, Zaki MS, Lebon P, Meguid NA (2004). “Aicardi-Goutières syndrome: clinical and neuroradiological findings of 10 new cases”. Acta Paediatr. 93 (7): 929–36. PMID 15303808.
  6. ↑ Ozaras R, Yemisen M, Mete B, Mert A, Ozturk R, Tabak F (2007). “Acrocyanosis developed with amphotericin B deoxycholate but not with amphotericin B lipid complex”. Mycoses. 50 (3): 242. doi:10.1111/j.1439-0507.2007.01360.x. PMID 17472626.
  7. ↑ 7.0 7.1 Mitchell JE, Crow S (2006). “Medical complications of anorexia nervosa and bulimia nervosa”. Curr Opin Psychiatry. 19 (4): 438–43. doi:10.1097/01.yco.0000228768.79097.3e. PMID 16721178.
  8. ↑ 8.0 8.1 Strumia R (2005). “Dermatologic signs in patients with eating disorders”. Am J Clin Dermatol. 6 (3): 165–73. PMID 15943493.
  9. ↑ Salluh JI, Soares M, De Meis E (2009). “Antiphospholipid antibodies and multiple organ failure in critically ill cancer patients”. Clinics (Sao Paulo). 64 (2): 79–82. PMC 2666481. PMID 19219311.
  10. ↑ Diallo M, Niang SO, Kane A, Dieng MT, Ndiaye B (2007). “[Antiphospholipid antibodies syndrome in dermatology in Dakar: 11 case report]”. Dakar Med. 52 (1): 41–5. PMID 19102091.
  11. ↑ Hall AH (2002). “Chronic arsenic poisoning”. Toxicol Lett. 128 (1–3): 69–72. PMID 11869818.
  12. ↑ Rubin DI, Schomberg PJ, Shepherd RF, Panneton JM (2001). “Arteritis and brachial plexus neuropathy as delayed complications of radiation therapy”. Mayo Clin Proc. 76 (8): 849–52. doi:10.1016/S0025-6196(11)63232-1. PMID 11499827.
  13. ↑ Smith P, Rice M, Ricci N, Toogood I, Roberton D (1993). “A case of Burkitt’s lymphoma presenting with digital ischaemia”. Acta Paediatr. 82 (2): 217–9. PMID 8477174.
  14. ↑ Hoegl L, Thoma-Greber E, Poppinger J, Röcken M (1999). “Butyl nitrite-induced acrocyanosis in an HIV-infected patient”. Arch Dermatol. 135 (1): 90–1. PMID 9923790.
  15. ↑ Di Lucca J, Mahé E, Saiag P (2007). “[Severe acrocyanosis in carcinoid syndrome]”. Ann Dermatol Venereol. 134 (11): 895–6. PMID 18033083.
  16. ↑ Hirono H, Kubota T, Funakoshi K, Watanabe T, Hasegawa K, Soga K; et al. (2011). “[A case of superior mesenteric artery occlusion associated with idiopathic chronic cold agglutinin disease]”. Nihon Shokakibyo Gakkai Zasshi. 108 (5): 791–8. PMID 21558747.
  17. ↑ 17.0 17.1 Lauritano D, Bussolati A, Baldoni M, Leonida A (2011). “Scleroderma and CREST syndrome: a case report in dentistry”. Minerva Stomatol. 60 (9): 443–65. PMID 21956352.
  18. ↑ Iguchi S, Toba K, Fuse I, Wada Y, Maruyama S, Takahashi M; et al. (1996). “Severe cryoglobulinemia in a patient with asymptomatic hepatitis C virus infection”. Intern Med. 35 (9): 712–6. PMID 8915697.
  19. ↑ Bruno B, Barbier C, Lambilliotte A, Rey C, Turck D (2002). “Auto-immune pancytopenia in a child with DiGeorge syndrome”. Eur J Pediatr. 161 (7): 390–2. doi:10.1007/s00431-002-0976-y. PMID 12111192.
  20. ↑ Tarach JS, Nowicka-Tarach BM, Matuszek B, Nowakowski A (2000). “Erythromelalgia–a thrombotic complication in chronic myeloproliferative disorders”. Med Sci Monit. 6 (1): 204–8. PMID 11208311.
  21. ↑ Koudstaal PJ, Koudstaal A (1997). “Neurologic and visual symptoms in essential thrombocythemia: efficacy of low-dose aspirin”. Semin Thromb Hemost. 23 (4): 365–70. doi:10.1055/s-2007-996110. PMID 9263353.
  22. ↑ Ozand PT, Rashed M, Millington DS, Sakati N, Hazzaa S, Rahbeeni Z; et al. (1994). “Ethylmalonic aciduria: an organic acidemia with CNS involvement and vasculopathy”. Brain Dev. 16 Suppl: 12–22. PMID 7726376.
  23. ↑ Heberle LC, Al Tawari AA, Ramadan DG, Ibrahim JK (2006). “Ethylmalonic encephalopathy-report of two cases”. Brain Dev. 28 (5): 329–31. doi:10.1016/j.braindev.2005.10.005. PMID 16376514.
  24. ↑ Fleming C, Rennie A, Fallowfield M, McHenry PM (1997). “Cutaneous manifestations of fucosidosis”. Br J Dermatol. 136 (4): 594–7. PMID 9155966.
  25. ↑ 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.
  26. ↑ Solak Y, Aksoy S, Kilickap S, Celik I (2006). “Acrocyanosis as a presenting symptom of Hodgkin lymphoma”. Am J Hematol. 81 (2): 151–2. doi:10.1002/ajh.20479. PMID 16432859.
  27. ↑ Nousari HC, Kimyai-Asadi A, Anhalt GJ (2001). “Chronic idiopathic acrocyanosis”. J Am Acad Dermatol. 45 (6 Suppl): S207–8. PMID 11712060.
  28. ↑ Anderson RP, Morris BA (1988). “Acrocyanosis due to imipramine”. Arch Dis Child. 63 (2): 204–5. PMC 1778745. PMID 3348672.
  29. ↑ Campo-Voegeli A, Estrach T, Marti RM, Corominas N, Tuset M, Mascaró JM (1998). “Acrocyanosis induced by interferon alpha(2a)”. Dermatology. 196 (3): 361–3. PMID 9621152.
  30. ↑ Jiminez MA, Polena S, Coplan NL, Patel K, Gintautas J (2007). “Methemoglobinemia and transesophageal echo”. Proc West Pharmacol Soc. 50: 134–5. PMID 18605250.
  31. ↑ Bodemer C, Rötig A, Rustin P, Cormier V, Niaudet P, Saudubray JM; et al. (1999). “Hair and skin disorders as signs of mitochondrial disease”. Pediatrics. 103 (2): 428–33. PMID 9925836.
  32. ↑ Legrain S, Raguin G, Piette JC (1999). “Digital necrosis revealing ovarian cancer”. Dermatology. 199 (2): 183–4. doi:18235 Check |doi= value (help). PMID 10559594.
  33. ↑ Jorquera-Barquero E, Súarez-Marrero MC, Fernández Girón F, Borrero Martín JJ (2013). “Oxalosis and livedo reticularis”. Actas Dermosifiliogr. 104 (9): 815–8. doi:10.1016/j.ad.2012.04.019. PMID 23103120.
  34. ↑ Blackmon JA, Jeffy BG, Malone JC, Knable AL (2011). “Oxalosis involving the skin: case report and literature review”. Arch Dermatol. 147 (11): 1302–5. doi:10.1001/archdermatol.2011.182. PMID 21768445.
  35. ↑ Penouil MH, Estève E, Millotte B, Bressieux JM (1997). “[Parvovirus B19 atypical acrosyndrome]”. Ann Dermatol Venereol. 124 (3): 254–6. PMID 9686060.
  36. ↑ Kermani TA, Pislaru SV, Osborn TG (2009). “Acrocyanosis from phenazopyridine-induced sulfhemoglobinemia mistaken for Raynaud phenomenon”. J Clin Rheumatol. 15 (3): 127–9. doi:10.1097/RHU.0b013e31819db6db. PMID 19300288.
  37. ↑ Miest RY, Comfere NI, Dispenzieri A, Lohse CM, el-Azhary RA (2013). “Cutaneous manifestations in patients with POEMS syndrome”. Int J Dermatol. 52 (11): 1349–56. doi:10.1111/j.1365-4632.2012.05648.x. PMID 23557151.
  38. ↑ Stewart JM, Weldon A (2000). “Vascular perturbations in the chronic orthostatic intolerance of the postural orthostatic tachycardia syndrome”. J Appl Physiol (1985). 89 (4): 1505–12. PMID 11007589.
  39. ↑ Semel JD (1984). “Cutaneous findings in a case of psittacosis”. Arch Dermatol. 120 (9): 1227–9. PMID 6476861.
  40. ↑ Das S, Roy AK, Maiti A (2010). “Remittent idiopathic necrotizing acrocyanosis – a rare entity”. Indian J Dermatol. 55 (1): 95–6. doi:10.4103/0019-5154.60362. PMC 2856383. PMID 20418987.
  41. ↑ Lotz BP, Schutte CM, Colin PF, Biermann LD (1993). “Sneddon’s syndrome with anticardiolipin antibodies–complications and treatment”. S Afr Med J. 83 (9): 663–4. PMID 8310361.
  42. ↑ Richter JG, Sander O, Schneider M, Klein-Weigel P (2010). “Diagnostic algorithm for Raynaud’s phenomenon and vascular skin lesions in systemic lupus erythematosus”. Lupus. 19 (9): 1087–95. doi:10.1177/0961203310374304. PMID 20693202.
  43. ↑ Feito Rodríguez M, García Macarrón J, Martín Díaz MA, Rubio Flores C, Vidaurrázaga Díaz-Arcaya C (2006). “[Acrocyanosis as a form of presentation of progressive systemic sclerosis]”. An Med Interna. 23 (10): 490–2. PMID 17134313.
  44. ↑ Cornejo R, Gatica H, Segovia E, Cortés C (2002). “[Intestinal necrosis as clinical presentation of Takayasu arteritis]”. Rev Med Chil. 130 (10): 1159–64. PMID 12491834.
  45. ↑ Karakaya I, Aydoğan M, Coşkun A, Gökalp AS (2003). “Acrocyanosis as a side effect of tricyclic antidepressants: a case report”. Turk J Pediatr. 45 (2): 155–7. PMID 12921305.

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Differentiating Acrocyanosis from other Disorders

Differentiating Acrocyanosis from other Disorders

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Differential Diagnosis

The blue discoloration that is seen in a person with acrocyanosis may also be caused by argyria or DADPS

References

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Epidemiology and Demographics

Epidemiology and Demographics

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Risk Factors

Risk Factors

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Screening

Screening

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Natural History, Complications, and Prognosis

Natural History, Complications, and Prognosis

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Rays | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

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