Acroangiodermatitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jesus Rosario Hernandez, M.D. [2].

Synonyms and keywords: Mali acroangiodermatitis; Pseudo-Kaposi’s sarcoma.

Overview

Acroangiodermatitis of Mali (also known as “Mali acroangiodermatitis” and “Pseudo-Kaposi’s sarcoma”^[1]) is a rare cutaneous condition often characterized by purplish-blue to brown papules and plaques on the medial and lateral malleolus of both legs.^[1]^:1604^[2]

Acroangiodermatitis is a rare skin condition characterised by hyperplasia of pre-existing vasculature due to venous hypertension from severe chronic venous stasis. It is associated with amputees, haemodialysis (HD) patients with arteriovenous (AV) shunts, and patients with paralysed legs, hepatitis C, chronic venous insufficiency or AV malformations (AVM). Patients present with itchy, painful, confluent, violaceous or brown-black macules, papules or plaques usually at the distal lower limbs. There may be ulceration and bleeding. The histologic features are capillary proliferation and perivascular inflammation involving eosinophils in the dermis with minimal epidermal changes. Management includes compression therapy, wound care and surgical correction of AVM. Dapsone combined with leg elevation and compression, and erythromycin for HD patients with AV fistulas have also been reported. The lesions may persist for years with complications like ulceration, bleeding and infection.

Differential Diagnosis

Acroangiodermatitis should be differentiated from other conditions presenting as purplish-blue to brown papules and plaques on extremities. The differentials include the following:

Diseases	Etiology	Congenital	Acquired	Demography	Clinical manifestations							Lab findings						Gold standard diagnosis	Associated findings
					Symptoms			Signs				CBC			LFT	ESR/CRP	Histopathology
					Appearance	Fever	Bleeding	BP	Hepatosplenomegaly	Lymphadenopathy	Other	WBC	Hb	Plt	LFT	ESR/CRP	Histopathology
Bacillary angiomatosis ^[3]	HIV Chronic lymphocytic leukemia Cytotoxic chemotherapy Organ transplantation	–	+	Any age, usually between 20 -50 years	Solitary or multiple red, purple, flesh-colored, or colorless papules	±	±	Nl	–	–	Anorexia Weight loss Abdominal pain Nausea and vomiting Headache	Nl	Nl	Nl	Nl	Nl	Lobular vascular proliferations of vessels lined by plump endothelial cells	Clinical manifestation	Psychiatric disorders Personality changes Seizure
Arteriovenous malformation ^[4]	Idiopathic	+	–	Any age	Nl	–	+	Nl	–	–	Headache Neurologic deficits Heart failure Macrocephaly	Nl	Nl	Nl	Nl	Nl	NA	Imaging	Hereditary hemorrhagic telangiectasia
Acroangiodermatitis^[5]	Idiopathic Hyperplasia of pre-existing vasculature HTN	–	–	Any age, more in males	Purplish-blue to brown papules and plaques	–	–	Nl	–	–	Paralysed legs	Nl	Nl	Nl	Nl	Nl	Hyperkeratosis Parakeratosis Acanthosis Mild spongiosis	Clinical manifesttations	Amputation Haemodialysis patients with arteriovenous shunts Hepatitis C Chronic venous insufficiency AV malformations
Angiosarcoma ^[6]	Idiopathic	–	–	Adults, more in males	Enlarging bruise, a blue-black nodule, or an unhealed ulceration	–	–	Nl	–	–	–	Nl	↓	↓	Nl	Nl	Intercellular and intracellular lumina with or without red cells Intermediate filaments and pinocytotic vesicles in cytoplasm Weibel-Palade bodies	Biopsy	NA
Diseases	Etiology	Congenital	Acquired	Demography	Appearance	Fever	Bleeding	BP	Hepatosplenomegaly	Lymphadenopathy	Other	WBC	Hb	Plt	LFT	ESR/CRP	Histopathology	Gold standard diagnosis	Associated findings
Masson’s hemangioma ^[7]	Idiopathic	–	–	Rare	Normal	–	–	Nl	–	–	–	Nl	Nl	Nl	Nl	Nl	Papillary fronds lined by proliferating endothelium	Biopsy	Hemangioma Pyogenic granulomas Lymphangiomas
Seborrheic keratosis ^[8]	Clonal expansion of a mutated epidermal keratinocyte	+	–	Any age	Usually asymptomatic Being stuck on the skin surface	–	–	Nl	–	–	–	Nl	Nl	Nl	Nl	Nl	Papillomatous epithelial proliferation containing horn cysts	Clinical manifestations	Dermatosis papulosa nigra Stucco keratosis Melanoacanthoma Polypoid lesions
Systemic lupus erythematosus (SLE) ^[9]	Idiopathic	–	–	More common in female, typically in the 20 to 30 years	Erythema on the nasolabial folds Macular or diffusely erythematous in sun-exposed areas Discoid rash	±	–	↑	±	±	Weight loss Headache Arthralgia Myalgia Nausea Dyspepsia Pleuritic chest pain Dyspnea Hematuria	↑	↓	↓	Nl	Nl	Hyperkeratosis, epidermal atrophy, vacuolar interface dermatitis Thickening of the basement membrane Superficial, perivascular, and perifollicular mononuclear cell inflammatory infiltrate	Clinical manifestations	Raynaud phenomenon Neuropsychiatric symptoms Pleural effusion Peptic ulcer disease Pericarditis Myocarditis
Pyogenic granuloma ^[10]	Trauma Hormonal influences Viruses Cytogenetic clonal deletion abnormalities	+	+	Any age, usually in 20-30 years	Painless red lesion Lobular capillary hemangioma	–	+	Nl	–	–	–	Nl	Nl	Nl	Nl	Nl	Neutrophilic infiltration Hemorrhage Necrosis of the overlying epidermis	Clinical manifestation	NA
Benign lymphangioendothelioma ^[11]	Idiopathic	–	+	Any ages, median age is 50 years	single, slowly expanding patch, plaque, or nodule	–	–	Nl	–	–	–	Nl	Nl	Nl	Nl	Nl	Thin-walled endothelial-lined spaces that are interspersed between strands of collagen	Biopsy	NA
Cavernous hemangioma ^[12]	Idiopathic	–	–	Usually in third to fifth decades of life.	Painless, slowly progressive protrusion or bulging of their globe	–	–	Nl	–	–	–	Nl	Nl	Nl	Nl	Nl	Engorged vascular channels, which are tightly knit and separated by fibrous septae	Clinical manidestation	Diplopia Decreased color vision Visual field deficits
Diseases	Etiology	Congenital	Acquired	Demography	Appearance	Fever	Bleeding	BP	Hepatosplenomegaly	Lymphadenopathy	Other	WBC	Hb	Plt	LFT	ESR/CRP	Histopathology	Gold standard diagnosis	Associated findings

Physical examination

Gallery

Acroangiodermatitis.
Adapted from [http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=9

Acroangiodermatitis.
Adapted from [http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=9

References

↑ ^1.0 ^1.1 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
↑ Scholz S, Schuller-Petrovic S, Kerl H (March 2005). “Mali acroangiodermatitis in homozygous activated protein C resistance”. Arch Dermatol. 141 (3): 396–7. doi:10.1001/archderm.141.3.396. PMID 15781687.
↑ Tappero JW, Perkins BA, Wenger JD, Berger TG (July 1995). “Cutaneous manifestations of opportunistic infections in patients infected with human immunodeficiency virus”. Clin. Microbiol. Rev. 8 (3): 440–50. PMC 174635. PMID 7553576.
↑ Whitehead KJ, Smith MC, Li DY (February 2013). “Arteriovenous malformations and other vascular malformation syndromes”. Cold Spring Harb Perspect Med. 3 (2): a006635. doi:10.1101/cshperspect.a006635. PMC 3552339. PMID 23125071.
↑ Lugović L, Pusić J, Situm M, Buljan M, Bulat V, Sebetić K, Soldo-Belić A (2007). “Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports”. Acta Dermatovenerol Croat. 15 (3): 152–7. PMID 17868541.
↑ Barttelbort SW, Stahl R, Ariyan S (July 1989). “Cutaneous angiosarcoma of the face and scalp”. Plast. Reconstr. Surg. 84 (1): 55–9. PMID 2734404.
↑ Park KK, Won YS, Yang JY, Choi CS, Han KY (July 2012). “Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review”. J Korean Neurosurg Soc. 52 (1): 52–4. doi:10.3340/jkns.2012.52.1.52. PMC 3440504. PMID 22993679.
↑ Noiles K, Vender R (2008). “Are all seborrheic keratoses benign? Review of the typical lesion and its variants”. J Cutan Med Surg. 12 (5): 203–10. doi:10.2310/7750.2008.07096. PMID 18845088.
↑ Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P (2012). “Cutaneous manifestations of systemic lupus erythematosus”. Autoimmune Dis. 2012: 834291. doi:10.1155/2012/834291. PMC 3410306. PMID 22888407.
↑ Kamal R, Dahiya P, Puri A (January 2012). “Oral pyogenic granuloma: Various concepts of etiopathogenesis”. J Oral Maxillofac Pathol. 16 (1): 79–82. doi:10.4103/0973-029X.92978. PMC 3303528. PMID 22434943.
↑ Guillou L, Fletcher CD (August 2000). “Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi’s sarcoma: clinicopathologic analysis of a series”. Am. J. Surg. Pathol. 24 (8): 1047–57. PMID 10935645.
↑ Goldberg RE, Pheasant TR, Shields JA (December 1979). “Cavernous hemangioma of the retina. A four-generation pedigree with neurocutaneous manifestations and an example of bilateral retinal involvement”. Arch. Ophthalmol. 97 (12): 2321–4. PMID 229814.

[Bolognia-1] 1.0 ^1.1 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.

[pmid15781687-2] Scholz S, Schuller-Petrovic S, Kerl H (March 2005). “Mali acroangiodermatitis in homozygous activated protein C resistance”. Arch Dermatol. 141 (3): 396–7. doi:10.1001/archderm.141.3.396. PMID 15781687.

[pmid7553576-3] Tappero JW, Perkins BA, Wenger JD, Berger TG (July 1995). “Cutaneous manifestations of opportunistic infections in patients infected with human immunodeficiency virus”. Clin. Microbiol. Rev. 8 (3): 440–50. PMC 174635. PMID 7553576.

[pmid23125071-4] Whitehead KJ, Smith MC, Li DY (February 2013). “Arteriovenous malformations and other vascular malformation syndromes”. Cold Spring Harb Perspect Med. 3 (2): a006635. doi:10.1101/cshperspect.a006635. PMC 3552339. PMID 23125071.

[pmid17868541-5] Lugović L, Pusić J, Situm M, Buljan M, Bulat V, Sebetić K, Soldo-Belić A (2007). “Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports”. Acta Dermatovenerol Croat. 15 (3): 152–7. PMID 17868541.

[pmid2734404-6] Barttelbort SW, Stahl R, Ariyan S (July 1989). “Cutaneous angiosarcoma of the face and scalp”. Plast. Reconstr. Surg. 84 (1): 55–9. PMID 2734404.

[pmid22993679-7] Park KK, Won YS, Yang JY, Choi CS, Han KY (July 2012). “Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review”. J Korean Neurosurg Soc. 52 (1): 52–4. doi:10.3340/jkns.2012.52.1.52. PMC 3440504. PMID 22993679.

[pmid18845088-8] Noiles K, Vender R (2008). “Are all seborrheic keratoses benign? Review of the typical lesion and its variants”. J Cutan Med Surg. 12 (5): 203–10. doi:10.2310/7750.2008.07096. PMID 18845088.

[pmid22888407-9] Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P (2012). “Cutaneous manifestations of systemic lupus erythematosus”. Autoimmune Dis. 2012: 834291. doi:10.1155/2012/834291. PMC 3410306. PMID 22888407.

[pmid22434943-10] Kamal R, Dahiya P, Puri A (January 2012). “Oral pyogenic granuloma: Various concepts of etiopathogenesis”. J Oral Maxillofac Pathol. 16 (1): 79–82. doi:10.4103/0973-029X.92978. PMC 3303528. PMID 22434943.

[pmid10935645-11] Guillou L, Fletcher CD (August 2000). “Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi’s sarcoma: clinicopathologic analysis of a series”. Am. J. Surg. Pathol. 24 (8): 1047–57. PMID 10935645.

[pmid229814-12] Goldberg RE, Pheasant TR, Shields JA (December 1979). “Cavernous hemangioma of the retina. A four-generation pedigree with neurocutaneous manifestations and an example of bilateral retinal involvement”. Arch. Ophthalmol. 97 (12): 2321–4. PMID 229814.

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