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Diffuse Verrucous, Vascular Nodules on the Extremities and Trunk—Diagnosis

Diffuse Verrucous, Vascular Nodules on the Extremities and Trunk—Diagnosis Diagnosis: Angiokeratoma corporis diffusum with normal enzyme activities. Microscopic findings and clinical course Histologic examination of multiple verrucous leg lesions showed sharply demarcated, verrucous epidermal hyperplasia; numerous dilated, papillary,dermal blood vessels adjacent to the epidermis; and subcorneal and dermal erythrocyte extravasation, consistent with angiokeratoma. Differentiationfrom verrucous hemangioma was made on the basis of the lack of deep dermal or subcutaneous extension in all the skin lesions sampled. Tissue cultures were positive for mixed flora and negative for fungi and mycobacteria. The results of the following laboratory investigations werenormal: blood chemistry profile, complete blood cell count, and 24-hour urine collection. Serologic testing revealed negativity for human immunodeficiencyvirus and normal levels of the following enzymes: α-galactosidase A, α-fucosidase, β-galactosidase, and β-mannidase. Also, electron microscopy revealed no lysosomal inclusionsin the endothelial cells, pericytes, or fibroblasts. The patient was treated with systemic antibiotics and periodic surgical debulking of numerous angiokeratomas on his legs, which resulted in significantlyimproved control of chronic cutaneous infection and lower-extremity bleeding. A tracheostomy for obstructive sleep apnea and an aggressive weight loss regimenalso improved management of his skin lesions. Discussion Angiokeratoma corporis diffusum is most commonly associated with Anderson-Fabry disease, due to a deficiency of α-galactosidase A. However, widespreadangiokeratomas may be present in at least 7 other metabolic disorders as well as in individuals with normal enzyme activities.1-3 Toour knowledge, 10 cases of angiokeratoma corporis diffusum with normal enzymeactivities have been reported, typically with skin lesions smaller than 5mm in diameter on the buttocks, thighs, and genitals.4,5 Our case is unique in the extensive skin involvement and clinical similarity toverrucous hemangioma, particularly on the lower extremities. In 1967, Imperial and Helwig6,7 established the difference between verrucous hemangioma and angiokeratoma. While bothconditions are characterized by ectatic vessels, verrucous hemangioma extends into the subcutaneous tissue, whereas angiokeratoma does not extend beyondthe papillary dermis.7,8 Individuals with angiokeratoma corporis diffusum (with or without an underlying metabolicdisorder) develop lesions beginning in late childhood or adolescence, whereas verrucous hemangioma presents at birth and becomes more noticeable with age.Verrucous hemangioma may be particularly troublesome owing to frequent bleeding after minimal trauma, secondary bacterial infection, and progressive enlargement.8 In contrast, angiokeratomas are typically smallerand exhibit stable size over time. Management of verrucous hemangioma using electrocautery, intralesional or topical corticosteroids, argon laser, and cryosurgery has been reported.9 However, high recurrence rates are common, asthe subcutaneous components of the lesion must be destroyed for complete eradication. The current preferred treatment for verrucous hemangioma is surgical excisionin combination with laser therapy to avoid partial removal and recurrence.9 In comparison, because angiokeratoma is not as deep, superficial ablative therapies are typically useful. Successful treatmentof multiple angiokeratomas has been reported using argon, copper vapor, or frequency-doubled Nd:YAG lasers. Our case is of interest because of the unusual presentation of angiokeratoma corporis diffusum with normal enzyme activities. To our knowledge, none ofthe previously reported cases demonstrated skin lesions up to 4 cm in diameter or such extensive involvement of the legs, features that are more characteristicof verrucous hemangioma. Since the correct histologic diagnosis was made, our patient has had a successful outcome with periodic surgical debulkingand electrocautery of the larger angiokeratomas on his legs. References 1. Epinette WWNorins ALDrew ALZeman WPatel V Angiokeratoma corporis diffusum with an L-fucosidase deficiency Arch Dermatol. 1973;107754- 757PubMedGoogle ScholarCrossref 2. Ishibashi ATsuboi RShinmei M β-Galactosidase and neuraminidase deficiency associated with angiokeratoma corporis diffusum Arch Dermatol. 1984;1201344- 1346PubMedGoogle ScholarCrossref 3. Kanzaki TYokota MMizuno NMatsumoto YHirabayashi Y Novel lysosomal glycoaminoacid storage disease with angiokeratoma corposis diffusum Lancet. 1989;1875- 877PubMedGoogle ScholarCrossref 4. Crovato FRebora A Angiokeratoma corporis diffusum and normal enzyme activities J Am Acad Dermatol. 1985;12885- 886PubMedGoogle ScholarCrossref 5. Marsden JAllen R Widespread angiokeratoma without evidence of metabolic disease Arch Dermatol. 1987;1231125- 1127PubMedGoogle ScholarCrossref 6. Imperial RHelwig EB Verrucous hemangioma: a clinicopathologic study of 21 cases Arch Dermatol. 1967;96247- 253PubMedGoogle ScholarCrossref 7. Imperial RHelwig EB Angiokeratoma: a clinicopathological study Arch Dermatol. 1967;95166- 175PubMedGoogle ScholarCrossref 8. Rossi ABozzi MBarra E Verrucous hemangioma and angiokeratoma circumscriptum: clinical and histologic differential characteristics J Dermatol Surg Oncol. 1989;1588- 91PubMedGoogle ScholarCrossref 9. Yang CHOhara K Successful surgical treatment of verrucous hemangioma: a combined approach Dermatol Surg. 2002;28913- 919PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Diffuse Verrucous, Vascular Nodules on the Extremities and Trunk—Diagnosis

Archives of Dermatology , Volume 140 (3) – Mar 1, 2004

Diffuse Verrucous, Vascular Nodules on the Extremities and Trunk—Diagnosis

Abstract

Diagnosis: Angiokeratoma corporis diffusum with normal enzyme activities. Microscopic findings and clinical course Histologic examination of multiple verrucous leg lesions showed sharply demarcated, verrucous epidermal hyperplasia; numerous dilated, papillary,dermal blood vessels adjacent to the epidermis; and subcorneal and dermal erythrocyte extravasation, consistent with angiokeratoma. Differentiationfrom verrucous hemangioma was made on the basis of the lack of deep dermal or subcutaneous...
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Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.3.353-b
Publisher site
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Abstract

Diagnosis: Angiokeratoma corporis diffusum with normal enzyme activities. Microscopic findings and clinical course Histologic examination of multiple verrucous leg lesions showed sharply demarcated, verrucous epidermal hyperplasia; numerous dilated, papillary,dermal blood vessels adjacent to the epidermis; and subcorneal and dermal erythrocyte extravasation, consistent with angiokeratoma. Differentiationfrom verrucous hemangioma was made on the basis of the lack of deep dermal or subcutaneous extension in all the skin lesions sampled. Tissue cultures were positive for mixed flora and negative for fungi and mycobacteria. The results of the following laboratory investigations werenormal: blood chemistry profile, complete blood cell count, and 24-hour urine collection. Serologic testing revealed negativity for human immunodeficiencyvirus and normal levels of the following enzymes: α-galactosidase A, α-fucosidase, β-galactosidase, and β-mannidase. Also, electron microscopy revealed no lysosomal inclusionsin the endothelial cells, pericytes, or fibroblasts. The patient was treated with systemic antibiotics and periodic surgical debulking of numerous angiokeratomas on his legs, which resulted in significantlyimproved control of chronic cutaneous infection and lower-extremity bleeding. A tracheostomy for obstructive sleep apnea and an aggressive weight loss regimenalso improved management of his skin lesions. Discussion Angiokeratoma corporis diffusum is most commonly associated with Anderson-Fabry disease, due to a deficiency of α-galactosidase A. However, widespreadangiokeratomas may be present in at least 7 other metabolic disorders as well as in individuals with normal enzyme activities.1-3 Toour knowledge, 10 cases of angiokeratoma corporis diffusum with normal enzymeactivities have been reported, typically with skin lesions smaller than 5mm in diameter on the buttocks, thighs, and genitals.4,5 Our case is unique in the extensive skin involvement and clinical similarity toverrucous hemangioma, particularly on the lower extremities. In 1967, Imperial and Helwig6,7 established the difference between verrucous hemangioma and angiokeratoma. While bothconditions are characterized by ectatic vessels, verrucous hemangioma extends into the subcutaneous tissue, whereas angiokeratoma does not extend beyondthe papillary dermis.7,8 Individuals with angiokeratoma corporis diffusum (with or without an underlying metabolicdisorder) develop lesions beginning in late childhood or adolescence, whereas verrucous hemangioma presents at birth and becomes more noticeable with age.Verrucous hemangioma may be particularly troublesome owing to frequent bleeding after minimal trauma, secondary bacterial infection, and progressive enlargement.8 In contrast, angiokeratomas are typically smallerand exhibit stable size over time. Management of verrucous hemangioma using electrocautery, intralesional or topical corticosteroids, argon laser, and cryosurgery has been reported.9 However, high recurrence rates are common, asthe subcutaneous components of the lesion must be destroyed for complete eradication. The current preferred treatment for verrucous hemangioma is surgical excisionin combination with laser therapy to avoid partial removal and recurrence.9 In comparison, because angiokeratoma is not as deep, superficial ablative therapies are typically useful. Successful treatmentof multiple angiokeratomas has been reported using argon, copper vapor, or frequency-doubled Nd:YAG lasers. Our case is of interest because of the unusual presentation of angiokeratoma corporis diffusum with normal enzyme activities. To our knowledge, none ofthe previously reported cases demonstrated skin lesions up to 4 cm in diameter or such extensive involvement of the legs, features that are more characteristicof verrucous hemangioma. Since the correct histologic diagnosis was made, our patient has had a successful outcome with periodic surgical debulkingand electrocautery of the larger angiokeratomas on his legs. References 1. Epinette WWNorins ALDrew ALZeman WPatel V Angiokeratoma corporis diffusum with an L-fucosidase deficiency Arch Dermatol. 1973;107754- 757PubMedGoogle ScholarCrossref 2. Ishibashi ATsuboi RShinmei M β-Galactosidase and neuraminidase deficiency associated with angiokeratoma corporis diffusum Arch Dermatol. 1984;1201344- 1346PubMedGoogle ScholarCrossref 3. Kanzaki TYokota MMizuno NMatsumoto YHirabayashi Y Novel lysosomal glycoaminoacid storage disease with angiokeratoma corposis diffusum Lancet. 1989;1875- 877PubMedGoogle ScholarCrossref 4. Crovato FRebora A Angiokeratoma corporis diffusum and normal enzyme activities J Am Acad Dermatol. 1985;12885- 886PubMedGoogle ScholarCrossref 5. Marsden JAllen R Widespread angiokeratoma without evidence of metabolic disease Arch Dermatol. 1987;1231125- 1127PubMedGoogle ScholarCrossref 6. Imperial RHelwig EB Verrucous hemangioma: a clinicopathologic study of 21 cases Arch Dermatol. 1967;96247- 253PubMedGoogle ScholarCrossref 7. Imperial RHelwig EB Angiokeratoma: a clinicopathological study Arch Dermatol. 1967;95166- 175PubMedGoogle ScholarCrossref 8. Rossi ABozzi MBarra E Verrucous hemangioma and angiokeratoma circumscriptum: clinical and histologic differential characteristics J Dermatol Surg Oncol. 1989;1588- 91PubMedGoogle ScholarCrossref 9. Yang CHOhara K Successful surgical treatment of verrucous hemangioma: a combined approach Dermatol Surg. 2002;28913- 919PubMedGoogle Scholar

Journal

Archives of DermatologyAmerican Medical Association

Published: Mar 1, 2004

Keywords: limb,trunk structure,fabry disease,enzymes

References