Case Reports

Diffuse Dermal Angiomatosis

Cutis. 2019 March;103(3):181-184

References

Krell JM, Sanchez RL, Solomon AR. Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis. J Cutan Pathol. 1994;21:363-370.
Rongioletti F, Robora A. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am Acad Dermatol. 2003;49:887-896.
Crickx E, Saussine A, Vignon-Pennamen MD, et al. Diffuse dermal angiomatosis associated with severe atherosclerosis: two cases and review of the literature. Clin Exp Dermatol. 2015;40:521-524.
Reusche R, Winocour S, Degnim A, et al. Diffuse dermal angiomatosis of the breast: a series of 22 cases from a single institution. Gland Surg. 2015;4:554-560.
Sriphojanart T, Vachiramon V. Diffuse dermal angiomatosis: a clue to the diagnosis of atherosclerotic vascular disease. Case Rep Dermatol. 2015;7:100-106.
Kimyai-Asadi A, Nousari HC, Ketabchi N, et al. Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with atherosclerosis. J Am Acad Dermatol. 1999;40:257-259.
Bassi A, Arunachalam M, Maio V, et al. Diffuse dermal angiomatosis in a patient with an iatrogenic arterio-venous fistula and Wegener’s granulomatosis. Acta Derm Venereol. 2013;93:93-94.
Ormerod E, Miller K, Kennedy C. Diffuse dermal angiomatosis: a contributory factor to ulceration in a patient with renal transplant. Clin Exp Dermatol. 2015;40:48-51.
Kim S, Elenitsas R, James WD. Diffuse dermal angiomatosis: a variant of reactive angioendotheliomatosis associated with peripheral vascular atherosclerosis. Arch Dermatol. 2002;138:456-458.
Requena L, Fariña MC, Renedo G, et al. Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas. J Cutan Pathol. 1999;26:159-164.
Villa MT, White LE, Petronic-Rosic V, et al. The treatment of diffuse dermal angiomatosis of the breast with reduction mammoplasty. Arch Dermatol. 2008;144:693-694.
Halbesleben JJ, Cleveland MG, Stone MS. Diffuse dermal angiomatosis arising in cutis marmorata telangiectatica congenita. Arch Dermatol. 2010;146:1311-1313.
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Steele KT, Sullivan BJ, Wanat KA, et al. Diffuse dermal angiomatosis associated with calciphylaxis in a patient with end-stage renal disease.J Cutan Pathol. 2013;40:829-832.
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Acroangiodermatitis, or pseudo–Kaposi sarcoma, presents as slow-growing, erythematous to violaceous, brown, or dusky macules, papules, or plaques of the legs.¹⁴ Histologically, acroangiodermatitis presents with relatively less proliferation of endothelial cells found intravascularly rather than extravascularly, as in DDA, forming new thick-walled vessels in a lobular pattern in the papillary dermis.¹⁴

Vascular malignancies, such as Kaposi sarcoma and angiosarcoma, may present similarly to DDA. Kaposi sarcoma, for example, presents as erythematous to violaceous patches, plaques, or nodules found mostly on the extremities.⁷ Histologically, spindle cells and vascular structures also are found but in a clefting pattern representative of Kaposi sarcoma (so-called vascular slits).⁷ Diffuse dermal angiomatosis and vascular malignancies can further be distinguished based on atypia of the proliferations and staining for HHV-8.^7,14 Lastly, DDA differs from vascular tumors in that vascular tumors are reactive to locations of occluded vessels, with vascular proliferation ceasing once the underlying cause of hypoxia is removed.²

Treatment
There is no standard treatment of DDA.⁷ Treatment of the underlying cause of ischemia is the primary goal, which will cause the DDA to resolve in most cases. Stenting, removal of an arteriovenous fistula, or other forms of revascularization may be warranted.^{1,5,6,10,17,29,30}

Reported medical therapies for DDA include systemic or topical corticosteroids used for their antiangiogenic properties with varying results.⁷ Isotretinoin also has been used, which has been found to be effective in several cases of DDA of the breast, though 1 study reported a subsequent elevated lipid profile, requiring a decrease in dosage.^14,15,27,31

Most interestingly, a study by Sanz-Motilva et al¹⁶ demonstrated that control of comorbidities, especially smoking cessation, led to improvement, which highlights the importance of incorporating nonpharmacotherapy rather than initiating treatment solely with medication. The Table summarizes treatments used and their efficacy.

Conclusion

Diffuse dermal angiomatosis is associated with medical conditions that predispose an individual to ischemia. Although rare, DDA can present as painful and visibly disturbing lesions that can affect the daily life of afflicted patients. By reporting the few cases that do arise and reviewing prior cases and their treatments, physicians can consider DDA within the differential diagnosis and identify which treatment is most efficient for a given patient. For all DDA patients, strict control of comorbidities, especially smoking cessation, should be incorporated into the treatment plan. When DDA affects the breasts, isotretinoin appears to provide the best relief. Otherwise, treatment of the underlying cause, revascularization, withdrawal of the offending agent, or steroids seem to be the best treatment options.

Diffuse Dermal Angiomatosis

References

Conclusion

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