Skin nonmelanocytic tumor

• ICD-10
  • L98.8 - other specified disorders of the skin and subcutaneous tissue
  • D18.01 - hemangioma of skin and subcutaneous tissue
• ICD-11
  • LC50.Y - other specified cutaneous capillary vascular malformation
  • 2F2Y & XH5AW4 - other specified benign cutaneous neoplasms & hemangioma, NOS

• Ischemic events or inflammation may cause hypoxia at the tissue site, inducing an inflammatory response
  • Subsequent production of angiogenic factors, such as vascular endothelial growth factor (VEGFR) in response to diminished tissue oxygenation
  • Endothelial cells proliferate at these sites of ischemia, causing extravascular endothelial cell hyperplasia (Case Rep Dermatol 2017;9:194, Acta Derm Venereol 2020;100:adv00202)
• Some propose that microthrombi are created at sites of vessel occlusion or inflammation, resulting in tissue hypoxia and subsequent endothelial cell hyperplasia (Cutis 2019;103:181, J Am Acad Dermatol 2003;49:887)
• In cases with breast involvement, the weight of large, pendulous breasts is thought to cause compression and increased venous hydrostatic pressure
  • Subclinical trauma to breast tissue causes angiogenesis
• Other risk factors, such as insulin resistance, smoking and hypercoagulability, may contribute to endothelial cell dysfunction and vascular inflammation, precipitating local tissue ischemia (Case Rep Dermatol 2017;9:194)

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Images hosted on other servers:

• Diffuse proliferation of endothelial cells and surrounding pericytes in the interstitia of the papillary and reticular dermis, dissecting the dermal collagen bundles
• Endothelial cells form small capillary vessels with regular, small lumina
• Endothelial cells may be spindle shaped, vacuolated or haphazardly arranged in the collagen but there is no cytologic or nuclear atypia since this is a benign proliferation of capillary vessels
• Erythrocytes may be present within vessels or extravasated, as these are functional blood capillaries
• There may be thrombi in vessels, reflecting possible tissue hypoxia or underlying hypercoagulability
• There is no intravascular proliferation of endothelial cells within the vascular lumina as is seen in other cutaneous reactive angiomatosis
• References: Case Rep Dermatol 2017;9:194, Acta Derm Venereol 2020;100:adv00202

Contributed by Kayley Erickson, M.D. and Bethany R. Rohr, M.D.

A 55 year old woman with a past medical history of end stage renal disease (ESRD) (on dialysis), coronary cardiovascular disease (history of coronary artery bypass grafts) and obesity presents with a 6 month history of painful, ulcerative lesions on the bilateral breasts. A skin punch biopsy is shown. HHV8 is negative. What is the diagnosis?

1. Angiosarcoma
2. Calciphylaxis
3. Diffuse dermal angiomatosis
4. Hemangioma
5. Kaposi sarcoma

C. Diffuse dermal angiomatosis. Diffuse dermal angiomatosis is a benign reactive angiomatosis to tissue ischemia that can present as painful, ulcerative and necrotic erythematous lesions often on the breast. It commonly affects middle aged women with comorbid conditions such as obesity, macromastia, smoking, atherosclerotic disease and ESRD. The slide above shows a proliferation of endothelial cells (confirmed by CD31 positivity) with regular, small vascular lumina interspersed in dermal collagen. Answer A is incorrect because angiosarcoma would show cytologic / nuclear atypia with mitotic figures and irregular vascular channels. Answer E is incorrect because Kaposi sarcoma would be HHV8 positive with spindled endothelial cells, inflammatory cell infiltrate and slit-like vascular lumina. Answer B is incorrect because calciphylaxis clinically resembles diffuse dermal angiomatosis but it would have subcutaneous calcifications, fibrointimal hyperplasia and thrombi in vessels. Answer D is incorrect because hemangioma is a benign proliferation of capillaries but it would not be expected to dissect collagen diffusely through the papillary and reticular dermis and would be a circumscribed lesion.

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Reference: Diffuse dermal angiomatosis

Which stain helps differentiate Kaposi sarcoma from a benign reactive angiomatosis such as diffuse dermal angiomatosis?

1. CD31
2. CD34
3. ERG
4. HHV8
5. Ki67

D. HHV8. Kaposi sarcoma is HHV8 positive as it is caused by the human herpesvirus 8. Diffuse dermal angiomatosis is not associated with HHV8. Answers A, B and C are incorrect because both Kaposi sarcoma and diffuse dermal angiomatosis stain positive for endothelial markers ERG, CD34 and CD31. Answer E is incorrect because Ki67 is a marker of cell proliferation that can be increased in angiosarcoma.

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Reference: Diffuse dermal angiomatosis