Cutaneous extramedullary hematopoiesis is a rare cutaneous manifestation of myelofibrosis, where hematopoiesis occurs in the skin rather than the bone marrow, causing polymorphic skin lesions. The condition most often presents as bilateral lower extremity ulcers, but it may also cause papules, nodules, and plaques on any body part. These lesions typically begin as pink erythematous lesions, which become violaceous, hemorrhagic, and ulcerated over time. In addition, the lesions also tend to increase in number and size if the underlying hematological condition remains untreated.
(Of note, cutaneous extramedullary hematopoiesis is a normal occurrence during early embryogenesis, and it occurs until about the fifth month of gestation. Cutaneous extramedullary hematopoiesis in pre-term or full-term neonates is thought to be an accentuation of a normal physiologic process. These lesions resolve spontaneously 3-4 weeks after birth without intervention. Multiple reports suggest also that cutaneous extramedullary hematopoiesis in neonates may be precipitated by congenital infections, erythroblastosis fetalis, and twin transfusion syndrome.)
Cutaneous extramedullary hematopoiesis in Adult
Alerts and Notices
Synopsis
Codes
ICD10CM:
D75.9 – Disease of blood and blood-forming organs, unspecified
SNOMEDCT:
73241006 – Abnormal hematopoiesis
D75.9 – Disease of blood and blood-forming organs, unspecified
SNOMEDCT:
73241006 – Abnormal hematopoiesis
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Marjolin ulcer – Usually seen in previously traumatized skin.
- Fungal infection – Fungal components are seen on skin biopsy.
- Stasis ulcer – Stasis changes are typically present.
- Langerhans cell histiocytosis – Skin biopsy reveals infiltrates of Langerhans cells in close approximation to the epidermis.
- Mycobacterial infection – Skin biopsy, cultures, and polymerase chain reaction (PCR) studies are positive for mycobacterium; look for a history of travel and exposure.
- Parasitic infestation – Parasites are seen on skin biopsy; look for a history of travel and exposure.
- Chemical burn – Look for a history of chemical injury.
- Thermal burn – Look for a history of thermal injury.
- Radiation damage – Look for a history of radiation injury.
- Trauma – Look for a history of trauma.
- Neoplasm
- Vasculitis
Best Tests
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Management Pearls
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Therapy
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References
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Last Updated:03/10/2019
Cutaneous extramedullary hematopoiesis in Adult