Cutaneous extramedullary hematopoiesis D75.9

Rare compensatory hematopoiesis in various systemic diseases; rarely as a localized event associated with skin tumors.

In newborns caused by intrauterine viral infections or hereditary spherocytosis, described there as blueberry muffin baby.

In adults, possible consequence of a myelodysplastic syndrome.

Very rarely as a local finding in connection with skin tumors (e.g. pilomatrixoma) or in spherocytosis (spherocytic cell anemia).

In neonates, see under Blueberry muffin baby.

In adults: wide spectrum of efflorescences of these specific lesions. This ranges from reddish-purple or pink spots, papules and nodules to blisters, hemorrhages and bilateral ulcerations.

Infiltrates in dermis and subcutis from differentially mature cells of hematopoiesis. Myeloid cells with vesicular nuclei and granulated cytoplasm. Sporadically also (mature cell) polymorphonuclear leukocytes. Furthermore, megakaryocytes and erythroblasts.

Immunohistology:

• Myeloid cells: positive for lysozyme, chloroacetate esterase, myeloperoxidase, elastase.
• Megakaryocytes: positive for factor VIII, CD 61.
• Erythroblasts: positive for glycophorin A.

treatment of the respective underlying disease.

1. Evole-Buselli M et al (1997) Neonatal dermal hematopoiesis associated with diffuse neonatal hemangiomatosis. Pediatric dermatol 14: 383-386
2. Kaddu S et al (1995) Extramedullary hematopoesis in pilomatrixomas. At J Dermatopathol 17: 126-130
3. Mizoguchi M et al (1990) Cutaneous extramedullary hematopoesis presenting as a leg ulcer. J Am Acad Dermatol 4: 592-596
4. Pizarro A et al (1995) Neonatal dermal erythropoiesis associated with severe rhesus immunization: amelioration by high-dose intravenous immunoglobulin. Br J Dermatol 133: 334-336
5. Vozza A et al (2003) Late onset Blueberry Muffin Syndrome following congenital rubella. J Eur Acad Dermatol Venereol 17: 204-205