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Myelofibrosis with skin lesions
The British Journal of Dermatology
|January 1, 1980
Summary
Primary myelofibrosis can manifest in the skin through papules and nodules. These lesions represent extra-medullary haematopoiesis, a finding previously unreported in cutaneous tissues.
Area of Science:
- Hematology
- Dermatopathology
Background:
- Primary myelofibrosis is a clonal hematopoietic stem cell disorder.
- It is characterized by bone marrow fibrosis and extra-medullary hematopoiesis.
Observation:
- An elderly male with primary myelofibrosis presented with skin lesions.
- Initially, papules appeared on the trunk, followed by subcutaneous nodules on the face.
Findings:
- Histological examination revealed extra-medullary haematopoiesis in the skin lesions.
- These lesions contained myeloid, erythroid, and megakaryocytic elements.
- This represents a novel cutaneous manifestation of myelofibrosis.
Implications:
- This case expands the known clinical spectrum of primary myelofibrosis.
- It highlights the importance of considering skin biopsy for diagnosing unusual presentations.
- Further research may elucidate the mechanisms of cutaneous extra-medullary haematopoiesis.