Extramedullary Hemopoiesis of the Skin

Deba P. Sarma, MD
r

Phe skin is a potential site for extramedullary hemopoiesis since hemopoiesis occurs in the skin in early embryonic life. However, a review of the English literature reveals" only five reported cases of extramedullary hemopoiesis of the skin:1' All of these patients suffered from myelofibrosis. I report herein another case of myelofibrosis associated with papulonodular and plaque-like lesions of the skin that microscopically exhibited extramedullary hemopoiesis.
Report of a Case A 64-year-old man with fever of unknown origin that had been present for 14 months was admitted to the Veterans Administration Medical Center, New Orleans, in March 1974. Physical examination showed a nontender, palpable liver, 5 cm below the right costal margin, and a palpable spleen, 3 cm below the left costal margin. There was no lymphadenopathy. Hematologic studies indicated the following values: hemoglobin, 9.1 g/ dL; hematocrit, 31.7%; RBCs, 3,900,000/cu mm; WBC, 8,300/cu mm; differential cell count, normal; and platelets, 186,000/cu mm. Other tests included blood culture tests, febrile agglutinin tests, lupus erythematosus cell preparation, skin test for tuberculosis, and serologic tests for fungi. All of these yielded negative results. Results of roentgenologic studies of the chest and gastrointestinal tract were within normal limits except for the finding of a widened duodenal loop, indicating a mass in the pancreas. Angiograms did not show any tumor vessels in the region of the pancreas. Scans using technetium Tc 99m sulfur colloid showed an enlarged liver and spleen and minimal localization in the region of the pancreas indicating chronic pancreatitis or tumor. A tentative diagnosis of lymphoma was made. An exploratory laparotomy and splenectomy were performed. No mass was noted in the pancreas. Microscopic examination of the spleen (which weighed 420 g), liver tissue, and the lymph nodes that were removed at the time of exploration did not yield any specific diagnosis. The results of microbiologic studies of the tissues were normal. Subsequently, the patient underwent a bone marrow biopsy that established a diagnosis of myelofibrosis. On examinations during the following months, the patient was found to have numerous asymptomatic plaque-like and papulonodular lesions on the skin of the arms, legs, feet, chest, and back. The lesions ranged from 0.4 to 1.5 cm in diameter. The plaque-like lesions were pink-tan with a zone of hemorrhage around them. Some of them were ulcerated. The papulonodular lesions were pink and were elevated 0.2 to 0.3 cm above the skin surface. A biopsy specimen of a nodular lesion showed a polymorphic cellular infiltration of the dermis consisting of myeloid and erythroid cells and megakaryocytes (Fig 1 and 2). The cellular infiltrate was particularly abundant around the vessels and skin appendages. The patient was treated symptomatically on an outpatient basis. In September 1975, after progressive deterioration, the patient died of acute pulmonary edema. At autopsy, fibrosis of the bone marrow, hepatomegaly, generalized lymphadenopathy, and numerous cutaneous plaque-like and papulonodular lesions were found. Extramedullary hemopoiesis was noted in the liver, lymph nodes, and skin lesions, as well as in an accessory spleen, epicardial adipose tissue, the kidneys, the adrenal glands, the lungs, and the" meninges.

Fig 1 .Skin biopsy specimen showing dermal infiltrate composed of erythroid and myeloid cells and megakaryocytes (arrows) (hematoxylin-eosin, x 60).

Fig 2.Higher magnification of specimen in Fig 1 showing megakaryocyte (left arrow) and large myeloid cells (right arrow) (hematoxylin-eosin, x 240).

From the Department of Pathology, Veterans Administration Medical Center, New Orleans. Keprint requests to Department of Pathology, VA Medical Center, 1601 Perdidu St, New Orleans, LA 70146 (Dr Sarma).

58

Arch Dermatol-Vol 117. Jan 1981

Extramedullary Hemopolesls-Sarnw

Comment The skin lesions of extramedullary hemopoiesis have been described as nodular, erythematous, infiltrative, and/ or papular.2"' In the present case, plaque-like lesions with hemorrhagic borders were also present. Microscopic examination of the cellular infiltrates of the skin showed only cells of the myeloid series in three reported cases.1-3 Cells of both myeloid and erythroid series were seen in one case.2 In only one patient, a 74-year-old man with myelofibrosis, were cells of myeloid, erythroid, and megakaryocytic series noted.' To the best of my knowledge, the present case seems to be the second documentation of extramedullary hemopoiesis of the skin with cells of all three series. The cause of this rare occurrence of cutaneous hemopoiesis is not clear. Some authors 5 have speculated that the removal of the spleen may stimulate hemopoiesis in unusual sites. Our patient did have a splenectomy before the

development of the skin lesions. In another reported case, 1 the patient had also undergone a splenectomy before the skin lesions appeared. A careful study of patients with myelofibrosis, especially after they have had splenectomies, may show additional cases of extramedullary hemopoiesis of the skin.
References
1. Pitcock JA, Reinhard EH, Justus BW, ct al: A clinical and pathological study of 70 cases of myelofibrosis. Ann Intern A/erf l%2;57:73-84. 2. Kuo T, Uhlemann J. Reinhard EH: Cutaneous cxtramodullary hematopoiesis: Report of a case. Arch Dermatal 1976;! 12:1302-1303. 3. Ortonne JP, Jcune R, Perrot H: Myeloid metaplasia of the skin in two patients suffering from primary myelofibrosis. Anh Dermatol 1977;! 13:1459. 4. Tagami H, Tashima M, Uehara N: Myelofibrosis with skin lesions. Br J Dermatol 1980;102:109-112. 5. Polliack A, Kosenmann E: Extramedullary hematopoietic tumonof the cranial dura mater. Ada llaematol 1969:41:43-48.

Sarma DP (1981): Extramedullary hemopoiesis of the skin. Arch Dermatol 117:58-59. PMID: 7458387 [PubMed - indexed for MEDLINE]