Granulocytic sarcoma is an extramedullary tumor of immature cells of granulocytic series, generally associated to acute myelogenous leukemia. The skin is one of the most commonly affected sites. Granulocytic sarcoma can complicate myelodysplastic syndromes and is considered a sign of poor prognosis. They are often misdiagnosed with non-Hodgkin lymphoma of the lymphoblastic type, Burkitt lymphoma and large cell lymphoma. In children, the differential diagnoses also include small, round cell tumors. It is important to diagnose these lesions early because they can precede peripheral blood and bone marrow transformation to acute myelogenous leukemia. We report a case of an elderly patient with myelodysplastic syndrome who developed multiple cutaneous granulocytic sarcoma lesions and discuss prognostic and treatment implications.
A 23‐year‐old, dark‐skinned man presented at the dermatology department with pigmented macules over the trunk and proximal thighs of 2 months’ duration. He reported that the number of lesions had increased progressively for a few weeks and then remained stable. Skin examination showed oval brown macules and patches, 5–40 mm in diameter, involving mainly the anterior trunk and proximal thigh, but also the neck and dorsum (Fig. 1). The lesions were asymptomatic. There was no previous history of an inflammatory process, erythema, scaling, or drug intake. The mucous membranes, palms, and soles were clear. The pigmentation was not influenced by sunlight. Darier's sign (urticaria or erythema around the macules after scratching or rubbing of the lesions) was absent. The previous use of emollients, keratolytics, and topical antifungal agents did not alter the aspect of the lesions. The results of physical examination and routine laboratory tests (blood cell count, blood chemistry) were normal. Venereal Disease Research Laboratory (VDRL) test and direct skin examination and culture for fungus were negative. A biopsy specimen was obtained from a pigmented macule. The histologic study showed hyperkeratosis and acanthosis, epidermal basal layer pigmentation with an irregular distribution, focal mild lymphohistiocytic infiltrate in the papillary dermis and dermal–epidermal junction, and discrete pigmentary incontinence (Figs 2–4). The mast cell population was normal. A further biopsy 1 year later showed similar findings.
1
Multiple brown macules involving the trunk
2
Mild inflammation in the papillary dermis and dermal–epidermal junction with focal lichenoid changes (hematoxylin and eosin, ×40)
3
Mild inflammation in the papillary dermis. Melanin pigmentation with an irregular distribution in the epidermal basal layer (hematoxylin and eosin, ×250)
4
Detail of the epidermal basal layer showing irregular melanin pigmentation; there are a few extravasated red cells and a melanophage in the papillary dermis (hematoxylin and eosin, ×400)
After 3 years of follow‐up, mild spontaneous fading occurred in some of the trunk lesions, but most remained unchanged.
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