Disseminated cutaneous granulomatous eruption occurring in the setting of myelodysplasia
Published Web Location
https://doi.org/10.5070/D35xp039v4Main Content
Disseminated cutaneous granulomatous eruption occurring in the setting of myelodysplasia
Kenneth A Katz MD MSc
Dermatology Online Journal 9(4): 22
From the Ronald O. Perelman Department of Dermatology, New York University
Abstract
A 66-year-old woman with a myelodysplastic syndrome developed a widespread, pruritic, nodular eruption. A skin-biopsy specimen showed a granulomatous infiltrate, with no evidence of clonality; a bone-marrow biopsy showed possible transition to acute myeloid leukemia. This presentation is consistent with two prior reports of granulomatous eruptions occurring in the setting of myelodysplasia.
Clinical summary
History.—A 66-year-old woman presented with a 3-month history of a generalized eruption. The patient, who is a candidate for a male-to-female transsexual operation, came to the Bellevue Hospital Emergency Department in 1999, requesting an evaluation of a supraventricular tachycardia. A physical examination was normal. A laboratory examination showed a platelet count of 19,000 x 109/L. White-cell count and hematocrit were normal. A bone-marrow biopsy obtained in April 2000 showed mild hypercellularity. A course of prednisone (60 mg daily) was initiated, with tapering and subsequent discontinuation after approximately 1 year. The platelet count has ranged between 40,000 x 109/L and 60,000 x 109/L since initiation of prednisone therapy.
A second bone-marrow biopsy specimen obtained in April 2001 showed mild hypercellularity and dysplastic megakaryocytic and erythroid elements, findings consistent with a myelodysplastic syndrome. The oncology service recommended treatment wih chemotherapy, but the patient declined.
In mid-December 2001 an eruption developed on the right arm and subsequently spread to the rest of the body. Pruritus affected the whole body and was not limited to the lesions. There were also lumps in the inguinal area. The patient denied recent fevers, chills, or fatigue. An intentional 30-pound weight loss occurred over the past 18 months. The patient presented for evaluation in the Bellevue Hospital Center dermatology clinic, where a biopsy specimen was obtained. Treatment was initiated with diphyenhydramine (50 mg four times a day) as well as with clobetasol (0.05 %) ointment applied twice daily to lesions on the body and triamcinolone (0.1 %) ointment applied twice daily to lesions on the face. The pruritus has diminished, and the lesions have decreased in extent.
Additional skin and bone-marrow biopsy specimens were obtained in January 2002. The bone-marrow biopsy specimen showed a hypercellular marrow with a left-shifted myeloid series and dysplastic megakaryocytic and erythroid elements. There were no granulomas. These findings were interpreted as suggestive of myelodysplasia with proliferation and concern for a transition to acute myeloid leukemia.
Physical examination.—Numerous, well-demarcated, nontender, erythematous nodules, ranging in diameter from 1-5 cm, were present diffusely, especially on the face, scalp, and neck. Bilateral, nontender lympadenopathy was noted in the inguinal areas only. There was no hepatosplenomegaly or oral lesions.
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Laboratory data.—A complete blood count obtained in late January 2002, showed a white-cell count of 30.2 x 109/L with 4 percent neutrophils, 20 percent lymphocytes, 66 percent monocytes, and 10 percent atypical cells. The hematocrit was 31 percent, platelet count 58,000 x 109/L, and erythrocyte sedimentation rate 5 mm/hr. Anti-platelet IgG titers were elevated. Anti-nuclear antibody and a rapid plasma reagin test were negative. A comprehensive metabolic panel, including liver function tests, and serum and urine protein electrophoreses were normal. IgG titers, but not IgM titers, were elevated for cytomegalovirus and Epstein-Barr virus. Hepatitis B and C serologies were negative.
Histopathology.—There is a superficial and deep, dense, perivascular, periadnexal, and multinodular infiltrate composed predominantly of mononuclear histiocytes admixed with small lymphocytes and plasma cells. Fite and periodic acid-Schiff stains for acid-fast bacilli and fungi, respectively, are negative. Immunostains show that histiocytes react positively with lysozyme, HAM 56, and to a lesser degree with KP-1. Leukocyte common antigen stain highlights the lymphocytes in the infiltrate that react predominantly with CD3.
Diagnosis.—Disseminated cutaneous granulomatous eruption in a patient with a myelodyplastic syndrome and possible transition to acute myeloid leukemia.
Comment
Myelodysplastic syndromes are preleukemic disorders that are characterized genetically by clonal expansion, and clinically by ineffective hematopoiesis and resultant cytopenias [1]. The median age of onset is the seventh decade, and 60 percent of cases are men. Cutaneous eruptions in myelodysplastic syndromes may herald transition to acute myeloid leukemia and are associated with poorer prognoses [2]. The specific cutaneous manifestation of myelodysplastic syndromes, a dermal infiltrate of malignant hematopoietic cells, is rare. More commonly seen are nonspecific manifestations, which include petechiae and purpura, cutaneous infections, vasculitis, and neutrophilic dermatoses (especially Sweet syndrome, but also pyoderma gangrenosum, subcorneal pustular dermatosis, and erythema elevatum diutinum). Other cutaneous manifestations are more rarely observed [2, 3, 4, 5].
Only two cases of disseminated, cutaneous, granulomatous eruptions associated with myelodysplastic syndrome have been reported [6]. In one case, a 66-year-old man presented with a widespread papular eruption. A perivascular lymphocytic infiltrate was present in the dermis with granulomas, rare giant cells, and central necrobiosis. A diagnosis of myelodysplastic syndrome was made 2 months later and the patient progressed to acute myeloid leukemia after 1 month. The patient was treated with chemotherapy and was in remission after 1 year. In the second case, a 71-year-old man with myelodysplastic syndrome developed a papular eruption that on histological examination showed epitheloid granulomas with numerous giant cells. The patient had not developed acute myeloid leukemia after 1 year.
The percentage of patients with myelodysplastic syndrome who progress to acute myeloid leukemia is higher in patients with greater percentages of blasts in the bone marrow [1]. Patients with preexisting myelodysplastic syndrome who develop acute myeloid leukemia tend to have less favorable prognoses than do patients without preexisting myelodysplastic syndrome [7]. The specific cutaneous manifestation of acute myeloid leukemia, leukemia cutis, develops in between 10-50 percent of patients, and often is associated with the relatively specific finding of gingival involvement [8]. In a case series of seventeen acute myeloid leukemia patients with leukemia cutis from the University of Graz in Austria, ten patients presented with single or multiple erythematous-to-violaceous papules, plaques, or nodules, and seven patients presented with a generalized erythematous-to-brown maculopapular eruption [9]. In seven of these cases the cutaneous eruption represented the first sign of acute myeloid leukemia. One patient presented with aleukemic leukemia cutis, and the hematologic signs of acute myeloid leukemia appeared 2 months after the cutaneous eruption [8]. As in myelodysplastic syndrome, neutrophilic dermatoses are common nonspecific manifestations of acute myeloid leukemia. Sarcoidosis also has been associated with acute myeloid leukemia [10, 11].
References
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