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• W2090424639 abstract "To the Editor: We report an interesting case of interstitial granulomatous dermatitis in a massive, infiltrating fasciitis-like pattern secondary to acute promyelocytic leukemia. A 28-year-old white female with a medical history significant for hyperthyroidism presented with bilateral large indurated erythematous plaques on her hips worrisome for cellulitis. Other presenting symptoms included fever, sore throat, headache, nausea, and vomiting. On physical examination, the left hip demonstrated a large, tender, erythematous, deeply indurated plaque (Fig. 1). There was a similar circumscribed area on the right thigh.FIGURE 1: Left hip plaque.Admission blood counts were suspicious for leukemia, and a subsequent bone marrow biopsy revealed acute promyelocytic leukemia (M3). A CT scan of the pelvic soft tissue showed extensive edema and stranding of the subcutaneous fat along the left flank and buttock, consistent with cellulitis. Inflammatory changes involved the lateral abdominal wall musculature, with edema between the facial planes. There was no evidence of abscess formation. Microscopic examination revealed marked edema of the papillary dermis with scattered neutrophils, lymphocytes, macrophages, and other inflammatory cells (Fig. 2). Extending deep into the reticular dermis and tracking down the septa into the subcutis was a more abundant infiltrate of predominantly chronic inflammatory cells including macrophages, scattered neutrophils, and other cells (Fig. 3). The infiltrate was accompanied by fibroblastic proliferation. There was no obvious monomorphic leukemic infiltrate. Myeloperoxidase stains showed scattered positive cells, many of which can be recognized as mature neutrophils. Occasional positive cells had a round or slightly irregular nucleus suggesting the possibility of promyelocytes. A CD68 stain showed far more positive cells than were seen in the myeloperoxidase stain, indicating that the bulk of the cellular infiltrate is composed of macrophages or monocytes (Fig. 4).FIGURE 2: Biopsy of left hip plaque. Macro.FIGURE 3: Chronic inflammatory infiltrate in the reticular dermis.FIGURE 4: CD68 staining highlighting macrophages/monocytes.The patient was treated with a multidrug regimen for her leukemia, and a repeat bone marrow on day 30 revealed no evidence of residual disease. Despite the chemotherapy and weeks of combination antibiotic therapy, the skin lesions failed to improve, and a repeat biopsy of the left flank revealed persistent histopathologic changes. Interstitial granulomatous dermatitis is a histopathologic condition initially described in 1993 by Ackerman et al.1,2 Clinically, it is associated with a wide variety of systemic disease states. More common associations include rheumatoid arthritis, lupus erythematosus, Sjögren syndrome, various systemic vasculitides, drug reactions, and myeloproliferative disorders including myelodysplastic syndrome.3-7 Less frequently, interstitial granulomatous dermatitis is seen with thyroiditis, hepatitis, inflammatory bowel disease, and infections.7 Clinical presentations can include subcutaneous linear cords, papules, nodules, or plaques most commonly on the extremities or trunk.8 Microscopically, there is a dense, diffuse infiltration of the reticular dermis by histiocytes often in a palisaded array associated with areas of collagen degeneration. The histiocytes form granulomas around the degenerating collagen and are accompanied by variable numbers of polymorphonuclear leukocytes and eosinophils.9 The etiology is unknown, but immune complex deposition in the dermal vessels has been postulated as a likely cause.10 The deposited immune complexes cause activation of complement and signal the neutrophils, which in concert cause damage of the dermal collagen. In our case, the granuloma formation was not pronounced; however, there was a diffuse infiltrate of histiocytic macrophages, neutrophils, lymphocytes, and eosinophils. Despite the fact that the patient had a leukemic process, there was no evidence of a clonal population in the area of the plaque. Donald C. Swing Jr, MD* Daniel J. Sheehan, MD* *Department of Pathology Wake Forest University Baptist Medical Center Winston-Salem, NC Omar P. Sangüeza, MD† †Department of Pathology and Dermatology Wake Forest University Baptist Medical Center Winston-Salem, NC R. W. Woodruff, MD‡ ‡Department of Pathology Wake Forest University Baptist Medical Center Winston-Salem, NC" @default.
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• W2090424639 date "2008-04-01" @default.
• W2090424639 modified "2023-09-27" @default.
• W2090424639 title "Interstitial Granulomatous Dermatitis Secondary to Acute Promyelocytic Leukemia" @default.
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• W2090424639 doi "https://doi.org/10.1097/dad.0b013e318162af78" @default.
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