Chronic Localized Fibrosing Leukocytoclastic Vasculitis Associated With Lymphedema, Intralymphatic and Intravascular Lymphocytosis, and Chronic Myelogenous Leukemia: A Case Report of Unilateral Erythema Elevatum Diutinum

Abstract

One of the pathogenic causes of cutaneous inflammatory pseudotumors is chronic localized fibrosing leukocytoclastic vasculitis (CLFLCV), a vasculitic reaction pattern seen in granuloma faciale (GF), a localized vasculitis, and erythema elevatum diutinum (EED), a generalized vasculitis. Patients with myelodysplastic syndromes (MDSs) are at risk for a diverse spectrum of cutaneous neutrophilic dermatoses such as EED. Herein, we report a 74-year-old man who presented with a large ulcerative, fungating tumor affecting the right flexor ankle caused by CLFLCV. During his workup and management, MDS and Philadelphia chromosome-negative chronic myeloid leukemia was diagnosed. Surgical excision of the inflammatory mass promptly triggered tumor recurrence, whereas antineutrophil therapy (dapsone coupled with hydroxyurea, and prednisone) lead to tumor regression. Histopathologic examination revealed an eosinophilic-rich small-vessel neutrophilic vasculitis associated with storiform and angiocentric fibrosis (GF-like). In the regions of fibrosis, dilated lymphatic and vascular spaces were numerous, some of which were congested with small CD3-positive lymphocytes (intralymphatic and intravascular lymphocytosis). These findings indicate coexisting localized lymphedema. By direct immunofluorescence, IgM and C4d vessel deposits were detected. The pathogenesis of the fibrotic nodules and plaques of CLFLCV is suspected to be due to recurring bouts of immune-complex vasculitis, creating a cycle of vessel damage followed by reparative granulation tissue. Poor lymphatic drainage may be the underlying factor initiating and maintaining recurrent, localized episodes of immune-complex vasculitis and progressive fibrosis. Although his tumor histopathology resembled GF-eosinophilic rich CLFLCV-the clinical context points to a solitary and paraneoplastic case of EED.

Atypical Presentation of Erythema Elevatum Diutinum in a Patient With Hashimoto's Disease

“…Histopathologic findings correspond to the age of the lesion: newer onset lesions show neutrophilic infiltrate, while chronic lesions show fibrosis of vessel walls and leukocytoclasia [2]. Direct immunofluorescence can also demonstrate IgM and C4d complex deposition in the vessels [5]. Less common findings include lipid deposition, granuloma formation, and abscess formation [2,3].…”

Diagnostic and therapeutic challenges in erythema elevatum diutinum

“…In the case of chronic localized fibrosing leukocytoclastic vasculitis (CLFLCV) described by Carlson's group [8], the location associated with poor lymphatic drainage may be the underlying factor maintaining recurrent episodes of immune-complex vasculitis and progressive fibrosis leading to verrucous hard nodule or ulcerative plaque formation. Therefore solitary lesion with histopathological resemblance to granuloma faciale, such as eosinophilic-rich small-vessel neutrophilic vasculitis associated with storiform and angiocentric fibrosis may correspond to the paraneoplastic vasculitis, but also may represent a unique example of cutaneous inflammatory pseudotumor due to lymphatic drainage obstruction.…”

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

“…1 c), has been reported as occurring in the context of a wide range of HMs, mainly represented by monoclonal gammopathies, particularly of the IgA isotype [ 88 , 89 ], and myelodysplastic syndrome [ 90 ]. Other HMs more rarely described as associated with erythema elevatum diutinum are multiple myeloma [ 91 ], B-cell chronic lymphatic leukaemia [ 92 ], chronic myeloid leukaemia [ 93 ] and non-Hodgkin’s lymphoma [ 89 , 94 ]. Dermatologic manifestations usually precede the onset of the HM by years [ 89 ].…”