A case of palisaded neutrophilic granulomatous dermatitis with subsequent development of chronic myelomonocytic leukemia

Abstract

Key Clinical Message Palisaded neutrophilic granulomatous dermatitis is a cutaneous marker of a systemic disease. Clinicians’ goal should be directed toward determining an underlying condition. Even if the initial investigation is inconclusive, it may be necessary that some tests are repeated, since a serious underlying disease could be revealed in the course of time.

Unique Recognizable Histopathologic Variant of Palisaded Neutrophilic and Granulomatous Dermatitis that Is Associated With SRSF2-Mutated Chronic Myelomonocytic Leukemia: Case Report and Review of the Literature

“…On review of the literature, there have been 4 previous reports of PNGD occurring in the context of CMML. 3 Our case is noteworthy because it has a striking similarity to 3 patients described in detail by Federman et al 2017 in which a diagnosis of PNGD preceded identification of underlying CMML. All patients in that series presented with a widespread erythematous to brown papular eruption involving the trunk and extremities.…”

“…The association between PNGD and hematologic malignancies, particularly chronic myelomonocytic leukemia (CMML), is rare with only a few scattered reports in the literature. 2,3 CMML is a malignant hematopoietic disorder with features of a myeloproliferative neoplasm and myelodysplastic syndrome. 4 Peripheral blood monocytosis and bone marrow dysplasia characterize this form of leukemia, which may also be accompanied by cytopenias and splenomegaly.…”

Palisaded neutrophilic and granulomatous dermatitis following a long-standing monoclonal gammopathy: A case report

“…PNGD was first discovered in patients with rheumatoid arthritis and hence primarily named rheumatoid papules and IGD with cutaneous cords and arthritis. 1 Subsequently, more clinicians observed similar features in other autoimmune conditions such as pediatric sarcoidosis, 4 Takayasu arteritis, 2 systemic lupus erythematosus, and EGPA.…”

“… 5 Recent studies have shown that different biopsies from multiple lesions within one patient with PNGD may present distinctively under the microscope. 2 …”

“…Palisaded neutrophilic granulomatous dermatitis (PNGD) is a neutrophilic skin condition and a well-reported cutaneous marker for a number of systemic associations including rheumatoid arthritis, lupus erythematosus, vasculitis, 1 lymphoproliferative disorders, 2 and drug reactions. 3 The clinical presentation of PNGD is diverse and often includes tender erythematous to violaceous papules or plaques on neck and extensor surfaces.…”