Pyoderma gangrenosum as a cause of splenomegaly and association with a T-cell clone

Abstract

The spectrum of clinical presentation of haematological disease is wide. We highlight two features of this principle: a rare cause of a 'haematological' presentation and a possible haematological cause of a disease not normally considered as such. A case of systemic pyoderma gangrenosum presented with splenomegaly in the absence of a rash. A clonal gamma- and beta-T-cell receptor rearrangement was demonstrated. Such clones may be a general phenomenon involved in the pathogenesis of this condition.

Effective Strategies for the Management of Pyoderma Gangrenosum

“…Keratinocytes and T cells are the two main sources of TNF in the skin. Correlating well with the possibility of an aberrant T cell response driving PG, T cell clonal expansions have been reported in patients with PG, 11,12 and lymphocytes are present in early PG lesion. 13 Thus, clonally expanded T cells may secrete TNF, which, in turn, drives overexpression of IL-8.…”

Effective Strategies for the Management of Pyoderma Gangrenosum: A Comprehensive Review

“…Keratinocytes and T cells are the two main sources of TNF in the skin. Supporting the possibility of an aberrant T-cell response driving PG, two groups have demonstrated the presence of T-cell clonal expansions in PG patients (42,43). Although these expansions have not been further characterized (45), they likely appear early-on in the course of disease (44).…”

“…In addition to IL-8, elevations of IL-1β, IL-6, interferon (IFN)-γ, G-CSF, tumour necrosis factor (TNF), matrix metallopaptidase (MMP)-9, MMP-10, and Elafin have all been reported (37)(38)(39)(40). TNF is a cytokine also well known to be associated with IBD (12,13,(17)(18)(19) and it is not surprising that the TNF-targeting biologics infliximab, etanercept and adalimumab, have all been successfully used to treat PG (41)(42)(43)(44). Of note, TNF is also known to induce the secretion of IL-8, which is a strong chemotactic factor for neutrophils, the predominant inflammatory cell type seen in PG biopsy specimens.…”

Systemic sclerosis in association with peristomal pyoderma gangrenosum

“…Pyoderma gangrenosum mainly shows T‐cell abnormalities. In a case of systemic pyoderma gangrenosum that presented with splenomegaly, clonal γ and β T‐cell receptor rearrangement was demonstrated 8 . The common T‐cell abnormality in the two diseases may have contributed to their association in the present case.…”