Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report

Table 2 Summary of perimysial/endomysial expression pattern of applied immunohistochemical markers in assessed myocardial and skeletal muscle tissue samples

Applied marker	Myocardial muscle	Skeletal muscle
CD³	+ + / +	+ / –
CD⁴	+ + / +	+ / –
CD⁸	+ / –	– / –
CD²⁰	– / –	– / –
CD²⁵	– / –	– / –
CD³⁸	+ / –	– / –
CD⁵⁶	+ / –	– / –
CD^79a	0 / 0	0 / 0
T-cell intracellular antigen-1	+ / +	0 / –

Legend to symbols: ++ = high expression, + = moderate expression, 0 = sporadic expression, – = no expression.
Legend to Table 2: Immunohistochemical expression pattern of lymphocytic specific markers revealed more frequent expression of CD3 in myocardial than in skeletal muscle tissue. In addition, within the CD3 positive T-cells CD4 positive cells were higher expressed than CD8 positive cells indicating a majority of the T-cell-helper-phenotype (CD4⁺) than of the T-cell-regulatory-phenotype (CD8⁺). This was supported by the perimysial expression of CD38 – a marker for activated CD4⁺-T-cells; and the lack of CD25 – a marker for activated CD8⁺-T-cells. Finally, evidence for an activated innate immune system was provided by the specific expression of CD56 and T-cell intracellular antigen (specific marker for a subpopulation of natural killer cells). Overall, immunohistochemical expression analysis revealed a myocardial-associated lymphatic-disordered status in dermatomyositis.

ISSN: 1752-1947