Serum levels of high mobility group box 1 correlate with disease severity in recessive dystrophic epidermolysis bullosa.

[dystrophic epidermolysis bullosa]

In the inherited blistering skin disease , recessive dystrophic epidermolysis bullosa (RDEB ), there is clinical heterogeneity with variable scarring and susceptibility to malignancy . Currently , however , there are few biochemical markers of tissue inflammation or disease progression . We assessed whether the non-histone nuclear protein , high mobility group box 1 (HMGB 1 ), which is released from necrotic cells (including keratinocytes in blister roofs ), might be elevated in RDEB and whether this correlates with disease severity . We measured serum HMGB 1 by ELISA in 26 RDEB individuals (median 21 .0 ng /ml , range 3 .6 -54 .9 ng /ml ) and 23 healthy controls (median 3 .6 , range 3 .4 -5 .9 ng /ml ) and scored RDEB severity using the Birmingham Epidermolysis Bullosa Severity Score (BEBSS ; mean 34 /100 , range 8 -82 ). There was a positive relationship between the BEBSS and HMGB 1 levels (r = 0 .54 , P = 0 .004 ). This study indicates that serum HMGB 1 levels may represent a new biomarker reflecting disease severity in RDEB .

Diseases
Validation

Diseases presenting "scarring" symptom

acute rheumatic fever

cutaneous mastocytosis

cystinuria

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erythropoietic protoporphyria

focal myositis

junctional epidermolysis bullosa

kindler syndrome

malignant atrophic papulosis

oral submucous fibrosis

papillon-lefèvre syndrome

primary hyperoxaluria type 1

proteus syndrome

This symptom has already been validated