Eosinophilic Infiltrates in Epidermolysis Bullosa

Abstract

Inherited epidermolysis bullosa encompasses many subsets of diseases, distinguished by skin fragility and blister formation after minor trauma. Histologically, epidermolysis bullosa usually presents as bullae without inflammatory cells. We present four cases of epidermolysis bullosa, including examples of epidermolytic, junctional, and dermolytic types that have eosinophilic infiltrates. These cases do not represent subtypes of epidermolysis bullosa, but simply the influx of eosinophils in neonatally manifested disease. Several hypotheses for the presence of these eosinophils are presented.

Burden of itch in epidermolysis bullosa

“…[8][9][10] Although it is tempting to postulate that the inflammatory infiltrates are responsible for the pruritus, pruritus was a prominent feature in some cases of DDEB pruriginosa in which inflammatory infiltrates were histologically absent. [8][9][10][11][12] Wally et al 13 found that the keratinocytes of patients with the Dowling-Meara subtype of EBS have constitutively activated IL-1b signalling. 13 Glial IL-1b and tumour necrosis factor-a can cause a central sensation of pain, but it is not yet known whether these cytokines activate pruriceptive neurons.…”

Dystrophic Epidermolysis Bullosa Associated with Eosinophilic Infiltrate and Elevated Serum IgE

“…Only a few cases of EB associated with an eosinophilic infiltrate of the skin lesions have been reported ( Table 1). In these patients there were no clinical or laboratory differences from those with EB without eosinophils ( 4–6).…”

Eosinophil infiltration in three patients with generalized atrophic benign epidermolysis bullosa from a Japanese family: molecular genetic and immunohistochemical studies

“…Lanes 1 and 4, 36 cycles; lanes 2 and 5, 38 cycles; and lanes 3 and 6, 40 cycles. Relative intensity ratio of COL17A1 to GAPDH in the proband (lanes 1-3) is not decreased compared to the control sample (lanes 4-6) Eosinophil infiltration has also been seen in infants with some types of EB, such as epidermolysis bullosa simplex, junctional epidermolysis bullosa, dominant dystrophic epidermolysis bullosa, but mutations in patients with these disorders were not confirmed (Roth et al 1990;Anton-Lamprecht and Schnyder 1982;SanzSantamaria and McNutt 1966;Grunwald et al 1999). Eosinophil infiltration seen in the neonates with EB was assumed to be a phenomenon of their immaturity and/or insufficiency of the immune response system (Roth et al 1990;Grunwald et al 1999).…”

“…In contrast, an association of EB with eosinophil/ mast cell infiltrations in the bulla is rare and, to our knowledge, such an association has been reported only in a few infants with some types of EB other than GA-BEB (Sanz-Santamaria and McNutt 1966;AntonLamprecht and Schnyder 1982;Roth et al 1990;Grunwald et al 1999); however, the cause of eosinophil infiltration remains unknown. Furthermore, in a few patients with autosomal recessive dystrophic EB (OMIM #226600) due to mutations in (COL7A1), amyloidosis was seen to cause renal dysfunction (Fine et al 2004).…”