Familial eosinophilic cellulitis, dysmorphic habitus, and mental retardation

Davis, Mark D.P.; Brown, Ashanti; Blackston, R. Dwain; Gaughf, Claudia; Peterson, Ellen A.; Gleich, Gerald J.; Leiferman, Kristin M.

doi:10.1016/s0190-9622(98)70588-2

Cited by 57 publications

(24 citation statements)

References 42 publications

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“…Wells described it in 1971 as 'recurrent granulomatous dermatitis with eosinophilia'. [4][5][6][7][8] Reported precipitants of Wells' syndrome include insect bites, drug reactions, varicella, mumps, onchocerciasis, myeloproliferative disorders and fungal infection. 2,3 The syndrome has been mainly described in adults with only a few childhood cases reported.…”

Section: Introductionmentioning

confidence: 99%

Wells' syndrome following thiomersal‐containing vaccinations

Koh¹,

Warren²,

Moore³

et al. 2003

Aust J Dermatology

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A 3 1/2-year-old boy presented on three occasions with painful, itchy, oedematous plaques on his limbs. On two occasions he had received hepatitis B vaccination 11-13 days previously, and on the third occasion received triple antigen (DTP) vaccination 10 days earlier. Skin biopsy revealed a prominent infiltrate of eosinophils involving the entire thickness of the dermis. In addition there were prominent 'flame figures' consisting of eosinophilic necrotic collagen surrounded by granular basophilic debris. The clinical and histological pictures were consistent with Wells' syndrome. The eruption settled on the second and third occasions with 0.1% mometasone furoate cream. Subsequent patch testing showed 2+ reaction to preservative thiomersal at 96 hours. This is the first description of Wells' syndrome with typical clinical and histopathological features associated with thiomersal in two different vaccines.

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Section: Introductionmentioning

confidence: 99%

Wells' syndrome following thiomersal‐containing vaccinations

Koh¹,

Warren²,

Moore³

et al. 2003

Aust J Dermatology

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“…Cytolysis is a process related to cell death (also called "necrosis") by which cell membrane (and sometimes organelle membrane) is ruptured and specific eosinophil granules are liberated into the surrounding tissue. Of note, there are some reports showing that, in human diseases, intact membrane-bound eosinophil granules accumulate into tissues while granule-derived toxins distribute in the eosinophil cytoplasm as granules disintegrate during cell disruption (Gleich et al 1984, Davis et al 1998, Erjefalt et al 1999, indicating that besides piecemeal degranulation, eosinophils can undergo "cytolysis". Whether the mechanisms involved in generating cytolytic eosinophils in vivo are accidental or regulated need to be investigated.…”

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confidence: 99%

Mechanisms of eosinophil cytokine release

Bandeira‐Melo

Weller

2005

Mem. Inst. Oswaldo Cruz

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Human eosinophils have been demonstrated to contain a multitude of cytokines and chemokines that exist preformed within these cells. This content of pre-formed cytokines, with diverse potential biologic activities, provides eosinophils with capabilities distinct from most other leukocytes. The localization of pre-formed cytokines within eosinophils is both within specific granules and associated with substantial numbers of morphologically distinct cytoplasmic vesicles. Stimulation for release of specific cytokines, such as IL-4, leads to a regulated signal transduction cascade, which is dependent on the formation of leukotriene C 4 within eosinophils where it acts as an intracrine mediator. IL-4 release occurs selectively and is by means of vesicular transport. The capabilities of eosinophils not only to rapidly release pre-formed cytokines but also to differentially regulate which cytokines are released endow eosinophils with distinct abilities in innate and acquired immunity.Key words: eosinophils -cytokines -degranulation BackgroundEosinophil specific granules contain four distinct cationic proteins, named major basic protein (MBP), eosinophil peroxidase (EPO), eosinophil cationic protein (ECP) and eosinophil-derived neurotoxin (EDN), whose extracellular release may cause dysfunction and destruction of surrounding cells (reviewed Gleich et al. 1992). In addition to these toxic proteins, eosinophil specific granules also store at least two-dozen of pre-formed cytokines and chemokines with pro-inflammatory or immunoregulatory properties. By a variety of techniques, such as subcellular fractionation, immunogold electron microscopy, and immunocytochemistry, a growing list of cytokines has been found within human eosinophil granules, including: (a) prototypical Th1 cytokines: IL-12 (Grewe et al. 1998) and IFN-γ (Lamkhioued et al. 1996); (b) prototypical Th2 cytokines: IL-4 (Moller et al. 1996b), IL-5 (Dubucquoi et al. 1994, Moller et al. 1996a, Desreumaux et al. 1998) and IL-13 (Woerly et al. 2002); (c) chemokines: RANTES , Ying et al. 1996, IL-8 (Braun et al. 1993), and eotaxin (Nakajima et al. 1998); (d) growth factors: granulocyte/macrophage colony-stimulatin factor (GM-CSF) (Levi-Schaffer et al. 1995, Desreumaux et al. 1998, IL-3 (Desreumaux et al. 1998), vascular endothelial growth factor (VEGF) (Horiuchi & Weller 1997), transforming growth factor (TGF)-α (Egesten et al. 1996) and stem cell factor (SCF) (Hartman et al. 2001); (e) pro-inflammatory cytokines: tumor necrosis factor (TNF)-α (Beil et al. 1993) and IL-6 (Lacy et al. 1998); and (f) immune-regulators: IL-16 (Lim et al. 1996), IL-2, and IL-10 (Woerly et al. 1999).In view of such diverse functional potentiality, eosinophil degranulation is a critical process by which eosinophils may damage, activate or down-regulate the neighboring cells. Hence, it was hypothesized that not all granule proteins are concomitantly released from eosinophils. Conversely, a regulated mechanism to specifically select and release proteins from eosinophil granules may exi...

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“…The following precipitants were described: drug reactions, thiomersal-containing vaccine, varicella, mumps, erysipelas, penicillin, insect bites, treatment of molluscum contagiosum with cryosurgery [1,2,4,5,8,9]. In some cases a genetic factor seems to play a role [1,3]. In our case there was a systemic illness with fever and pain in the legs two weeks prior to the eruption.…”

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confidence: 66%