Muckle-Wells syndrome

Muckle-Wells syndrome (MWS) is an intermediate form of cryopyrin-associated periodic syndrome (CAPS; see this term) and is characterized by recurrent fever (with malaise and chills), recurrent urticaria-like skin rash, sensorineural deafness, general signs of inflammation (eye redness, headaches, arthralgia/myalgia) and potentially life-threatening secondary amyloidosis (AA type).

Urticaria

Raised, well-circumscribed areas of erythema and edema involving the dermis and epidermis. Urticaria is intensely pruritic, and blanches completely with pressure.

Total: 10

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PMID (PMCID)
29797525	MIXED_SAMPLE	Adult
	Four cases of acute infectious urticaria showing significant elevation of plasma D-dimer level.
	Takahashi T, Minami S, Teramura K, Tanaka T, Fujimoto N. J Dermatol. 2018;45(8):1013-1016.
	In our cases, clinical features accorded with acute infectious urticaria, and their histological features were similar to those of neutrophilic urticaria as described previously.
24510061	MALE	Adult
	Renal involvement in secondary amyloidosis of Muckle-Wells syndrome: marked improvement of renal function and reduction of proteinuria after therapy with human anti-interleukin-1beta monoclonal antibody canakinumab.
	Scarpioni R, Rigante D, Cantarini L, Ricardi M, Albertazzi V, Melfa L, Lazzaro A. Clin Rheumatol. 2015;34(7):1311-6.
	Muckle-Wells syndrome (MWS) is a rare hereditary autoinflammatory disorder characterized by recurrent urticaria-like skin rashes, arthralgias, conjunctivitis, hypoacusia, and risk of reactive AA amyloidosis due to the progressive accumulation of amyloid fibrils in different organs.
24150829	MIXED_SAMPLE	Adult
	Muckle-Wells cryopyrinopathy: complex phenotyping and response to therapy in a new multiplex kindred.
	Headley AP, Cordingley F, Hawkins PN, Riminton DS. Inflammation. 2014;37(2):396-401.
	Muckle-Wells syndrome (MWS) is a member of the cryopyrin-associated periodic syndrome family of auto-inflammatory diseases, originally described as a triad of urticaria, sensorineural deafness and amyloidosis.
16556280	FEMALE	Adult
	A probable case of Muckle-Wells syndrome.
	Kagami S, Saeki H, Kuwano Y, Imakado S, Tamaki K. J Dermatol. 2006;33(2):118-21.
	Muckle-Wells syndrome may go undiagnosed for many years or be misdiagnosed as refractory urticaria.
9797932	MIXED_SAMPLE	Adult
	Circadian elevation of IL-6 levels in Muckle-Wells syndrome: a disorder of the neuro-immune axis?
	Gerbig AW, Dahinden CA, Mullis P, Hunziker T. QJM. 1998;91(7):489-92.
	Muckle-Wells syndrome (MWS) is a rare autosomal dominant hereditary disorder characterized by chronic recurrent urticaria, arthralgia, sensorineural deafness, and in some cases nephropathy due to amyloidosis (AA type).
7802040	MIXED_SAMPLE	Adult
	Autosomal dominant Muckle-Wells syndrome associated with cystinuria, ichthyosis, and aphthosis in a four-generation family.
	Berthelot JM, Maugars Y, Robillard N, Pascal O, Stalder JF, David A, Prost A. Am J Med Genet. 1994;53(1):72-4.
	Muckle-Wells syndrome is a rare autosomal dominant disorder characterized by chronic recurrent urticaria, periodic arthritis, sensorineural deafness, general signs of inflammation, and secondary amyloidosis (AA type).
8000753	MIXED_SAMPLE	Adult
	The arthropathy of the Muckle-Wells syndrome.
	Watts RA, Nicholls A, Scott DG. Br J Rheumatol. 1994;33(12):1184-7.
	Muckle-Wells syndrome (MWS) is a rare condition characterized by urticaria, arthralgias, deafness and amyloid nephropathy.
1737560	FEMALE	Adult
	[Complications in the course of the Muckle-Wells syndrome].
	Fuger K, Fleischmann E, Weber M, Mann J. Dtsch Med Wochenschr. 1992;117(7):256-60.
	The triad of renal amyloidosis, inner-ear deafness and recurrent urticaria is characteristic of Muckle-Wells syndrome, which has a hereditary basis.
1893010	FEMALE	Middle Aged
	[Muckle-Wells syndrome associated with idiopathic interstitial pneumopathy].
	Gomez Rodriguez F, Munoz Lucena F, Pena Grinan N, Umbria Dominguez S. An Med Interna. 1991;8(2):85-6.
	Muckle-Wells syndrome is characterized by recurrent episodes of urticaria, fever, polyarthralgia, deafness and secondary amyloid (AA type), familial type with autosome dominant features; few cases have been described.
1674741	MALE	Adult
	[Urticarial vasculitis as a symptom of Muckle-Wells syndrome?].
	Grassegger A, Greil R, Feichtinger J, Fritsch P, Hintner H. Hautarzt. 1991;42(2):116-9.
	This patient is likely to represent a variant of the Muckle-Wells syndrome (chronic relapsing urticaria, fever, arthralgia, deafness and renal amyloidosis).