How not to miss autoinflammatory diseases masquerading as urticaria

Abstract

Urticarial skin reactions are one of the most frequent problems seen by allergists and clinical immunologists in daily practice. The most common reason for recurrent wheals is spontaneous urticaria. There are, however, several less common diseases that present with urticarial rash, such as urticarial vasculitis and autoinflammatory disorders. The latter include cryopyrin-associated periodic syndrome and Schnitzler's syndrome, both rare and disabling conditions mediated by increased interleukin-1 secretion. Apart from the urticarial rash, patients are suffering from a variety of systemic symptoms including recurrent fever attacks, arthralgia or arthritis and fatigue. Autoinflammatory diseases are often associated with a diagnostic delay of many years and do not respond to antihistamines and other treatments of urticaria. Also, the chronic inflammation may lead to longterm complications such as amyloidosis. It is therefore important not to miss these diseases when diagnosing and treating patients with chronic recurrent urticarial rash. Here, we present clinical clues and tips that can help to identify autoinflammatory disorders in patients presenting with chronic urticarial rash and discuss their clinical picture and management.

BSACI guideline for the management of chronic urticaria and angioedema

“…This is a clinical diagnosis presenting with a distinctive rash over the extensor surfaces of the legs and buttocks [102]. Rare causes of chronic urticaria should be considered in patients with other systemic symptoms or raised inflammatory markers [103]. A diagnostic lesional skin biopsy could be considered if features such as fever, painful lesions, arthralgia, raised ESR, lesions lasting 24 h or more or lesions that resolve revealing purpura or petechiae.…”

The Turkish Guideline for the Diagnosis and Management of Urticaria-2016

“…Ürtikerin ayırıcı tanısında düşünülecek diğer önemli bir grup hastalık otoenflamatuvar hastalıklardır. Otoenflamatuvar hastalıkları düşündüren bulgular şu şekilde sıralanır; -Yirmi dört saate kadar süren ürtikeryal ve/veya makülopapüler döküntüler (kaşıntılı/kaşıntısız), -Yirmi yaş öncesi başlangıç, -Ateş, eklem ağrısı, halsizlik gibi sistemik semptomlar, -Histopatolojide nötrofil baskınlığı, -Sistemik amiloidoz eğilimi [64][65][66][67] . Ürtikerde tanısal algoritma Şekil 1'de gösterilmektedir.…”

Neurophilic urticarial dermatosis

“…Auch bei einer chronisch spontanen Urtikaria mit sehr langer Dauer sollte (insbesondere bei Kindern) eine NUD differenzialdiagnostisch erwogen werden. Schließlich sollte eine chronische Urtikaria, die gar nicht oder schlecht auf adäquat dosierte Antihistaminika anspricht, den Ausschluss einer NUD veranlassen (sehr gut zusammengestellt in [5]). …”

“…Teilweise wurden von den Autoren Autoinflammation und Autoimmunität müssen eher als Enden eines überlappenden Spektrums angesehen werden IL-1β kommt eine zentrale Rolle zu Der Hautarzt 5 · 2016 407 CME Tab. 2 Krankheiten, bei denen eine neutrophile urtikarielle Dermatose beschrieben wurde Systemischer und kutaner Lupus erythematodes [2,11] Schnitzler-Syndrom [2] Morbus Still [2,5] Cryopyrin-assoziierte periodische Syndrome (CAPS) [2,13] Hyper-IgD-Syndrom [5] Tumornekrosefaktor-Rezeptor-1-assoziiertes periodisches Syndrom (TRAPS) [5] NLRP12-assoziierte familiäre Kälteurtikaria [5] Sjögren-Syndrom [3,11] Dermatomyositis [11] Hepatitis C [2] Asthma [2] Rheumatoide Arthritis [12] Primär biliäre Zirrhose [3] Chronisch entzündliche Darmerkrankungen [3] andere Bezeichnungen wie "autoimmune-related neutrophilic dermatosis" für Läsionen bei Autoimmunerkrankungen wie dem SLE verwendet [4,9,10,11,12]. Die klinischen und histologischen Veränderungen dieser Hautveränderungen entsprechen aber der NUD [3].…”