Initial Cutaneous Manifestations Consistent With Mononeuropathy Multiplex in Churg-Strauss Syndrome

Clinical and histopathologic features of 8 patients with microscopic polyangiitis including two with a slowly progressive clinical course

“…We have observed that cutaneous eruptions can be seen in patients with CSS and in a patient with Wegener's granulomatosis with a chronic clinical course. 20,21 These findings might be considered to be key elements in the smoldering type of ANCA-associated vasculitis.…”

Cutaneous Vasculitis Update: Small Vessel Neutrophilic Vasculitis Syndromes

“…Three phases have been described in CSS 182 : the first phase consists of persistent asthma and other atopic diatheses for many years; the second phase is characterized by the onset and relapses of eosinophilia affecting one or more organs (eg, eosinophilic pneumonia) and blood; and the third and final phase is systemic vasculitis, which may be triggered by massive inhalation of particles, rapid steroid tapering, desensitization, or exposure to drugs such as leukotriene receptor antagonists. 187,189,190 Compared to WG and MPA, CSS patients have less frequent renal disease (,40%), and more frequent cutaneous (,80%), peripheral nerve (#80%), and cardiac diseases (#50%), the latter of which is a common cause of death. 186,187 CSS presenting in the skin, which can occur in less than 30% of patients 9 , is frequently accompanied by mononeuritis, a clinical clue to the possibility of CSS.…”

A Case of Churg-Strauss Syndrome

“…Skin lesions are an important feature of CSS because they are one of the most common extra-pulmonary findings, and these occur in up to 50∼70% of the cases 1,3,6 . The main types of skin lesions found in CSS include (1) erythematous maculopapules resembling erythema multiforme; (2) hemorrhagic lesions ranging from petechiae to extensive ecchymosis, and these lesions are often associated with wheals, necrosis and ulceration, and (3) cutaneous and subcutaneous nodules that are usually deep-seated and tender with a predilection for the scalp and temple region 7 . Other skin findings include livedo reticularis, papulovesicles, non-pitting periorbital edema and nail-fold infarcts 8,9 .…”

“…These cytokines mediate the accumulation of mast cells and basophils and especially eosinophils. One study suggested that the IgE levels as well as the eosinophil counts have a pathological central role in CSS 7 . Therefore, assessing the eosinophil count and measuring the IgE level may be useful to follow the disease activity and the response to therapy.…”

“…However, there are significant side effects with administering steroid and/or immunosuppressants that frequently appeared with their continuous or repeated use. Thus, there are some alternative immunosuppressive drugs such as interferon-α, mycophenolate mofetil and leukotriene receptor antagonist (e.g., montelukast, pranlukast, and zafirlukast) that have been shown to be both efficacious and safe 7,[17][18][19] . Our current case also showed a good response to oral corticosteroids, but other immunosuppressant drugs are probably necessary to treat the patient's sacral pain.…”