Cutaneous Kikuchi Disease Clinical Presentation

Updated: Nov 14, 2019
  • Author: Jenny E Liles, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Kikuchi disease (KD) commonly presents with painful cervical lymphadenopathy accompanied by systemic symptoms such as fever, arthralgias, myalgias, anorexia, weight loss, night sweats, and chills. Hepatosplenomegaly and aseptic meningitis have also been reported. [3, 9] Cutaneous lesions, known as cutaneous KD, are relatively common and may occur in about one third of patients. [3] These cutaneous eruptions are varied and may include erythematous indurated papules or plaques, nodules, acneiform eruptions, or urticarial lesions. The eruption favors the upper body, especially the head and neck. [9, 14]

A prior history of autoimmune disease is often elicited in patients with KD. One study reported that 12% of patients with KD had a preceding diagnosis of systemic lupus erythematosus, which is the most commonly associated autoimmune disease. [3] Cutaneous lupus erythematosus, including discoid lupus and subacute cutaneous lupus, may also be associated with KD. [8, 9, 10, 11, 12, 22] Other associated autoimmune diseases include systemic sclerosis, antiphospholipid antibody syndrome, rheumatoid arthritis, Hashimoto thyroiditis, and Wegner granulomatosis. [3, 23] Therefore, it is important to take a thorough medical history in patients with possible KD to elicit any of these associated and possibly predisposing conditions.

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Physical Examination

Physical examination most commonly demonstrates cervical lymphadenopathy in Kikuchi disease (KD), although other lymph nodes may be involved, such as the axillary or supraclavicular nodes. Lymphadenopathy is usually painful. [9] Cutaneous KD occurs in about one third of patients (16-40%) and is often of a nonspecific nature. [3, 24] Most cutaneous lesions present as erythematous macules, papules, plaques, nodules, or ulcers on the upper part of the body, such as the trunk, the upper extremities, and the face. [9, 14, 25]

Several reports in the literature highlight the different physical examination findings associated with cutaneous KD. For example, Kim et al [26] reported a case of KD with a papulopustular eruption over the torso, arms, and legs. Imai et al [27] described a patient with lip edema. This patient had facial, neck, and arm desquamation and erosions, as well as transient indurated erythematous papules. Kaur et al [28] described a patient with Kikuchi disease with erythema multiforme–like lesions and a facial rash.

Other atypical presentations include oral ulcers, vesicles, [29] and eyelid edema. [30]

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Complications

Kikuchi disease (KD) usually resolves on its own in a matter of months without sequelae. However, KD can evolve into lupus erythematosus (either systemic or cutaneous). Therefore, long-term follow up and counseling patients about this risk is prudent.

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