Erythema multiforme associated with Kawasaki Disease in a Korean child

ARTICLE

Auteur(s) : Lucy Youngmin Eun¹, Jae Wan Go², Won Hyoung Kang², Shin Han Kim², Han Kyoung Cho²

¹Department of Pediatric Cardiology & Department of Dermatology, Myongji Hospital, Kwandong University College of Medicine, 697-24 Hwajeong-dong, Deokyang-gu, Goyang-si, Gyeonggi-do, 412-270 Korea
²Department of, Pediatric Cardiology, Myongji Hospital, Kwandong University College of Medicine, Goyang-si, Gyeonggi-do, Korea

Kawasaki disease (KD) is a systemic vasculitis with cardiovascular involvement, especially coronary arteriopathy. It usually occurs in younger children, including infants. Although there is no definite proven etiology, KD, according to current notions, may result from immune system activation [1], which can possibly be induced by various infections. Approximately twenty to twenty five percent of untreated patients face cardiac problems such as coronary artery aneurysms [2].

The diagnosis of KD is based on the clinical criteria. The criteria of classic KD are [1] fever for more than 5 days, [2] skin changes on the extremities including erythema on the palms and soles, [3] polymorphic skin rashes, [4] bilateral conjunctival injections, (5) changes in the oral mucosa, reddening of lips, and “strawberry” tongue, and (6) cervical lymphadenopathy [3]. For diagnosis of classic KD, the presence of fever for more than 5 days with at least 4 of the above five principal criteria are required. Among the six criteria of classic KD, four are mucocutaneous findings. The typical findings of cutaneous changes are multiple symmetrical erythematous eruptions on the extensor surfaces of the extremities developing after 3-5 days of fever [4]. EM as cutaneous manifestation of KD was first reported without skin biopsy from a 22-month-old girl with KD in 1979 [5]. Later, annular lesions as prominent cutaneous findings of KD have been reported in 3 patients [6].

A 16-month-old Korean boy was admitted to the pediatric cardiology department for high grade fever, which had continued for about 5 days. By the time of admission, skin rashes had appeared on the patient's trunk and extremities. His medical history was otherwise unremarkable. Physical examination revealed an acutely ill-looking febrile child with multiple target-like erythematous skin rashes on the trunk and upper and lower extremities (figures 1A, B). However, these rashes were not seen on the palms and soles. The diagnosis of classic KD was made by the presence of typical clinical features. Other investigations were non-contributory. Intravenous immunoglobulin (IVIG) and aspirin were given for treatment.

A skin biopsy was performed on a typical lesion of the left forearm. Histological findings showed lymphocyte infiltrations at the dermal-epidermal junction, with exocytosis into the epidermis, satellite cell necrosis, spongiosis, vacuolar degeneration of the basal cell layer, and focal junctional and sub-epidermal cleft formation. The papillary dermis was edematous and showed a mononuclear cell infiltrate (figure 1C). Target-like erythematous skin rashes improved with the application of topical steroids.

Our patient showed sufficient clinical findings related to the diagnostic criteria of classic KD; high fever, bilateral injected conjunctivae, strawberry tongue, enlarged cervical lymph nodes, and multiple target-like skin rashes. Except for these lesions, no other typical lesions of Kawasaki disease, such as palmoplantar erythema, were observed. Skin changes are important features of KD and include the following pattern. A brownish, indurative edema and erythema develop on the palms and soles in 3 to 5 days after the onset of high fever, shortly thereafter, erythematous macular skin rashes begin to develop on the extensor surfaces of the extremities, which may progress to either morbilliform, scarlatiniform, or multiform [4].

EM is mostly associated with medications and various infections, but it may develop as the cutaneous findings of immune diseases, including KD. Also, it seems to be important in severe diseases such as Stevens-Johnson syndrome and toxic epidermal necrolysis [6], but its treatment is considerably different from KD.

In summary, this case suggests that EM can be a cutaneous manifestation of KD. We believe that identification of the associated diseases presenting EM is important for the proper initial patient care.

Acknowledgements

References

2 Suzuki A, Kamiya T, Kuwahara N, et al. Coronary arterial lesions of Kawasaki disease: cardiac catheterization findings of 1100 cases. Pediatr Cardiol 1986; 7: 3-9.

3 Kim DS. Kawasaki disease. Yonsei Med J 2006; 47: 759-72.

4 Weston WL, Huff JC. The mucocutaneous lymph node syndrome; a critical re-examination. Clin Exp Dermatol 1981; 6: 167-78.

5 Bitter JJ, Friedman SA, Paltzik RL, Mofenson HC. Kawasaki's disease appearing as erythema multiforme. Arch Dermatol 1979; 115: 71-2.

6 Ming A, Wargon O. Annular lesions in Kawasaki disease; a cause of confusion. Austral J Dermatol 2008; 49: 207-12.