|Year : 2000 | Volume
| Issue : 1 | Page : 45-47
Source of Support: None, Conflict of Interest: None
Mucocutaneous lymph node syndrome has been rarely seen or reported in India because of its rare occurrence and probably because of its close resemblance to many exanthematous conditions. It is a characteristic disease with a unique clinical presentation. Knowledge about this established disease is essential as it can involve the coronary and other medium and small sized arteries causing vasculitis in about 20% of cases. This probably accounts for a significant number of ischemic heart diseases and sudden deaths in the young, i.e. in the second and third decades, later in life for which no other cause is found then. It is a condition diagnosed entirely on clinical basis and hence the need to be aware of its characteristic features.
Keywords: Mucocutaneous lymph node syndrome, Kawasaki disease
|How to cite this article:|
. Kawasaki disease. Indian J Dermatol Venereol Leprol 2000;66:45-7
| Introduction|| |
Kawasaki disease or more correctly known as the mucocutaneous lymph node syndrome was first reported by Tomasio Kawasaki in 1967 in a series of Japanese children with an unusual febrile illness. It is now known to occur worldwide commonly in childhood though the exact cause is not yet established. The diagnosis of this condition is based on recognition of its characteristic clinical features, and exclusion of other diseases, that may have similar manifestations. As the name itself is self explanatory it is a febrile illness in children with associated rash of the skin and mucosae, conjunctival congestion and enlarged lymph nodes. It is strongly likely to be confused with scarlet fever, measles, other viral exanthemas, drug eruptions and rare disorders like rickettsial / leptospiral fever as it mimics them.
| Case Report|| |
A 4-year-old male child from Pen, Raigad, near Bombay presented with fever and generalized rash of 5 days duration. The child was highly irritable and lethargic. There was a history of measles about 3 weeks back in the child from which he had recovered without any complications. The child was receiving antiuberculosis treatment with INH and rifampicin for tubercular effusion of the right knee since last 5 months. The mother was extremely worried as she thought that this was a relapse of measles.
On examination the child was febrile, irritable and pale. He had generalized lymphadenopathy. Cervical, occipital, axillary and inguinal lymph nodes were enlarged bilaterally. These visibly enlarged lymph nodes were nontender, firm and discrete. There was conjunctival congestion and a seropurulent discharge from the eyes. The lips were dry and fissured with ulceration and crusting at the angles of the mouth.Oral mucosa showed raw erythematous areas on the hard palate with a few petechial lesions and a white coating. The tongue was red. There was a diffuse maculopapular erythematous rash involving both the extremities and the trunk with scaling and exfoliation. Palms and soles showed intense red erythema and scaling.The glans penis also showed erythema and scaling. There were a few areas of normal skin. Systemic examination was normal except for the right knee swelling.
Investigations revealed a hemoglobin of 7gm%, WBC count 4500, with 74% lymphocytes and a normochromic, normocytic anemia. Liver and renal function tests were within normal limits. There was thrombocytopenia, while ASO titers were normal. CReactive protein (CRP) was negative and blood culture also did not grow anything. Skin biopsy showed no specific changes except for a mild mononuclear infiltrate in the dermis. There was no evidence of vasculitis. A 2-D echo cardiography of the heart was done, during the desquamative phase of the rash for detecting any involvement of the coronaries but was normal. Though no serological investigations could be done for rickettsial spotted fevers, this and other possible differential disorders were considered and ruled out on clinical grounds.
The child was treated symptomatically with antipyretics, antibiotics and rest. Fever and irritability subsided in 7 to 10 day's time while the morbilliform and erythematous rash started desquamating gradually after 2 weeks starting from the nail fold skin margin upwards. Complete resolution of the rash took about 6 to 8 weeks. The intensely red palms and soles became indurated as they resolved and this took 8 weeks. Lymphadenopathy and the dry fissured lips were the last to resolve which took about 10 to 12 weeks. A 2-D ECHO of the heart was repeated at the end of three months which was normal ruling out involvement of the heart till then.
| Discussion|| |
Kawasaki disease, better termed as mucocutaneous lymph node syndrome (MLNS) is a diffuse vasculitis of unknown aetiology affecting children under 4 years with a characteristic clinical presentation. Though its occurrence is most common in Japan where it was first described in an epidemic form, it occurs through out the world typically as sporadic cases.The exact etiology and pathogenesis still remain incompletely understood. An infective agent inciting an immune complex mediated vasculitis is thought to be the causative factor. The list of probable causative agents implicated and being studied so far include Rickettsia-like organisms, house dust mites, mite associated organisms, Propionibacterium acnes, and retroviruses.
The diagnosis of this disease is based on its characteristic presentation and exclusion of other diseases with similar manifestations. ,The diagnostic criterion are shown in the [Table - 1]. Some of the unique features of this condition which were seen in our patient were the intense erythema, scaling and induration of the palms and soles, irritability of the child, visibly enlarged lymphnodes especially the cervical and occipital nodes, and the changes in the nails during recovery as peeling and fissuring at the nail bed.Though a maculopapular drug rash with mucosal involvement as in Stevens-Johnson syndrome More Details was also thought of as the child was on isoniazid and rifampicin the association of all the said features together ruled it out.
Our child also had a preceding history of measles two weeks before this disease which had a similar maculopapular rash. This seems to be mere co- incidental as no such association in known. We could not come across report on Indian children so far though it might have been encountered by quite a few pediatricians and dermatologists.
| References|| |
|1.||Kawasaki T. Acute febrile muco-cutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children (Japanese). Jap J Allergy 1967; 16: 178-222. |
|2.||Highet AS, Kurtz J. Viral infections Kawasaki disease, In:Rook A, wilkinson DS, Ebling FJH, et al, Editors. Textbook of Dermatology. Fifth edition oxford, Blackwell Scientific Publications.1992:947-948. |
|3.||Levin M, Dillon MI. Kawasaki disease. In: Champion RH, Pye FJ. Editors. Recent Advances in Dermatology. Number Nine. New Delhi. B.I. Churchill Livingstone Pvt.Ltd. Indian Editon 1994: 81-93. |
|4.||Bierman F, Cersony WM. Kawasaki disease: clinical perspective. J Pediatr 1987; Iii:789 -792. |
|5.||Rowley AH, Gonzalez-Crussi F, Shulman ST Kawasaki disease. Rev infect Dis 1988 ; 10: 1-15. |
[Table - 1]