Dr.Sruthi Suresh, Dr.C.Jayakumar, Dr.Praveena
Department of Paediatrics
Amrita Institute of Medical Sciences, Kochi
Sixteen year old female child, known case of Paroxysmal Kinesigenic Dyskinesia who was on Carbamazepine, developed high grade, intermittent fever and on third day of fever she developed swelling of face along with pruritic rash which started on face and ear and gradually spread to neck, limbs and trunk.
For this symptom she has received Amoxicillin, hydrocortisone and oral prednisolone for three days from a near health care facility
Referral to AIMS was fined due to persisting symptom
Her past pediatric medical records were normal
One month back she was evaluated for dystonic movements occurring on and off and was diagnosed with paroxysmal kinesigenic dyskinesia for which she was started on carbamazepine from an outside hospital.
History of similar complaints of dystonic movements in mother and younger brother elicited for which her brother is also on Carbamazepine
At admission she was afebrile with other stable vitals. She had bilateral tender cervical and axillary lymphadenopathy of 2x2cm along with facial edema, periorbital puffiness, conjunctival congestion, congested throat and extensive very itchy maculopapular rashes over face, trunk and extremities. System examination was within normal limits.
Fig. 1: Patient at the time of presentation with significant facial edema and maculopapular rash and erythema.
Labs Neutrophilic leucocytosis with 15% eosinophils and mildly elevated inflammatory markers.
SGOT and SGPT 100.2 /200.6 IU/L
Renal function, serum electrolytes levels and urine analysis were normal.
USG abdomen showed normal study.
She was started on IV dexamethasone which was given for three days and switched to oral prednisolone 2mg/kg which was continued for 9 days.
She was also managed with round the clock one or two antihistamines (Hydroxyzine and levo cetrizine ) owing to increased itching episodes.
In view of transaminitis, N-acetyl cysteine and Urso deoxy colic acid was started.
After admission for high grade fever spikes, IV Piptaz was given after sending for blood culture. As her fever subsided and cultures were found to be negative, IV Piptaz was stopped. As liver enzymes started rising investigations to rule out autoimmune liver disease and hepatitis work up was done and they were found to be negative
Her repeat labs showed a decreasing trend in liver enzymes. As she improved significantly over the hospital stay and remained euglycemic and normotensive she was discharged with oral prednisolone which is planned to be tapered down slowly.
Carbamazepine was stopped immediately and planned for her Paroxysmal Kinesigenic dyskinesia later
During the hospital stay she has no worsening of the dyskinesia
Fig. 2: Patient at the time of discharge with the resolution of facial edema and clearing of maculopapular rashes.
Drug reaction with eosinophilia and systemic symptoms (DRESS Syndrome)
• Severe adverse drug reaction
• Characterized an extensive skin rash in association with visceral organ involvement, lymphadenopathy, eosinophilia, and atypical lymphocytosis.
• Drugs which are known to cause DRESS syndrome includes allopurinol, aromatic antiepileptic agents such as Carbamazepine, Phenytoin, Lamotrigine, Oxcarbazepine, Phenobarbital, Sulfonamides, Vancomycin, Minocycline, Nevirapine and Antituberculosis agents.
• Systemic symptoms associated with DRESS include the following:
a. Fever ≥101.3°F
b. Lymphadenopathy
c. Hematologic abnormalities:
•Eosinophilia >700/microL
•Leukocytosis
•Neutrophilia
•Lymphocytosis
•Monocytosis
•Atypical lymphocytes
d. Symptoms and/or laboratory abnormalities related with any visceral involvement
▪ The most widely used criteria to confirm or exclude the diagnosis of DRESS are those included in the Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system, based on the main clinical manifestations of DRESS :
1) Fever >101.3°F or >38.5°C (core) or >100.4°F or >38°C (axillary)
2) Enlarged lymph nodes in at least two different body areas
3) Eosinophilia
4) Atypical lymphocytes
5) Skin involvement
6) Organ involvement (eg, at least twofold elevation of liver enzymes on at least two different days)
7) Resolution >15 days
A value between -1 and 2 is assigned to each feature .The cumulative score ranges from -4 to 9 and defines four levels of certainty regarding the diagnosis of DRESS: excluded, possible, probable, and definite.
Management:
1. Withdrawal of the offending drug
2. Symptomatic management with local application corticosteroids for mild disease
3. Systemic glucocorticoids
4. Second line therapy such as cyclosporine and IV Ig