Dr. Venumadhav, Dr. Suma Balan, Dr. Greeshma, Dr.C. Jayakumar
A 14-year-old female child was referred with complaints of multiple extensive erythema and scaling all over the body, accompanied loss of appetite and weight over the past month. History of taking oral Amoxclav for an upper respiratory tract infection and Naproxen for knee pain from a local hospital. Vitals stable, but clinical examination revealed extensive thick scaling over the scalp, erythema and scaling throughout the body, and scarring alopecia. The patient experienced difficulty in movements due to pain and laceration.
CBC showed elevated white blood cell counts, SGOT >SGPT levels, marked eosinophilia, high creatine kinase levels, low C3 levels, and electrolyte imbalances.
Differentials were dermatomyositis, paraneoplastic syndrome and DRESS(Drug Reaction with Eosinophilia and Systemic Symptoms). Antimyositis profile showed Mi2a2+, TIF 1Y+. MRI suggestive of myositis.
The presence of an extensive skin rash with scarring alopecia, circular overgrowth, a positive Anti-myositis profile, and MRI showing myositis pointed towards dermatomyositis. However, marked eosinophilia, renal involvement, and dyselectrolytemia were against dermatomyositis.
A history of drug intake, acute renal failure, and marked eosinophilia favoured the diagnosis of DRESS. On the other hand, scarring alopecia and a skin biopsy showing spongiotic dermatitis were not consistent with DRESS.
Childs acute phase was treated with IV steroids .Methotrexate was also added due to extensive symptoms. Due to overlapping symptoms, a diagnosis of Juvenile Dermatomyositis with concomitant DRESS syndrome was considered.
Gold standard treatment for JDM is involves systemic glucocorticoids as first-line therapy, often in combination with immunosuppressants such as azathioprine or methotrexate. In resistant cases, rituximab may be considered. Skin disease is managed with sun-protective measures, topical agents, and systemic medications like hydroxychloroquine. Physical therapy is crucial, and prophylaxis against complications, such as osteoporosis and infections, is recommended.
Electrolyte imbalance corrected and Antibiotic and Naproxen were stopped.
DRESS is a T cell-mediated drug hypersensitivity reaction. Factors such as elevated drug levels, viral infections/reactivations, contribute to disease onset. Advances in pharmacogenomics enable laboratory testing for certain HLA alleles, aiding in prevention. Clinical presentation involves diverse cutaneous manifestations(>50% of body surface) , fever, lymphadenopathy, visceral involvement, and potential hepatic complications.
Identification and prompt discontinuation of the causative drug is paramount in Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome. Treatment predominantly involves systemic corticosteroids, with a personalised dosing approach and gradual tapering to prevent relapses.
Carry home message- When faced with a case having overlapping symptoms, it is conceivable that a single patient may have multiple coexisting pathologies.