LETTERS TO THE EDITOR

Juvenile Alk Phosphatase and electrolytes were presenting with Anasarca normal. Blood culture sterile. CRP-positive. RA factor - negative. ANA-negative. Total serum proteins- 7.8, (5.5-8.0 g/L), serum albumin-2.8, CPK-220 units/L (55-170 Juvenile dermatomyositis (JDM) is a U/L), LDH-2033 units/L (150-500U/L). multisystem disease characterized by Urine albumin - nil (on repeated non-suppurative inflammation of striated examinations), 24-hrs urinary protein-normal. muscles with characteristic cutaneous Urine culture - sterile. USG abdomen revealed findings in the form of erythematous mild hepatomegaly, and mild pleural (heliotrope) periorbital (1). The classical effusion. Kidneys - normal in size and features of disease include proximal muscle echotexture. (An USG repeated 15 days later weakness, dermatitis associated with revealed no free fluid). EMG revealed MUAP constitutional symptoms and edematous and Duration and Amplitude reduced in Deltoid R, induarated muscles. Localized of Biceps Brachii R and Tibialis Ant R. Muscle muscle is a common feature but anasarca biopsy revealed infiltration with inflammatory as a presenting feature is exceedingly cells and scattered areas of degeneration and rare(2). necrosis. An 8-year-old female child presented with The diagnosis of juvenile dermato- irregular fever, anorexia, myalgia and myositis was based on clinical features, generalized arthralgia. It was associated with muscle biopsy and EMG findings. The patient muscle weakness and progressive deformity was treated with prednisolone 2 mg/kg/24 hr. of both elbow and knee joints making her Oliguria resolved within a few days and pedal bedridden. Patient received irregular edema subsided a little but weakness and non treatment prior to admission. On admission pitting edema of both upper and lower child was febrile, had mild , generalized extremities persisted. Her general well being nonpitting edema of upper and lower and appetite improved in 5 weeks of therapy extremities and facial puffiness. There was but later on she developed severe mucosal ascites and mild hepatomegaly. Neurological ulcers, dysphagia and fresh bleeding per examination revealed grade III muscle power rectum. Patient died due to septicemia in all four limbs, muscle atrophy with flexion and disseminated intravascular coagulation. contractures at elbows and knees. Plantar JDM presents characteristically with reflex was flexor. The joint mobility was proximal muscle weakness associated with limited due to flexion contractures and the periorbital heliotrope rash(1,3). Localized movements were painful. There was no joint muscle edema is commonly encountered but swelling but there was tenderness and generalized edema is extremely uncommon induration of muscles in both extremities. (2). In our case generalized non pitting edema There was an erythematous periorbital was presenting feature along with muscle violaceous rash. Rest of the systemic weakness and arthralgia. The absence of examination was normal. clinical features and laboratory parameters Investigations revealed Hb 11g%, TLC that ruled out any other cause of anasarca 11, 300, DLC P80, L16, M2, E2, ESR-14 mm suggests that anasarca in this case was due to Urea, Creatinine, Bilirubin, SGOT, SGPT, dermatomyositis.

INDIAN PEDIATRICS 752 VOLUME 41__JULY 17, 2004 LETTERS TO THE EDITOR

Generalized edema in association with Correspondence to: JDM is infrequently reported(2). Such Sumit Mehndiratta, patients respond poorly to oral steroid B-246 Yojna Vihar, Delhi 110 092, India. therapy. The exact mechanism of generalized E-mail: [email protected] edema in JDM in not known. Autoantibodies REFERENCES are believed to play an important role in pathogenesis. The pathological hallmark is an 1. Cassidy JT. Systemic Lupus Erythematosus, immune complex vasculitis - the damage to Juvenile Dermatomyositis, Scleroderma and Vasculitis: Chapter 88. In: Ruddy S, Harris endothelium caused by complement C5b-9 ED, Sledge CB, editors. Kelly's Text Book of attack complex(4,5). It is suggested that as a Rheumato1ogy. 6th ed vol II, USA: W B sequel to diffuse and widespread capillary Sanunders & Company. 2001; p. 1319-1323. endothelial damage there is increase in 2. Mitchell JP, Dennis GJ, Rider LG. Juvenile capillary permeability in muscles leading to dermatomyositis presenting with anasarca: A generalized edema. possible indicator of severe disease activity. J Pediatr 2001, 138: 942-945. Sumit Mehndiratta, 3. Singh S, Kumar L, Ravushankar R. Juvenile Prabhabati Banerjee, Dermatomyositis in North India. Indian Department of Pediatric Medicine, Pediatr 1997; 37: 193-198. R.G. Kar Medical College & Hospital, 4. Cassidy JT, Petty Rose E. Juvenile Kolkata, Dermatomyositis. In: Cassidy JT and Petty West Bengal 700 004, RE, editors. Text Book of Pediatric India. Rheumatology, 4th Ed. USA: WB Saunders & Company; 2001. p 465-504. 5. Norins AL. Juvenile Dermatomyositis. Med Clin North America 1989; 73: 1193-1209.

INDIAN PEDIATRICS 753 VOLUME 41__JULY 17, 2004