Dr Theresa, Dr Suma Balan(Paed Rheumato), Dr C Jayakumar, Amrita Institute of Medical Sciences, Kochi
Eight year old child who was apparently normal 1 year back presented with swelling under left eye and fever following a dental procedure
Fever was high grade in nature with no chills ,rigors, cough, cold, and loose stools. Child had another Dental consultation and extraction and excision of the swelling was done
Following this swelling and pain reduced.
During this period blood counts were low but Bone marrow was for low and that turned out to be normal .Biopsy of the lesion was suggestive of chronic inflammatory lesion with granuloma formation.
Culture and sensitivity showed candida organisms. At this point child was apparently better but later on developed high grade fever and erythematous lesions all over the body .
At this juncture child came to AIMS for management.
Child was febrile, sick looking, irritable. Lymph nodes in the inguinal region is palpable and tender. There were multiple tender erythematous skin induration in both upper and lower limbs and trunk.
Systemic examination was normal. Auxiology was normal.
Labs done showed TC: 3.20K/ul, N:40.0%, L:57.2%,, ESR: 23mm/hr, LDH: 1040U/L, Serum ferritin: 958.40ng/ml, Serum Fibrinogen: 147mg/dl,
IgG:1090mg/dl, IgA: 318.1mg/dl, IgM:75.70mg/dl,
After skin biopsy from the lesion child was started on Inj Linezolid, InjClindamycin, Inj Cefotaxime and Naproxen Histopathology of the biopsy specimen with IHC was reported as subcutaneous nodule with the possibilities of Cytophagic Histiocytic Panniculitis (CHP). This case was discussed with the tumour board and the consensus was to do TCR gene rearrangement and perforin gene mutation studies and to treat like CHP and follow up. He was pulsed with IV Methylprednisiolone 30 mg /kg mg for 3 days and later converted to Oral Prednisolone at 1.5mg/kg/day in divided doses. He was started on Cap Cyclosporin. He became asymptomatic and he was observed in the ward for 1 more week after starting cyclosporine. The child was discharged on steroids and cyclosporine and was asked to monitor BP twice daily and maintain BP chart.
Cytophagic Histiocytic Panniculitis:
It is a rare disease associated with either non malignant conditions or subcutaneous panniculitis like T cell lymphoma and often also associated with hemophagocytic lymphohistiocytosis(HLH)
The clinical manifestations can be attributed to increased secretions of cytokines from activated T/NK cells and macrophages, which in turn suppress the hematopoiesis causing prominent hemophagocytosis in bone marrow, spleen, liver and lymph nodes. In addition cytokine activated monocytes and vascular endothelial cells may produce procoagulant substances, plasminogen activator inhibitor-1, von Willebrand factor.
Diagnosis of CHP relies mainly on the histhopathology which shows prominent chronic inflammation of fat tissue infiltrated mainly benign appearing T lymphocyte and occasional phagocytic histiocytes. Erythrophagocytosis and cytophagocytosis benign appearing histiocytes can also be observed in internal organs, particularly the lymph nodes, spleen, liver and bone marrow
CHP is generally treated with systemic corticosteroids.
Methyl prednisolone pulse therapy is the treatment option. Alternative immunosuppressive therapy such as Cyclosporine may be used as a first line treatment..
In severe relapse cases high dose chemotherapy followed autologous peripheral blood stem cell transplantation can be considered as alternative treatment option.
CHP is rare and often fatal form of panniculitis with multisystem involvement. However, it can also present in a benign form involving only the subcutaneous tissue, thus having a broad clinical spectrum.