Systemic lupus erythematosus with cytophagic histiocytic panniculitis successfully treated with high-dose glucocorticoids and cyclosporine A

Abstract

A 37-year-old male with systemic lupus erythematosus (SLE) presented with high fever, subcutaneous indurations, anemia, thrombocytopenia, elevated liver enzymes and hyperferritinemia. Skin biopsy revealed hemophagocytic histiocytes in the adipose tissues. The patient was diagnosed with SLE with cytophagic histiocytic panniculitis (CHP). Treatment with high-dose glucocorticoids and cyclosporine A induced remission of SLE and CHP. CHP is generally a systemic disorder affecting subcutaneous adipose tissues with a high mortality rate. However, based on the present and previously reported cases, we believe that intensive immunosuppression can ameliorate CHP that occurs as a skin manifestation of SLE.

Cytophagic Histiocytic Panniculitis (CHP) in a Patient with SLE Found after Autopsy: When a Rash Is “Complicated!”

“…CHP in patients with SLE is a rare combination and to date only a handful such cases have been reported in medical literature. Fever, lymphadenopathy, and pancytopenia are some of the clinical features that indicate poor prognosis in such patients [9]. Our patient had pancytopenia and had a rapidly progressive decline in clinical picture that is characteristic of this disorder in severe cases as mentioned earlier.…”

“…The current treatment options include high dose of steroids and immunosuppressants such as cyclosporine A as the first line agents [9, 18]. Recently other treatment modalities like chemotherapy have also been used successfully, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) being the most commonly used chemotherapy regimen.…”

“…CHP has a poor prognosis and a high mortality up to 70% in severe cases, while other cases are successfully treated with immunosuppressive agents like cyclosporine [9, 15, 18, 19]. CHP in patients with SLE is a rare combination and to date only a handful such cases have been reported in medical literature.…”

“…Reports of CHP after H1N1 vaccination are available in the literature [8]. Its association with autoimmune diseases like systemic lupus erythematosus (SLE) is also reported and has a high mortality rate [9–12]. However, only a few such cases confirming the association between SLE and CHP are available.…”

British Association of Dermatologists guidelines for the safe and effective prescribing of oral ciclosporin in dermatology 2018

“…Ciclosporin has been prescribed and elsewhere in the U.K. (both within and outside its licence) for the dermatological conditions listed in Table …”