Lupus erythematosus panniculitis resistant to standard treatment, complicated with macrophage activation syndrome

Lupus erythematosus panniculitis (LEP) is an uncommon form of chronic cutaneous lupus erythematosus [1–3]. Terms LEP and lupus profundus were used interchangeably, however it is suggested that the term “lupus profundus” should be used when the subcutaneous inflammatory process is accompanied by overlying lesions typical for discoid lupus erythematosus (DLE) [4]. The LEP occurs more frequently in adult women. Typical lesions for the disease are deep-seated, indurated, firm nodules and plaques in locations rich in adipose tissue often healing with ulcerations, atrophic scars and/or calcification [5, 6]. There are no univocal criteria for LEP established. Histopathologic findings are reported to be crucial, however there is no agreement considering specific histopathological results [7, 8]. A positive lupus band test revealed in direct immunofluorescence studies, positive ANA test, leucopenia and hypocomplementemia stated in laboratory findings might be helpful. Differential diagnosis for LEP includes many diseases such as erythema nodosum, pancreatic panniculitis, morphea profunda, cold-panniculitis and, first of all, subcutaneous panniculitis-like T-cell lymphoma (SPTCL), which may be clinically identical with LEP [9, 10]. Immunohistochemical analysis of cells constituting infiltrate is in most cases crucial to distinguish these two diseases. All known therapy strategies for LEP are based on clinical experience and single reported cases, they include antimalarials, immunomodulators, immunosuppressants, intravenous immunoglobulins (IVIG) and rituximab [4, 5, 8, 11, 12].
Macrophage activation syndrome (MAS) is a severe, life-threating hematologic condition and is classified in the group of secondary hemophagocytic lymphohistiocytosis (HLH). The MAS is caused by uncontrolled activation and proliferation of T lymphocytes and macrophages and is characterized by hemophagocytosis in bone marrow, hepatosplenomegaly, cytopenia, hyperferritinemia, hypofibrinogenemia, elevated liver cell enzymes, lymphadenopathy and fever [13]. Therapeutic options for the disease include high doses of steroids, cyclosporine A, intravenous immunoglobulins, etoposide, infliximab, rituximab and anakinra [14].
We present a case of a 40-year-old woman, who was admitted to the Department of Dermatology with deep-seated, firm in consistency, subcutaneous nodules and plaques on the face, neck, neckline, arms and buttock with high grade fever and fatigue, with no lesions typical...


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