Marko Barešić

The lobster sign in SAPHO syndrome: unusually extensive osteitis of the anterior chest wall partially responsive to infliximab

The synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is an uncommon entity comprising several osteoarticular and cutaneous features [1]. Osteitis and hyperostosis remain key diagnostic features, since the proposed clinical criteria [2] have never been validated, especially regarding the distinction between the SAPHO syndrome and psoriatic arthritis [3]. Tumor necrosis factoralpha (TNF-a) antagonists are starting to play an important role in the treatment of patients inadequately responsive to conventional treatment [4]. We present a 48-year-old female patient diagnosed with SAPHO syndrome 5 years ago. She has been treated with nonsteroidal anti-rheumatics, sulfasalazine and methylprednisolone, with partial and unsatisfactory response in terms of clinical and laboratory features, as well as radiological and bone scan findings [5, 6]. Four years after initiating conventional treatment, she underwent a reevaluation to assess disease extent and activity. Physical examination revealed palmoplantar pustulosis and multiple joint tenderness, including sternoclavicular, costochondral, sacroiliac and peripheral joints. Laboratory investigation revealed elevated inflammatory markers, also suggesting disease activity. A technetium 99-m bone scan was subsequently performed [5]. Increased tracer uptake was observed in both sternoclavicular joints, the sternum, first ribs bilaterally, fifth and sixth ribs near the costosternal junctions and the anterior portion of the eighth left rib, resembling a lobster. It was also revealed in the right hip and pubic bone, as well as in the pubic symphysis (Fig. 1). Less pronounced accumulation was noticed in the L4 and L5 vertebrae. Infliximab was added to the treatment, leading to an almost complete regression of osteoarticular complaints and normalization of laboratory findings. However, a follow-up bone scan performed after the fourth application of infliximab revealed a pattern of tracer accumulation almost identical to the one described previously. Moreover, psoriasiform skin lesions developed on the palms and trunk following the introduction of the biological agent: although similar lesions were observed before, they were now more pronounced. The skin lesions disappeared within several weeks following the fifth application of infliximab. TNF-a antagonists are included in standard treatment strategies for seronegative spondyloarthropathies; however, their use in the SAPHO syndrome is still considered as off-label [4]. This might change in the future due to new insights into their role on the molecular level [7] and an increasing number of individual reports suggesting a positive impact on disease activity [4]. Nevertheless, some questions still remain to be answered. Our patient experienced a temporary aggravation of cutaneous lesions, which is in accordance with other authors’ findings [8]. The aggravation is probably a side effect of infliximab and not a result of worsening of the disease course. Furthermore, the impact of infliximab on bone tracer uptake should also be addressed. Although an alleviation of osteoarticular complaints was observed soon after the beginning of the biological treatment, no regression was observed on the control B. Anić I. Padjen (&) M. Barešić Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Kispaticeva 12, 10000 Zagreb, Croatia e-mail: ivan_padjen@yahoo.ca

Improvement of overlapping hidradenitis suppurativa and ankylosing spondylitis after the introduction of adalimumab

Hidradenitis suppurativa is a chronic inflammatory disorder characterized by occlusion of the follicular pilosebaceous units of the skin. The treatment options are sometimes very limited and unpleasant odor and abundant drainage complicate the disease. Ankylosing spondylitis is a form of seronegative spondyloarthritis with predominantly axial but also peripheral joint involvement. Both of the conditions lower the patient’s quality of life and affect everyday activities. We describe a 39-year-old male patient with both diseases treated with different medications with only a modest result. After the initiation of a tumor necrosis factor α (TNF-α) inhibitor (adalimumab) the patient experienced first the musculoskeletal and later on the skin improvement. The introduction of TNF-α inhibitors should be considered early in the treatment of overlapping hidradenitis suppurativa and the spondyloarthritis spectrum of conditions. Available medical data confirm the positive results and beneficial effect on disease course, activity and, most importantly, quality of life.

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

Schnitzlers syndrome is a rare autoinflammatory syndrome with unidentified mechanism of disease and etiology with unknown definitive treatment algorithm. The two obligatory criteria for the diagnosis of Schnitzlers syndrome include chronic urticarial rash and monoclonal gammopathy (immunoglobulin M or immunoglobulin G). In this article, we describe two patients with different courses of disease with different average lengths of time between initial symptoms and the final diagnosis (6 months to 8 years). Exclusion of more common conditions is needed to ensure the correct diagnosis. Treatment strategy depends on the patients constitutional symptoms (fever, malaise, generalized myalgia, and arthralgias) and laboratory tests of inflammation. Treatment includes usage of conventional drugs and cytokine blockade (interleukin-1 and interleukin-6). Further studies are needed to determine the precise mechanism of disease and the appropriate targeted therapy.

Eosinophilia-myalgia syndrome induced by excessive L-tryptophan intake from cashew nuts

Eosinophilia is characterized by more than 0.5 × 109 eosinophils per liter in the full blood count. A wide range of conditions, from asthma to parasitic infections, autoimmune diseases, and certain forms of cancer, have been known to trigger abnormally high amount of eosinophils. It is essential to reach the correct diagnosis and treat the underlying disease aggresively. Definition of the eosinophilia-myalgia syndrome was offered in 1980s by Centers for Disease Control and Prevention for surveillance purposes, and criteria were revised in 2001, with high specificity. We report a case of 59-year old female who started a special weight-reducing diet regimen that included excessive cashew nut ingestion. Several months after she has presented with periferal blood eosinophilia and constitutional symptoms. Detailed work-up has not found elements for haematological, systemic autoimmune, neoplastic or infectious disease. She was diagnosed with eosinophilia-myalgia syndrome due to extreme L-tryptophan intake, a compound found in the cashew nut’s oil. She responded well to cashew nut withdrawal and steroid therapy. In the follow-up period she remained stable with normal eosinophil count and there was not a need for any specific therapy.