Cutaneous, systemic features and laboratory characteristics of late- versus adult-onset systemic lupus erythematosus in 1006 Thai patients

Background Age at disease onset may modulate systemic lupus erythematosus (SLE), but its relation to cutaneous/extracutaneous manifestation remains understudied. Objective To compare the cutaneous, systemic features, laboratory characteristics, and disease severity between late- and adult-onset SLE patients Methods Analyses of the cutaneous, systemic involvement, laboratory investigations, SLE disease activity index 2000 (SLEDAI-2K), and disease damage were performed to compare between groups. Results Of 1006 SLE patients, 740 and 226 had adult- (15–50 years) and late-onset (>50 years), respectively. Among 782 with cutaneous lupus erythematosus (CLE), acute CLE (ACLE) and chronic CLE (CCLE) were more common in the adult- and late-onset SLE, respectively ( p = 0.001). Multivariable logistic regression analysis demonstrated that male patients and skin signs, including papulosquamous subacute CLE, discoid lupus erythematosus, and lupus profundus, were associated with late-onset SLE (all p < 0.05). Late-onset SLE had lower lupus-associated autoantibodies, and systemic involvement (all p < 0.05). ACLE, CCLE, mucosal lupus, alopecia, and non-specific lupus were related to higher disease activity in adult-onset SLE (all p < 0.001). There was no difference in the damage index between the two groups. Conclusions Late-onset SLE had a distinct disease expression with male predominance, milder disease activity, and lower systemic involvement. Cutaneous manifestations may hold prognostic values for SLE.

A Longstanding, Persistent and Recurrent Case of Cryptogenic Panniculitis

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous T-cell lymphoma. There may be a significant histologic overlap with traumatic panniculitis and lupus profundus. We describe a 54-year-old woman who had received a diagnosis of SPTCL based upon a left parietal scalp biopsy 5 years earlier. This diagnosis was supported by immunohistochemistry (IHC) demonstrating a CD8+ predominant lymphocyte population in the subcutis. T-cell gene rearrangement studies were not performed at that time. The patient was treated and showed significant clinical improvement. When several tender erythematous subcutaneous nodules appeared on the upper back, left plantar surface and pretibial region, repeat biopsy was performed. Histology revealed a lobular and septal panniculitis with no vasculitis. The infiltrate contained abundant eosinophils and histiocytes not seen in the original biopsy specimen. IHC demonstrated a mixture of CD4+, CD8+ and CD7+ lymphocytes with abundant CD68+ histiocytes. T-cell gene rearrangement studies performed on one of the lesions failed to demonstrate clonality. It is important to recognize that patients with SPTCL are not exempt from other types of panniculitis, and complete histologic, IHC and molecular workups are essential to properly classify all cutaneous lesions in these patients.

Bilaterally symmetrical lupus profundus with livedo reticularis as a sole cutaneous manifestation in a case of systemic lupus erythematosus

<p>A 31-year-old female diagnosed as a case of systemic lupus erythematosus (SLE) two years back with no cutaneous lesions, with lupus nephritis and anti-ds DNA positivity presented with the hardness of skin over the inner aspect of both thighs for four months associated with mild pain and itching. Lupus profundus was diagnosed clinically and histopathologically. She had livedo reticularis over the left leg, which can be a sign of the central nervous system's future involvement. The recent development of bilaterally symmetrical lupus profundus over thighs and livedo reticularis in a case of SLE with lupus nephritis diagnosed two years back is a rare presentation. </p>

EP10 A rare cause of lobular panniculitis

Background Weber-Christian disease is a rare inflammatory disorder of the subcutaneous fatty tissue. It is characterised by painful relapsing, subcutaneous nodules occurring preferentially at the upper arm, thigh and trunk regions. The disease is often accompanied by recurrent temperatures and constitutional symptoms. Methods We aimed to increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. Results A 65 year old female had been investigated thoroughly within a period of 7 years by a number of dermatology and rheumatology departments of North West hospitals. Symptoms started after mastitis with skin rash in form of macular blanching on upper abdomen and episodes of fever ranging between 38 and 40 degree Celsius, fatigability and arthritis. C-reactive protein was 300 mg/l, Erythrocytes Sedimentation Rate 150 mm/H and haemoglobin 95 g/L, positive lupus anticoagulant and negative anti-nuclear antibodies as well as extractable nuclear antibodies and normal complement levels C3 and C4 . Investigations results showed no evidence of infection, malignancy, vasculitis or connective tissue disease. PET scan revealed subcutaneous fat inflammation with increased uptake in mediastinum fat, initial skin biopsy showed lobular panniculitis with mild dermatitis suggestive of lupus profundus. Symptoms and inflammatory markers responded well to steroids however all tried DMARDs (methotrexate, azathoprime, hydroxychlorquine and mycophenolate) as well as dapsone and colchicine were not successful. In addition, 2 doses of rituximab were also ineffective in controlling her disease. Following a required excessive steroid therapy she developed cataract, osteopenia, and diabetes and became cushingoid. Repeated biopsy from the skin showed mixed inflammatory lobular pannicuilitis with neutrophils, lymphocytes and histiocytic. As per Dermatology Multi-Disciplinary Team the diagnosis of Weber-Christian disease was raised. As her condition continued to be very active she was also referred to London, where treatment with anakinra was organised. Conclusion The diagnosis of Weber-Christian disease depends on the presence of relapsing fever, systemic inflammation and panniculitis and prognosis varies depending on the response to steroids or DMARDs. For diagnosis you need to exclude all likely differential diagnoses of lobular panniculitis, like infections, certain malignancies, alpha-1-antitrypsin deficiency, pancreatitis and systemic lupus erythematosus. In conclusion, the patient has symptoms and signs as well as investigation results in keeping with Weber-Christian disease. Due to the rare nature of this disease the patient went through, multiple rheumatology and dermatology reviews and investigations and treatment trials over a period of 7 years prior to the correct diagnosis being reached. We believe reporting this case would raise awareness and improve on future patients journeys and care. Disclosures T. mudawi None. C. Amoasii None. A. Clewes None.