Bullous systemic lupus erythematosus

Bullous systemic lupus erythematosus (BSLE) is rare heterogeneous cutaneous manifestation in patient with systemic lupus erythematosus (SLE). BSLE encompasses a subepidermal autoimmune bullous disease with type VII collagen autoantibodies, leading to epidermolysis bullosa acquisita in patients with SLE. Alternatively, an acute generalized hemorrhagic vesiculo-bullous eruption may also occur in patients with SLE caused by the extensive inflammatory reaction without the presence of type VII collagen. Vesicular eruptions can also be seen in subacute cutaneous lupus erythematosus due to severe inflammatory reaction with subepidermal clefting, which in extreme cases may resemble erythema multiforme (Rowell syndrome) or toxic epidermal necrolysis.</p

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is a subtype of pemphigoid that may present with scarring similar to hereditary dystrophic epidermolysis bullosa, of which the naming of EBA was derived. The clinical subtype of EBA with scarring is named mechanobullous EBA, because blisters are evoked by sudden mechanical trauma to the skin. The other subtype of EBA with erythematous lesions without scarring is named inflammatory EBA, and may look like bullous pemphigoid. The mucous membranes can be involved in both subtypes. The pathogenesis is mediated by IgG or IgA against type VII collagen, which is the component of anchoring fibrils below the lamina densa. Diagnosis is confirmed by detection of a u-serrated linear pattern of immune depositions by direct immunofluorescence microscopy of a skin biopsy. The pathogenesis of both clinical subtypes is unknown, and is not related to binding of a particular epitope of the auto-antigen. EBA is associated with systemic lupus erythematosus and colitis ulcerosa. The disease is relative refractory to treatment.</p

Drug-induced pemphigoid and linear IgA disease

Drug-induced pemphigoid (DIBP) and drug-induced linear IgA bullous dermatosis (DILAD) can be difficult to differentiate from idiopathic bullous pemphigoid (BP) or mucous membrane pemphigoid (MMP), respectively linear IgA disease (LAD). Possible absence or only minor differences in clinical, histopathologic and immunopathologic features complicate the recognition. However, differentiation can be of major importance because of a different approach, prognosis, and treatment. Diagnosis is mainly based on a clear time-relation between start of the suspected drug(s) and onset of the lesions, but can be complicated by polypharmacy and comorbidity, especially in the elderly. After withdrawal of the culprit drug, both DIBP and DILAD tend to be self-limiting. With the introduction of immune checkpoint inhibitors in treatment of malignancies, pemphigoid variants may present as an immune-related adverse event, leading to a dilemma in treatment choices.</p

Autoimmune bullous diseases in childhood

Autoimmune bullous diseases (AIBDs) rarely occur in childhood and in contrast to the chronic disease course in adults, often have a milder clinical course, a better treatment response and better prognosis. Although literature is scarce, several case reports and case series describe childhood cases of linear IgA disease, bullous pemphigoid, dermatitis herpetiformis, mucous membrane pemphigoid, epidermolysis bullosa acquisita, pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Misdiagnosis and consequently delay in diagnosis is common in childhood AIBD with often initial diagnoses of infectious causes, such as hand-foot-mouth disease or impetigo bullosa. Clinical features of childhood AIBDs may overlap between subtypes, like adult cases, but the differential diagnosis is different in childhood. Another difference between the adult and childhood bullous pemphigoid is the mainly acral distribution of bullous lesions in children and the prognosis of the disease. Bullous pemphigoid and linear IgA disease might possibly be drug-induced or triggered by vaccination.</p

Magnetic trapping of buffer-gas cooled chromium atoms and prospects for the extension to paramagnetic molecules

We report the successful buffer-gas cooling and magnetic trapping of chromium atoms with densities exceeding $10^{12}$ atoms per cm$^{3}$ at a temperature of 350 mK for the trapped sample. The possibilities to extend the method to buffer-gas cool and magnetically trap molecules are discussed. To minimize the most important loss mechanism in magnetic trapping, molecules with a small spin-spin interaction and a large rotational constant are preferred. Both the CrH ($^6\Sigma^+$ ground state) and MnH ($^7\Sigma^+$) radicals appear to be suitable systems for future experiments.Comment: 9 pages, 4 Figure

Diagnosis of pemphigoid diseases

Itch in elderly due to newly recognized autoimmune diseases pemphigoid With ageing comes itch, a common and burdensome complaint in elderly people and comparable to chronic pain. Itch in elderly is related to ageing of the skin, and possibly due to the recently identified autoimmune disease nonbullous pemphigoid. The studies in the thesis are focused on diagnosis of this disease, to support early recognition. Pemphigoid is a itching skin blisering disease. However, one in four patients does not have skin blistering, but does have complaints of itch. The disease is associated with ageing and most common in persons above 70 years. With the SSENIOR study, we analyzed the prevalence of itch in nursing home residents, and presence of pemphigoid as the cause of itch. The results showed almost half of the 125 participating nursing home residents had complaints or signs of itch, often of chronic duration and in cases of dementia often not recognized. Half of the participants with moderate to severe itch had pemphigoid, with a total prevalence of 6% and 200-fold higher in this high-risk population compared to the general population. Nonbullous pemphigoid is a newly identified blistering disease without skin blisters and a frequent cause of pruritus in elderly, that is well treatable. Another study in this thesis used the database of the national Center for Blistering Diseases of the UMCG and showed the disease can be identified using blood testing, which enables screening and early recognition in a high-risk population