Xanthoma disseminatum with respiratory tract involvement and fatal outcome

Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans' cell histiocytosis. The case history is presented of a 61 year old woman with XD who developed dyspnoea and spirometric features of airflow obstruction. Bronchoscopy and computed tomography confirmed involvement of the large and medium sized bronchi and she subsequently died from acute respiratory failure. The post-mortem findings and the importance of respiratory tract disease in this unusual condition are discussed.     

Cicatricial pemphigoid and lichen sclerosus

Two patients with clinical and laboratory evidence of co-existing lichen sclerosus and eicatricial pemphigoid are reported. Autoimmune bullous diseases affecting the vulva may mimic lichen sclerosus but in these two patients both diseases were present. These two diseases have not previously been reported simultaneously to date.     

The quality-value proposition in health care

Powerful forces are converging in US health care to finally cause recognition of the inherently logical relationship between quality and money. The forces, or marketplace "drivers," which are converging to compel recognition of the relationship between cost and quality are: (1) the increasing costs of care; (2) the recurrence of another medical malpractice crisis; and (3) the recognition inside and outside of health care that quality is inconsistent and unacceptable. It is apparent that hospital administrators, financial officers, board members, and medical staff leadership do not routinely do two things: (1) relate quality to finance; and (2) appreciate the intra-hospital structural problems that impede quality attainment. This article discusses these factors and offers a positive method for re-structuring quality efforts and focusing the hospital and its medical staff on quality. The simple but compelling thesis of the authors is that health care must immediately engage in the transformation to making quality of medical care the fundamental business strategy of the organization.     

Drug-associated linear IgA disease mimicking toxic epidermal necrolysis

Linear IgA disease (LAD) is an uncommon, acquired, autoimmune blistering disorder with a characteristic linear IgA deposition along the basement membrane zone. LAD can be idiopathic or drug-induced. Drug-related LAD most commonly occurs after exposure to vancomycin, but phenytoin and other medications have been implicated. A small number of cases of drug-induced LAD clinically resembling TEN have been reported. We report a case of phenytoin-associated LAD clinically mimicking TEN in a 57-year-old woman. This case shows the necessity of investigating TEN patients for autoimmune bullous diseases, using immunofluorescence studies.     

Genital lichen sclerosus associated with morphoea or systemic sclerosis: clinical and HLA characteristics

Although patients with both morphoea and lichen sclerosus have been reported previously, in the majority of these reports the lichen sclerosus has been extragenital. We report nine patients in whom genital lichen sclerosus coexisted with scleroderma spectrum disorders including seven with morphoea, one with morphoea and lichen planus, and one with systemic sclerosis. The clinical features, associated autoimmune disease, autoantibodies and HLA type are reported. Antibodies to Borrelia burgdorferi were not detected in any of the patients. The coexistence of these diseases raises a number of intriguing questions about the relationship between them.