Sebaceous lesions and their associated syndromes: part II.

Sebaceous lesions are associated with two syndromes with widespread multisystem disorders and tumors. Linear sebaceous nevus syndrome has been traditionally known as the triad of sebaceous nevus of Jadassohn, seizures, and mental retardation. This syndrome encompasses a much broader spectrum of multisystem disorders, which is explored below.

Sebaceous lesions and their associated syndromes: part I.

Sebaceous neoplasms have long been a source of confusion to dermatologists and pathologists alike. Disagreements regarding nomenclature, classification, and management have been longstanding. Sebaceous lesions represent a broad spectrum of interesting entities that range from hamartomas, hyperplasias, and benign tumors to highly malignant neoplasms.

Acrodermatitis enteropathica and an overview of zinc metabolism.

Acrodermatitis enteropathica is a rare autosomal recessive disorder of zinc deficiency. The genetic defect has been mapped to 8q24 and the defective gene identified as SLC39A4, which encodes the zinc transporter Zip4. The diagnosis is made by way of clinical presentation together with histopathology and laboratory tests.

Drug-induced linear IgA bullous dermatosis.

A 73-year-old man was admitted to the University of California Davis Medical Center for treatment of a pleural effusion and congestive heart failure. His hospital course was complicated by asymptomatic sustained ventricular tachycardia requiring placement of an implantable cardiac defibrillator. The patient was treated with vancomycin and cefazolin during the procedure.

Acute hemorrhagic edema of infancy.

An 11-month-old boy initially presented to an outside hospital with fever, rhinorrhea, swelling, and papular lesions involving the left foot. He was diagnosed with necrotizing fasciitis and he subsequently underwent debridement of the lower left leg. Tissue cultures were submitted and were negative.

Receptor editing in peripheral B cell tolerance.

Receptor editing or secondary Ig gene rearrangement occurs in immature, autoreactive B cells to maintain self-tolerance. Here we show that nonspontaneously autoimmune mice immunized with a peptide mimetope of DNA develop peptide- and DNA-reactive antibodies. Antigen-specific B cells display a follicular B cell phenotype.