Epidermolysis bullosa acquisita, also known as acquired epidermolysis bullosa, is a longterm autoimmune blistering skin disease. It generally presents with fragile skin that blisters and becomes red with or without trauma. Marked scarring is left with thin skin, milia and nail changes. It typically begins around age 50-years.It is caused by antibodies to type VII collagen within anchoring fibril structures located at the dermoepidermal junction in skin. Damaged skin may become infected.Diagnosis is by observing the persistence of the condition, direct immunofluorescence, and detecting autoantibodies against type VII collagen. It can appear similar to porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, or blistering drug eruption. The condition is longterm and has no cure. A good response may be seen with corticosteroids, either alone or combined with azathioprine or dapsone.It is rare, with around 0.08 to 0.5 new cases per million people per year, and it affects males and females equally. Signs and symptoms
It generally presents with fragile skin that blisters and becomes red with or without trauma. Marked scarring is left with thin skin, milia and nail changes. It typically begins around age 50-years. Cause
It is caused by antibodies to type VII collagen within anchoring fibril structures located at the dermoepidermal junction in skin. Diagnosis
Diagnosis is by observing the persistence of the condition, direct immunofluorescence, and detecting autoantibodies against type VII collagen. It can appear similar to porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, or blistering drug eruption. Treatment
The condition is longterm and has no cure.
Plummer–Vinson syndrome is a rare disease characterized by difficulty swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome. While exact data about the epidemiology is unknown, this syndrome has become extremely rare. The reduction in the prevalence of Plummer–Vinson syndrome has been hypothesized to be the result of improvements in nutritional status and availability in countries where the syndrome was previously described. It generally occurs in perimenopausal women. Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx. Presentation
Patients with Plummer–Vinson syndrome often have a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny, red, dorsum of the tongue. Symptoms include:
Dysphagia (difficulty swallowing)
Pain
Weakness
Odynophagia (painful swallowing)
Atrophic glossitis
Angular cheilitis
Koilonychia (Abnormally thin nails, also called spoon nails)
Splenomegaly (an enlarged spleen). Upper esophageal webs (post cricoid region -  contrasts with Schatzki rings found at the lower end of esophagus).Serial contrasted gastrointestinal radiography or upper-gastrointestinal endoscopy may reveal the web in the esophagus. Blood tests demonstrate a hypochromic microcytic anemia that is consistent with an iron-deficiency anemia. Biopsy of involved mucosa typically reveals epithelial atrophy (shrinking) and varying amounts of submucosal chronic inflammation. Epithelial atypia or dysplasia may be present. It may also present as a post-cricoid malignancy which can be detected by loss of laryngeal crepitus. Laryngeal crepitus is found normally and is produced because the cricoid cartilage rubs against the vertebrae.