Adult hard swelling disease, first described in 1886 by Piffard's, in 1902 by Buschke's report, he said Buschke scleredema (scleredema of Buschke), while the common name is the adult scleredema (scleredema Adultorum), but children and many see, Carrington's statistics, more than half of the patients under 20 years of age. Its characteristics are more extensive skin non-pitting edema refers sclerosis. Before the onset of acute infection often have a history (90% ~ 95%), especially after the onset of common hemolytic streptococcus infection. Rash often begin by neck, sides of neck or head, and gradually spread to the face, shoulders, upper chest, upper arm proximal and back, individual patients until the buttocks, abdomen and thighs, usually very light distal limbs, hands and feet and more are not involved, genitalia often without lesions. Swelling of the non-means concave, tough and hard, often described as waxy, stiff, plate, cartilage-like, doughy or wooden shape. Not pinch the skin, skin lesions edge is unclear, loss of normal skin surface wrinkles, facial lack of expression, but there is no skin atrophy, pigmentation and hair loss and so on. Damage to the neck and shoulder is particularly significant. Few patients have pericardial, pleural and peritoneal exudate, heart palpitations, shortness of breath and difficulty breathing. Tongue edema damage caused difficulty swallowing. Eye damage including protruding eyeball, eyelid edema and significant conjunctival edema, but also have tachycardia, ECG abnormalities, double parotid gland enlargement, hepatosplenomegaly, joint effusion, bone changes.
The disease is currently unknown etiology, often after the onset of infection, some people think may be due to infection caused by an autoimmune process, is hard acknowledged. The disease is self-limiting, usually 2 to 6 weeks to reach the peak, more than about half of the patients cured or substantially improved within two years, there are also extended to more than two years subsided. And within six months subsided by about 29% (Greenberg's). Sclerosis lesions subsided starting at the site of the latest occurred, the prognosis does not leave traces, but can recur.
Pathological changes: the epidermis was normal, and sometimes sudden disappearance of the epidermis, the basal layer of slightly flattened. Visible mild perivascular lymphocytic upper dermis, tissue cells, plasma cells. Reticular dermis thickening, swelling of collagen fibers, homogenized collagen bundles separated significantly increased iron glue positive acid mucopolysaccharides on a separate site. Hair follicles, sebaceous or sweat glands can be seen in most cases.
In recent years, found a different form of hard swelling disease, characterized by severe disease occurs in patients with lesions, longer duration of obesity diabetes, known as diabetic scleredema or longevity of adult scleredema. |
