Disease Name
Chinese name: gangrenous septicemia
English name: pyoderma gangrenosum
Alias: Necrotizing pyoderma
Disease Overview
The disease presents as a destructive necrotizing, non-infectious skin ulcer with clinical appearance of boil-like nodules, pustules or hemorrhagic blisters. In terms of early nodular erythema or pustules, the disease can be attributed to vasculitis. Tender nodular erythema, initially red, later turning blue centrally and eventually forming an ulcer. One or more blistering pustules resemble acne, folliculitis, transient acantholytic dermatosis, or herpes-like dermatitis. The two lesions may appear simultaneously or may transform into each other. The lesions may occur on normal skin or on the site of a pre-existing skin disease. Painful ulcers with submerged margins and oozing yellow-green pus with a foul odor are diagnostic. Once the diagnosis is made, high doses of oral corticosteroids are administered.
Etiology of the disease
It has been demonstrated that patients with this disease have a defective delayed response to DNCB, Candida and streptokinase. This may explain the extreme hypoplasia of the reticuloendothelial system, which allows for the development of lesions when there is minimal damage or injury, and the hypersensitivity of new lesions to needling, especially in the acute phase of the disease and in the vicinity of the lesions. A serum skin necrosis factor has been shown to cause skin necrosis in guinea pig skin, but its specificity is unknown. Recognized evidence of a defective immune mechanism is the presence of gammopathy, heteroplasmosis, T-cell dysregulation, or phagocytic defects in numerous patients.
Pathogenesis
The pathogenesis of the disease is unknown and may be a Shwartzman reaction. The low or abnormal immune response in many patients with this disease suggests that the disease is associated with a defective immune system.
Histopathology
The nonspecific changes appear as aseptic abscesses in which venous and capillary thrombosis, hemorrhage, necrosis, and mast cell infiltration are present. Coagulation is an important manifestation. Presentation of lymphocytic vasculitis at the active margins suggests that the vascular endothelium is an early target organ. Early lesions resemble Behcet’s disease and neutrophilic dermatitis. It is also partially similar to leukocytoclastic vasculitis with more polymorphonuclear leukocytes in the infiltrating cells and also epithelial cells and giant cells especially in chronic cases with significant mononuclear cells and even epithelioma-like hyperplasia Pathological examination can exclude amebiasis and deep fungal infections.
Clinical manifestations
The primary lesions, depending on the depth of involvement, may present as.
1, Tender nodular erythema, initially red, later turning blue centrally and eventually forming an ulcer health search.
2. One or more blisters or pustules, similar to acne, folliculitis, transient acantholytic dermatosis or herpes-like dermatitis. The two lesions may appear simultaneously or may be transformed into each other.
3. The lesions can occur on normal skin or on the site of the original skin disease. The primary lesion gradually edematous, and the rapid formation of ulcers, clear boundaries, light blue margins, often thickened and elevated, sometimes uneven and submerged destruction of the central ulcer base is red, varying shades, like a crater, the surface with foul-smelling yellow-green pus ulcers around the early surrounded by a red halo. The lesions are continuously expanding eccentrically in all directions due to capillary-venous thrombosis of the skin and subcutaneous tissue. The ulcers vary in size, with those as small as a soybean reaching 10 cm or more in diameter. The number of lesions is large, up to a hundred, and the lesions are painful, but in some cases they are not painful for a long time and can heal spontaneously, leaving an atrophic sieve scar. They are often not accompanied by lymph node or lymphatic vessel lesions.
Clinical features
The lesions are usually found on the lower extremities, buttocks, and trunk. The initial lesions are varied and may be red papules, blisters, pustules or nodules. The initial lesions rapidly necrotize and form ulcers within a short period of time, with purplish-red skin at the edges of the ulcers, subterranean destruction of the underlying tissue, and a flushed granular base with foul-smelling yellow-green pus on the surface, often with light yellow-green oyster shell-like obsessions. The center of the ulcer heals continuously, while expanding telecentrically in all directions, and the ulcer can reach the fascia in huge cases, leaving a hypertrophic scar and sieve-like atrophic scar after healing. There is severe local pain and pressure pain.
Laboratory tests
Due to unknown etiology, no relevant content is currently described.
Ancillary examinations
Diagnosis is made on the basis of painful ulcers with submerged margins and exudation of yellow pus with a foul odor. The histopathology is mainly necrotizing vasculitis changes with occlusion of the lumen of small vessels, thrombosis, inflammatory cell infiltration with degenerative necrosis of the vessel wall, followed by skin ulceration and necrosis.
Differential diagnosis
Differentiate from the following diseases.
1, Behcet’s disease sudden onset, pustular component of lymphocytes without ulcerated lesions without scarring after healing.
2, postoperative progressive gangrene Most often seen in the chest or abdomen, often a single damage can be isolated from the lesions microaerobic streptococci, which are sensitive to antibiotics.
3, Meleney gangrene Subcutaneous ulcers are similar to this disease, but nowadays infections caused by Clostridium perfringens are uncommon.
4, Wegener’s granulomatosis There are multiple organ damage lesions polymorphic, the respiratory tract is the site of prevalence, C-ANCA positive .
5, fulminant purpura The lesions are more widely distributed and progress more rapidly.
6.Amoebiasis, cryptococcosis and budding bacteriosis can be clarified by microbiological and pathological examination .
Disease treatment
It is not difficult to treat this disease by using the external treatment method of Chinese medicine, which can soften the knots and replenish the qi, break the silt, move the silt and eliminate the swelling, and lift the residual poison outside the body.
Disease care
1. Remove the necrotic tissues on the surface of the ulcer, rinse with hydrogen peroxide and then saline, disinfect the ulcer surface and surrounding skin with Aner’s iodine type III once or twice a day. The ulcer surface can be alternately coated with safflower injection and gentamicin injection + 50% glucose once every half hour to promote wound healing, and after two weeks, safflower can be boiled with 6~8 layers of gauze for half an hour and then applied wet and hot, 30 minutes~1 hour/time, 3 times/day. Gentamicin injection or safflower injection, wet compress after still exposing the wound surface.
2.In the early stage when there is much pus secretion, after rinsing with the above method, the method of gooseneck lamp irradiation is used 2~3 times a day for 30 minutes/time, and the number of times of gooseneck lamp irradiation is changed according to the exudation of the trauma.
3, the person after two weeks of hospitalization, the ulcer surface purulent exudate is obviously reduced, around which fresh granulation tissue is visible, the ulcer surface is treated with exposure therapy, the patient’s quilts are irradiated with ultraviolet light twice a week for 30 minutes / time, and the nursing operation is strictly performed aseptically to prevent medical source infection.
4, psychological care Because of the long history and recurrent attacks, patients are often anxious, nervous, silent, and lack of sufficient knowledge about whether the healing is good, should do a good job of explaining and comforting work, take the initiative to introduce the relevant knowledge of gangrenous sepsis, treatment and prognosis, so that patients establish confidence, communicate more with patients, explain relevant knowledge to their families and friends, so as to encourage patients to actively cooperate with treatment Care.
Prognosis of the disease
Although some people think that the prognosis of this disease will leave hidden problems due to the involvement of the immune system, the prognosis is relatively satisfactory after the cure of herbal external treatment method as long as the patients usually pay attention to the avoidance of food and strengthen nutrition.
Dietary guidance
Due to the long duration of the disease, the ulcer surface is deep and large, and the restriction of fish and meat food for a long time has led to malnutrition and decreased resistance of the body. Therefore, patients should be given high protein, high vitamin and easily digestible food, more eggs, milk, fresh vegetables, fruits and collagen-rich food such as thick chicken soup, etc. Avoid eating spicy and stimulating things, and quit smoking and alcohol.