Primary with diabetes
insulin-dependent diabetes mellitus (type 1 diabetes mellitus).
Non-insulin-dependent diabetes mellitus (type 2 diabetes mellitus): non-obese, obese, juvenile non-insulin-dependent diabetes mellitus (MODY – maturity-onset diabetes of the young).
Surgical diseases of the pancreas, disorders of the secretion of hormones of the adrenal glands and adenohypophysis, exposure to toxic substances and drugs, syndromes of insulin resistance, other hereditary syndromes.
Skin damage in diabetes
Lipoid necrobiosis (see below).
Synonyms: scleroedema, adult sclerodema, Bushke’s disease. It occurs with diabetic microangiopathy. It manifests itself as persistent edema of the skin and subcutaneous tissue, which does not leave pits when pressed. The boundaries of the lesions are not expressed. The clinical picture is partly reminiscent of skin lesions with scleroderma. Localization – the upper back, neck and proximal limbs. The disease begins suddenly and progresses rapidly.
Calciphylaxis (see below).
In diabetes mellitus – generalized, polymorphic rashes.
Acanthosis nigricans and lipodystrophy
Develop with insulin resistance. Hyperplasia of the epidermis is caused by insulin-like growth factors.
They occur with decompensated diabetes mellitus and a very high level of triglycerides in the blood.
Synonym: bullosis diabeticorum. Bubbles appear on the rear of the hands, fingers, legs and feet (Fig. 1) suddenly, without previous inflammation; reach large sizes. Histological examination: subepidermal or intraepidermal cavities, no acantholysis.
Microangiopathy. The defeat of arterioles, venules and capillaries. Manifested by erythema of the extremities, resembling an erysipelas, sometimes – ulceration. Thickening of the vascular basement membrane and proliferation of endothelial cells are characteristic.
Macroangiopathy The defeat of large vessels. Most often, the vessels of the legs suffer. The consequences are ischemia, trophic ulcers, gangrene, a variety of infectious complications.
Patients with diabetes mellitus, especially decompensated, are predisposed to staphylococcal and streptococcal infections (furunculosis, carbuncle, paronychia, suppuration of wounds and ulcers, erysipelas, phlegmon), erythrasma, dermatophytosis of the feet, onychomycosis, candidiasis of the skin and mucous membranes and mucous membranes.
Usually – sensorimotor. Damage to the motor nerves is manifested by weakness and muscle atrophy; damage to the sensory nerves leads to neurotrophic ulcers on the foot, usually above the bony protrusions. Autonomic neuropathy is accompanied by anhidrosis.
Side effects of diabetes medications
Insulin. Local: post-injection lipodystrophy (in places of subcutaneous injection), the Arthus phenomenon (blisters in places of intradermal injection). General: urticaria, serum sickness.
Oral hypoglycemic agents. Spotted-papular rash, urticaria, polymorphic exudative erythema, photosensitivity.
Synonym: dermopathia diabetica. Round, atrophic, slightly sunken brown spots on the anterior surface of the legs. It is believed that circulatory disorders play a leading role in pathogenesis, since dermatopathy is often combined with diabetic microangiopathy.
Lipoid necrobiosis is manifested by multi-colored (red, yellow, brown) plaques with clear boundaries on the anterior and lateral surfaces of the legs. Diabetes is a common but not the only cause of the disease.
Synonyms: necrobiosis lipoidica, cutaneous dyslipoidosis, diabetic lipoid necrobiosis, Oppenheim-Urbach disease.
Epidemiology and etiology
Among patients with diabetes mellitus – less than 1%.
Usually – adults under 40 years old, but it also happens in children.
Women get sick 3 times more often.
Provoking factors The
onset of the disease is often preceded by trauma, so the typical localization of lipoid necrobiosis is the anterior surface of the legs and sections of the foot over the bony protrusions.
In most patients, many years after the onset of insulin-dependent diabetes mellitus.
Foci gradually increase in size over several months. Exist for years.
Cosmetic defect. With plaque ulceration, pain occurs.
One third of patients have diabetes mellitus, another third have impaired glucose tolerance.
Elements of the rash
Fresh foci: waxy plaques with clear boundaries (Fig. 2). The sizes are various. The surface is glossy, through the atrophied epidermis, dilated vessels of the dermis are visible.
Old foci: peripheral plaque growth, fusion of small foci; in the center – ulceration (Fig. 3). After healing of the ulcers, depressed scars remain. In the center of the plaque, where atrophy is most pronounced, there are multiple telangiectasias.
Healing foci: the surface of the plaques is smoothed out, the yellow color changes to brown. Teleangiectasia.
Colour. Fresh plaques – yellow, orange (an important diagnostic sign); the old ones are brown.
The form. Incorrect, since large plaques are formed due to the fusion of small ones.
Location Sometimes symmetrical.
Localization. In more than 80% of cases – legs; less commonly, feet, hands, torso, face, scalp; even less commonly, a generalized lesion.
Sarcoidosis, annular granuloma (often associated with lipoid necrobiosis), xanthomatosis.
Light microscopy. Collagen degeneration, destruction and disordered arrangement of collagen bundles. Connective tissue necrobiosis; granulomatous inflammation of the lower layers of the dermis. Xanthoma cells loaded with lipids give yellow color to plaques. Microangiopathy: proliferation of endothelial cells, deposition of polysaccharides in the walls of blood vessels (positive CHIC reaction).
Immunofluorescence staining. Immunoglobulins and complement component C3 are detected. In the walls of arterioles, capillaries and venules, immune complexes are sometimes deposited.
Glucose tolerance test
Glucose tolerance is impaired.
The appearance of the foci is so characteristic that a biopsy is not required. It is carried out only to exclude annular granulomas.
Granulomatous inflammation is believed to occur in response to collagen degeneration. The defeat of the arterioles in the foci of necrobiosis is apparently due to increased platelet aggregation. The severity of lipoid necrobiosis does not depend on the severity of diabetes. Moreover, normalizing blood glucose levels does not change the course of the disease.
Course and prognosis
Due to the absence of pain and itching, patients do not consult a doctor for a long time. Meanwhile, the disease progresses, plaques grow and lead to significant cosmetic defects. Pain appears only with ulceration. Ulcers can also heal without treatment.
External use. Occlusive dressings with potent corticosteroids help many patients, but occasionally provoke ulceration.
Injection into the lesion. The introduction of triamcinolone (5 mg / ml) into a growing (yellow) plaque, as a rule, stops its growth.
Treatment of ulcers
In most cases, conservative treatment is sufficient. With inefficiency, excision of the entire plaque followed by skin transplantation.
Calciphylaxis is a syndrome that manifests itself as progressive necrosis of the skin due to the deposition of calcium salts in the walls of small and medium vessels. Calciphylaxis is usually observed in chronic renal failure and hyperparathyroidism. First, foci of ischemia of irregular shape appear, then ulceration of the subcutaneous tissue, dermis and epidermis begins, gangrene develops. Secondary infections, sepsis are frequent.
Synonyms: calciphylaxia, calceria.
Epidemiology and etiology
Men and women get sick equally often.
Middle and old.
Chronic renal failure (uremia), diabetes mellitus, AIDS.
Even fresh foci are very painful.
. Many patients have diabetes mellitus. Chronic renal failure; calciphylaxis often occurs after patients are transferred to hemodialysis or peritoneal dialysis.
Elements of a rash. Foci of ischemia – spots, marbled skin pattern (Fig. 4A). Bubbles sometimes form above ischemic skin. Subsequently, necrosis of the epidermis and dermis, rejection of necrotic tissue and the formation of ulcers (Fig. 4B). If a secondary infection joins, phlegmon and sepsis are possible.
Colour. At first – dark red, cyanotic, violet. Then the heart attack zone turns black. Over the course of several weeks or months, the foci gradually expand.
Palpation. If there is no sensory neuropathy, even fresh foci are extremely painful. The fabric is sealed. Sites of necrosis do not have clear boundaries.
Localization. The distal extremities, most often calves and the lateral surface of the legs. Belly, buttocks. Fingers. The head of the penis.
Painful foci of ischemia
Panniculitis; vasculitis; lipoid necrobiosis; dystrophic calcification of the skin (calcification of scars, fibromas, cysts, etc.); metastatic calcification of the skin (calcification of the dermis, but not subcutaneous tissue); systemic scleroderma; atheroembolism; atherosclerosis obliterans; DIC (fulminant purpura); gangrenous pyoderma; warfarin necrosis, heparin necrosis; cladosporiosis; cellulitis caused by Vibrio vulnificus; putrefactive phlegmon.
Biochemical analysis of blood
Azotemia. The product of calcium x phosphorus is usually increased. (Serum concentrations of calcium and phosphates, expressed in mg%, are multiplied. A product higher than 70 is considered high).
As a rule, the level of the hormone in the blood is elevated.
Allows to exclude a secondary infection.
Pathomorphology of the skin
An incisional biopsy is indicated. Calcification of the media of small and medium arteries of the skin and subcutaneous tissue. In the lumen of blood vessels – fibrin thrombi. Ischemia leads to necrosis of adipose tissue (fat lobules and connective tissue layers). In the subcutaneous tissue – loose infiltrates from lymphocytes and histiocytes.
Visible calcified small and large vessels of the affected limb.
Renal failure in the anamnesis; characteristic clinical picture; elevated levels of parathyroid hormone, high product of calcium x phosphorus; skin biopsy results.
Pathogenesis is not well understood. The pioneer of calciphylaxis G. Selye defined it as an acquired state of increased sensitivity of the body to calcium. In animal experiments, it was shown that two factors are involved in the development of calciphylaxis – sensitizing and resolving. In response to the action of the resolving factor, calcium salts are deposited in the tissues. In humans, calciphylaxis develops in chronic renal failure, secondary hyperparathyroidism, and high production of calcium x phosphorus. The decisive factors are corticosteroids, albumin infusion, i / m injections of tobramycin, glandular dextran, heparin calcium salt, immunosuppressants, vitamin D.
Course and prognosis
As a rule, the disease progresses gradually, despite treatment. Due to tissue ischemia and the attachment of a secondary infection, severe pain occurs. In the later stages of gangrene of the fingers or penis can lead to self-amputation. The disease is often complicated by phlegmon and sepsis. In general, the prognosis is unfavorable, mortality is very high.
Basic principles: early diagnosis, treatment of renal failure, resection of the parathyroid glands, excision of necrotic tissue. Avoid corticosteroids and other triggering factors.
High product of calcium x phosphorus. Phosphate binders are prescribed internally. In patients undergoing hemodialysis or peritoneal dialysis, they lower the level of calcium in the blood, reducing the concentration of calcium in the dialysis solution.
Antibiotics. Necessary for secondary infections.
Renal failure. Dialysis.
Excision of necrotic tissue. All necrotic tissue is excised and the wound is closed with a split skin flap.
Subtotal resection of the parathyroid glands. Effective in secondary hyperparathyroidism. The earlier the operation, the better.
Amputation. Indications: extensive gangrene, severe infectious complications, excruciating pains that cannot be eliminated by analgesics.
Kidney transplantation. In some cases, very effective.