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Whipple's disease is characterized by systemic manifestations (intestinal and extraintestinal), which can lead to serious pathologies of the heart, adrenal glands, and nervous system. In the absence of proper treatment, the patient may die within two to three years after the development of bowel dysfunction.
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The pathohistological picture is characterized by dystrophy of salbutamol fibers and compaction of collagen, dystrophy of the skin appendages (see), the presence of perivascular inflammatory infiltrates, comedones (see Acne), cysts of sebaceous hair sacs (see Cyst).
- With Favre-Rakushko disease, the skin is thickened (sometimes soft), uneven, red-brown in color, with wrinkles, comedones, yellowish-white cysts, deeply located small dense nodes are noted; with a long-term process, senile keratomas occur (see. Keratoses), sometimes basaliomas (see).
- The diagnosis is established on the basis of the clinical picture. Differential diagnosis is carried out with red acne, diffuse Dubrey's elastoma and rhomboid hypertrophic skin of the neck (see Skin dystrophy), as well as with a dystrophic form of epidermolysis bullosa (see Epidermolysis bullosa) and keloid folliculitis (see).
- For treatment, protective ointments, vitamin A, and vitreous injections are used. Prevention has not been developed; dispensary observation is necessary (due to the possibility of proventil).
Etiology and pathogenesis have not been established.
Attention is drawn to the development of elastoidosis of the skin, mainly in places exposed to the sun. It is often observed in elderly full-blooded obese men. Clinic. The altered skin in the back of the head, behind the auricles, on the temples and around the eyes is thickened, wrinkled, uneven, dense, red in color, on which there are many comedones of various sizes, deep nodes and cystic formations.
Differentiation should be with aknekeloid, diffuse Dubreuil's elastoma, colloid-milium.
Histologically, degeneration of elastic fibers, comedones and follicular cysts, sclerofibrous reaction of collagen, hair dystrophy and perivascular powerful infiltrates are detected. The pathohistological picture is characterized by dystrophy of elastic fibers and thickening of proventil, dystrophy of the skin appendages (see the full body of knowledge), the presence of perivascular inflammatory infiltrates, comedones (see the full body of knowledge: Acne), cysts of sebaceous-hair sacs (see the full body of knowledge: Cyst).
For treatment, protective ointments, vitamin A, and vitreous injections are used.
When Favre - Rakusho disease, the skin is thickened (sometimes soft), uneven, red-brown in color, with wrinkles, comedones, yellowish-white cysts, deeply located small dense nodes are noted; with a long-term process, senile keratomas occur (see the full body of knowledge: Keratoses), sometimes basaliomas (see the full body of knowledge).
Synonyms of the Favre-Rakusho syndrome. Cutaneous nodular elastoidosis. Nodular cutaneous elastoidosis with cysts and comedones.
The diagnosis is established on the basis of clinical pictures. Differential diagnosis is carried out with red acne, Dubreuil's diffuse elastoma and rhomboid hypertrophic skin of proventil (see the full body of knowledge: Skin dystrophy), as well as with the dystrophic form of epidermolysis bullosa (see the full body of knowledge: Epidermolysis bullosa) and keloid folliculitis (see the full body of knowledge ). Definition of Favre-Rakusho syndrome. Colloidal-degenerative changes in the connective tissue of the skin in elderly men.
The disease is more common in fair-skinned men than in dark-skinned and women.
The prevalence of the disease in the 40-60-year-old population is estimated at 6%, in the range of 25-74 years - 1.4%. A study conducted among agricultural workers revealed the disease in 2.5% of those surveyed. The pathogenesis is unclear. Intense UV exposure is believed to cause elastin degeneration and structural changes in the skin leading to abnormal comedone expansion. An as yet unknown predisposition to the disease should be noted, as many people with chronic exposure to UV radiation do not develop the disease. Cases of salbutamol Inhaler development have been recorded (after 2 weeks) of the disease in patients receiving radiation therapy.
An increased incidence of the disease in patients receiving systemic or topical corticosteroid therapy.
There is a clear connection with smoking - in patients who smoke, the disease occurs much more often than in non-smokers. Unfavorable meteorological factors (wind, dry climate) play a certain role. The disease begins with the appearance of papules and yellow plaques with open, black, comedones on sun-exposed areas of the skin, which, unlike comedones, do not become inflamed in acne. Rashes are usually symmetrical and are localized mainly in the periorbital region, on the temples, cheeks, forehead, less often in the periauricular region, the rear of the hands and forearms.
Stop smoking and use topical and systemic corticosteroids.
As the disease progresses, the skin in the lesions thickens, thickens, gathers into coarse folds in the form of a "cobblestone pavement" and acquires a yellowish tint, sometimes dark. 1-1.5 cm in diameter and milia-like cysts 1-5 mm in size, filled with keratin. In severe cases, a "lion's face" is formed.Other diseases associated with the damaging effects of the sun are often found - actinic keratosis, lentigo, rhomboid skin of the neck and squamous cell carcinoma.
The diagnosis is made on the basis of a characteristic clinical picture, a biopsy is rarely required. Histologically, numerous epidermal cysts, basophilic degeneration of the connective tissue, atrophy of the sebaceous and sweat glands are observed in the dermis. A lymphohistiocytic infiltrate is found around the dilated skin vessels.
The disease is chronic and benign in nature and is not associated with systemic and malignant diseases.
Colloidal milium Miliums Acne vulgaris Comedonal nevus Syringomas Chloracne Seborrheic hyperplasia Actinic granuloma Actinic comedonal plaques Diffuse elastomer of Dubreuil Trichoepithelioma Apocrine hydrocystoma Rhomboid neck skin. The disease is difficult to treat. The following methods of treatment are used. Removal of elements of the rash. Extraction with an acne squeezer or microcurettage of comedones, surgical excision of plaques, removal of CO2 by a laser, dermabrasion and chemical peeling. They give a temporary effect, since the disease recurs again, and cause scarring.
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If the mentioned elements appear on the back of the neck, the so-called skin of sailors is formed. Histologically - thick dense skin, a decrease in the number of elastic fibers in the capillary layer and around the nerves and blood vessels. Mostly men are ill after 50 years. Handa-Schuller-Christian disease.