symptoms of primary local cutaneous amyloidosis, early symptoms and signs of primary local cutaneous amyloidosis
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Symptoms of primary localized cutaneous amyloidosis
Typical symptoms: Brown spots of needle size, then become pointed papules and gradually increase, flat bulge, hemispherical, conical or polygonal, with a diameter of about 2mm.
Related symptoms: amyloid deposition, skin hypertrophy, itching and dry skin
1. Macula and mossy skin amyloidosis Macula skin amyloidosis, MA) and lichenoidamyloidosis (lichenoidamyloidosis, LA) are the two most common types of primary localized cutaneous amyloidosis, Is confined to the skin, There is no report that they develop into systemic amyloidosis, Macula is more common in middle-aged women, Mainly located in the interscapular area of the back, It can also affect the trunk and limbs, The rash is brown or purple-brown pigmented spots, It is formed by polymerization of punctate pigment spots, It is reticulated or corrugated, Has diagnostic value, The rash is not itchy, Itchy people have keratinized papules and rough surface. Moss-like type is also called amyloid moss, More common in middle age, Both sexes can be affected, Most of the rashes were symmetrically distributed in front of the tibia of the lower leg (Fig. 1 ~ 3), Secondly, on the lateral side of the arm, waist, back and thighs, Start with brown spots the size of needles, After becoming pointed papules and gradually increasing, Flat bulge, Hemispherical, conical or polygonal, The diameter is about 2mm, Hard and brown, Brown or similar to normal skin, Smooth and shiny or with a little scale on the surface, Hyperkeratosis and roughness, There are black horny plugs at the top, After peeling, umbilical depression is left, Early rashes are scattered, In the future, it can be densely packed into pieces but often do not integrate with each other. Scattered brown papules can still be seen around the edge, The rash of the lower leg and upper back is characteristic in that it is arranged in rosary shape along the dermatoglyphics, Conscious itching is severe, Pruritus can precede the rash for 1 ~ 2 months. Long-term scratching can deepen the dermatoglyphics at the damaged area, or the rash fuses into flaky verrucous hyperplasia, and some are ichthyosis-like, eczema-like or nodular prurigo-like. The skin lesions often have pigmentation or hypopigmentation, and the disease progresses slowly. Once the rash occurs, it is difficult to subside, but it has no important impact on the health of the whole body.
2. Amyloidosis cutis dyschromica is common in men, which is clinically divided into prepubertal onset and adult onset. The rash is mainly distributed in limbs and develops slowly, with skin atrophy, telangiectasia, pigmentation, hypopigmentation and other skin heterochromia changes, which may be accompanied by blisters and palmoplantar keratosis.
3. Nodular cutaneous amyloidosis is rare, and its clinical manifestations have two subtypes: large nodules and atrophic nodules. The former is intradermal or subcutaneous nodules with normal skin color or brown skin on the surface; The skin on the surface of the latter nodule is atrophic and yellow-white, the rash can be single or multiple, the nodule tissue is very fragile, and mild trauma is easy to cause blisters and/or bleeding.
1. Typical rash, histopathological changes and special staining show that amyloid protein is the main basis for diagnosis of this disease. Positive Nomland test is helpful for diagnosis.
2. TCM syndrome differentiation
(1) Rheumatic accumulation type:
Main syndrome: Local skin is thick and rough, forming local mossy changes, repeated severe itching, thin or white greasy tongue coating, and slow or heavy pulse.
Syndrome differentiation: Rheumatism accumulates and skin is out of nourishment.
(2) Blood deficiency and wind dryness type:
Main syndrome: The rash is pale, rough and hypertrophic, and the complexion is pale. The tongue is pale and the pulse is heavy and thin.
Syndrome differentiation: blood deficiency, wind dryness and skin loss.
(3) Spleen deficiency and dampness stagnation type:
Main syndrome: dry skin, solid small papules on the extended side of both lower limbs, dense into pieces, showing moss-like changes, conscious itching, dry stool or diarrhea, heavy limbs, pale tongue, white and greasy tongue coating, and slow pulse condition.
Syndrome differentiation: spleen deficiency and dampness stagnation, skin loss.
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