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[Disease name] Primary limited cutaneous amyloidosis

ÿEnglish nameÿprimary localized cutaneous amyloidosis

ÿabbreviationÿ

ÿAliasÿpsoriasis; primary localized cutaneous amyloidosis

ÿICD No.ÿL99.0*

ÿOverviewÿ

Amyloidosis refers to a homogeneous, unstructured amyloid with a specific reaction

(amyloid) is deposited in tissues or organs, resulting in different degrees of morphological changes and functional changes in the corresponding tissues and organs.

Disabled diseases. Amyloids are a group of protein and mucopolysaccharide complexes due to their

Similar chemical reactions (such as with iodine, etc.) are called amyloidosis, and actually have nothing to do with starch. precipitate

Powdery disease is a protein biochemical metabolic disorder, there are many different clinical types, classification methods

Not consistent. Simply can be divided into primary and secondary according to its etiology, and each according to the location of occurrence

and scope differences are divided into localized and systematic.

primary localized cutaneous amyloidosis

Refers to amyloidosis that originates locally in the skin without involvement of other systems and is not secondary to other skin disorders

sick. It is similar to the "skin psoriasis" recorded in traditional Chinese medicine literature; as recorded in "Golden Mirror of Medicine, Essentials of Surgical Heart Law":

"Pine skin ringworm, as strong as the skin of pine, with red and white spots connected together, itching from time to time."

ÿEpidemiologyÿ

The disease incidence is regional. People in the Middle East, Asia, and Central and South America are susceptible to this disease, and in Chinese

more common in.

ÿCausesÿ

The cause is unclear. Macular and lichenoid cutaneous amyloidosis may be associated with familial genetic factors,

It has been observed that the incidence of family history is as high as 28%, with autosomal dominant inheritance;

Natural factors in the area, such as high temperature and humidity, are also the cause of the disease. Long-term scratching and rubbing of the skin will cause the skin to increase.

Thick, hyperkeratotic, and abnormally pigmented, resulting in so-called friction melanosis, followed by

The amyloidosis that can occur is called frictional amyloidosis (friction amyloidosis).

ÿPathogenesisÿ

Macular cutaneous amyloidosis and lichenoid cutaneous amyloidosis are closely related and often coexist.

The former can develop into the latter after being stimulated by scratching, and the latter can also be transformed into the former after treatment.

The two are considered to be different stages of development of the same type. Regarding the formation of amyloid, it is currently believed that the
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The "keratin bodies" produced after the apoptosis of skin keratinocytes. Its morphology and colloid-like bodies of lichen planus

Similar, contains tonic fibrils, and immunofluorescence finds antikeratin around amyloid

Deposition of antikeratin auto antibodies. Cutaneous heterochromatic amyloidosis is a common

Chromosomal recessive inheritance. Sunlight UV exposure is an important predisposing factor, of which UVB is the most obvious, but also

There are reports of other spectra and even gamma rays inducing the disease. nodular cutaneous amyloidosis in amyloid

There was obvious clonal proliferation of plasma cells in the mass, and its essence was actually extramedullary plasmacytoma. local immersion

Infiltrate a large number of plasma cells to form plasma cell cachexia, secrete excess immunoglobulin and degenerate into amyloid

protein, which also induces reactive infiltration of T lymphocytes.

ÿClinical manifestationsÿ

1. Macular and lichenoid cutaneous amyloidosis

(macularamyloidosis, MA) and lichenoid amyloidosis (lichenoid amyloidosis,

LA) are the two most common types of primary limited cutaneous amyloidosis, confined to the skin, and neither has been seen yet.

Development of systemic amyloidosis has been reported. The macular type is more common in middle-aged and older women, mainly on the back and shoulders

The interscapular region may also involve the trunk and extremities. The rash is a brown or purplish-brown hyperpigmented patch consisting of punctate pigmented spots

Aggregated, in the form of reticulate or corrugated, with diagnostic value. Most rashes are not itchy, but those with itching have less keratinization

Papules and rough surfaces. Lichenoid type, also known as lichen amyloidosis, is more common in middle age and can affect both sexes.

The rash is distributed symmetrically on the front of the tibia of the lower leg (Figure 1-3), followed by the outer side of the arm, waist, back and thigh. open

At first it was a needle-sized brown spot, and then it became a pointed papule and gradually enlarged, flat, hemispherical, round

Conical or polygonal, about 2mm in diameter. Hard texture, brown, tan or similar to normal skin, smooth

Shiny or slightly scaly on the surface, hyperkeratotic and rough, with black keratin plugs at the tip, leaving a navel after peeling

type depression. In the early stage, the rash is scattered, and later it can be densely integrated but often not merged with each other, and the surrounding edges are still scattered

Brown papules in. The rash on the lower legs and upper back is characteristically rosary along the striae. conscious itching

Severe, itching may precede the rash for 1 to 2 months, long-term scratching can deepen the skin lines on the damaged area, or the rashes may interact with each other.

Fusion into flaky verrucous hyperplasia. There are also ichthyosis-like, eczema-like or nodular prurigo-like. skin lesions

Often there is hyperpigmentation or hypopigmentation. The disease progresses slowly, and once the rash occurs, it is difficult to subside, but it can affect the whole body.

No major health effects.

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2. Amyloidosis cutis dyschromica is common in men

Sex, clinically divided into two categories: prepubertal onset and adult onset. The rash is mainly distributed on the extremities and develops slowly

slow. There are skin atrophy, telangiectasia, hyperpigmentation, hypopigmentation and other changes in skin heterochromia.

May be accompanied by blisters and palmoplantar keratosis.

3. Nodular cutaneous amyloidosis (nodular cutaneous amyloidosis) is relatively rare. Pro

There are two subtypes of bed manifestations: nodular enlargement and nodular atrophy. The former manifests as intradermal or subcutaneous nodules,

The surface is covered with normal skin or brownish-yellow skin; the latter nodules have atrophy and yellowish-white skin on the surface. rash can

Single or multiple, the nodular tissue is very fragile, and mild trauma can easily cause blisters and/or bleeding.

ÿcomplicationÿ

Currently there are no related content description.

ÿLaboratory examinationÿ

There may be increased erythrocyte sedimentation rate and abnormal globulin such as increased alpha or gamma globulin. The Nomland test is to put

1.5% Congo red solution was injected into the skin of suspicious skin lesions, and only red residue remained at the amyloid after 24 to 48 hours.

Color, the positive rate of this disease with skin microscopy can reach 80%.

ÿOther auxiliary inspectionÿ

Histopathology: macular and lichenoid cutaneous amyloidosis with deposits confined to the dermal papilla

Hemispherical, conical or clump-shaped, ranging in size, with fissures between the epidermis and the epidermis. HE staining showed eosin
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Qualitative, with a small amount of blue-stained cell debris in between. Epidermal hyperkeratosis, granular and spinous cell layers

Irregular thickening, the epidermis above the amyloid can shrink. liquefaction degeneration of basal cells, especially in the plaque type

protrude. Cutaneous heterochromia-like amyloidosis with perivascular amyloid deposits and visible epidermal irregularities

The hyperplasia, epidermal process lengthening. Perifollicular amyloid deposits are sheathed. nodular cutaneous amyloidosis

The epidermis of the disease atrophies and thins, and the epidermis disappears. Subepidermal bulky amyloid extends from the dermis to the epidermis

Down. Vascular walls can be thickened by amyloid deposition, and sweat glands and surrounding adipocytes can also be involved.

The skin is accompanied by chronic inflammatory cell infiltration. Stain with methyl violet or crystal violet, amyloid metachromatic

Can be dyed bright purple-red; Congo red staining is green and birefringent under polarized light microscopy;

PAS staining was positive for amyloid-resistant amylase. Immunofluorescence is visible around the amyloid

Deposition of immunoglobulins, now confirmed as antikeratin autoantibodies

autoantibody, AK auto Ab). Disulfide bands on DACM staining also indicate amyloid

Derived from keratin.

ÿdiagnosisÿ

1. Typical rash, histopathological changes and special staining show that amyloid is the main diagnosis of this disease.

to be based on. A positive Nomland test helps confirm the diagnosis.

2. TCM syndrome differentiation

(1) Rheumatism accumulation type:

Main symptoms: local skin hypertrophy and roughness, localized lichen-like degeneration, recurrent severe itching, thin tongue coating or

White greasy, slow or slow pulse.

Diagnosis: rheumatism accumulation, skin dystrophy.

(2) Blood deficiency and wind dryness:

The main symptoms: the rash is light in color, rough and hypertrophic, and the complexion is not gorgeous. Pale tongue, thin pulse.

Diagnosis: blood deficiency and wind dryness, skin dystrophy.

(3) Spleen deficiency and dampness stagnation type:

Main symptoms: dry skin, firm small papules on the extensor sides of both lower extremities, densely packed, lichen-like changes,

Consciously severe itching, dry or loose stools, heavy and weak limbs, pale tongue, white and greasy tongue coating, slow pulse.

Syndrome differentiation: spleen deficiency and dampness stagnation, skin dystrophy.

ÿDifferential diagnosisÿ

The disease should be associated with neurodermatitis, prurigo nodularis, hypertrophic lichen planus, colloid miliary, lipid

Identification of proteinosis, etc.

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ÿtreatÿ

1. There is no satisfactory treatment method yet. Patients often suffer from severe itching, and attention should be paid to avoid scratching and rubbing.

rub, give oral antihistamines, intravenous procaine, topical corticosteroid injection or external

use. Encapsulation therapy can improve efficacy and effectively avoid physical irritation to the skin. DMSO alone or with cortex

Mixed topical steroid hormones have certain curative effects, and tretinoin drugs, especially the second-generation acitretin (retinoic acid)

Etretinate acid) has also received good results for this disease. You can also try salicylic acid ointment, turpentine oil

cream, fluorouracil ointment, etc.

2. Traditional Chinese Medicine

(1) Rheumatism accumulation type:

Governing Law: Dispel wind and dampness, nourish blood and soothe the skin.

Selection: 6g whole worm, 6g soap thorn, 10g windproof, 10g sophora flavescens, 30g white fresh skin, 30g tribulus terrestris,

Angelica 10g, Salvia miltiorrhiza 15g, Chixueteng 30g, Shouwuteng 30g, Chuanxiong 10g.

(2) Blood deficiency and wind dryness:

Governing Law: Nourishing and moisturizing, activating blood and softening firmness.

Selection: Shouwu Teng 30g, Angelica 10g, Chuanxiong 10g, Safflower 10g, Chibaishao 10g, Baixian

Skin 30g, Sophora flavescens 15g, Tribulus terrestris 30g, Prunella vulgaris 15g, Silkworm 10g, Salvia miltiorrhiza 15g, Fangfeng 10g.

(3) Spleen deficiency and dampness stagnation type:

Governing Law: invigorating the spleen and removing dampness, nourishing the blood and moisturizing the skin.

Formula selection: Atractylodes 10g, Poria 10g, Citrus aurantium 10g, Magnolia 10g, Sophora flavescens 15g, White fresh skin 30g,

Plantago 15g, Alisma 15g, Shouwu Teng 30g, Tribulus terrestris 30g, Angelica 10g, Chuanxiong 10g.

Single prescription and single drug can be used Huoxue Xiaoyan Pill, Rhubarb Zhechong Pill, etc.

ÿPrognosisÿ

1. Macular and lichenoid cutaneous amyloidosis progresses slowly, and once the rash occurs, it is difficult to subside,

But it has no important effect on general health.

2. Heterochromic amyloidosis of the skin, which develops slowly. Skin atrophy, telangiectasia, pigmentation

Changes in skin heterochromia such as hyperpigmentation and hypopigmentation

ÿpreventionÿ

Currently there are no related content description.