Pityriasis rubra pilaris

Introduction

Introduction to hair red pityriasis Pityriasisrubrapilaris, also known as Pityriasispilaris, are characterized by scaly erythema on the skin, followed by clusters of small follicular papules, which are fused together like psoriasis patches, often accompanied by There is a palm keratosis. Red pityriasis is a chronic inflammatory skin disease. The etiology of this disease is unknown. The incidence of childhood may be related to autosomal dominant inheritance (hereditary type), mild symptoms, prolonged course of disease; adult patients may be associated with vitamin-binding protein deficiency, low plasma vitamin A or thyroid dysfunction. Related (acquired type), the incidence is faster, can develop into erythroderma. basic knowledge The proportion of illness: 0.01%. Susceptible people: no special people Mode of infection: non-infectious Complications: Acne

Cause

Hair red pity

Congenital inheritance (30%):

The familial type is autosomal dominant, often in childhood. According to Leitner, 64 cases (51%) of the 125 patients have a positive family history. In addition, it is found that there is a special acidity in the damaged skin. Keratin protein (K17), but not found in normal skin.

Acquired (30%):

The cause is still unclear. Some scholars divide the disease into two types: family type and acquired type. The latter can occur at any age, and both men and women can get sick.

Pathogenesis:

The pathogenesis is still unclear. Porter and other scholars found that the epidermal hyperplasia of the disease is excessive. The conversion time of 1AC-labeled methionine and glycine through the epidermis is shortened, the epidermal activity is excessive, and the marker index is increased, which is 3% to 27.3% of the average normal value. ±8.6%, the formation of hair red pimples epidermis is significantly faster than normal skin, but generally slower than or similar to psoriasis, and nail growth rate is lower than psoriasis, but faster than normal.

Some scholars have suggested that this disease is related to the thyroid dysfunction or dysfunction of the adrenal pituitary system affecting the metabolism of vitamin A in the blood, or related to the defects in the synthesis of retinoic acid-binding protein in the serum of patients.

In addition, liver, kidney dysfunction, tuberculosis, tonsillitis, colds, etc. can also induce the disease, but have not been confirmed.

Prevention

Red pityriasis prevention

The etiology of this disease is unclear. It is currently believed that its etiology may be related to the following factors: genetic factors, vitamin deficiency, keratosis, endocrine, etc., due to timely supplementation of vitamin A, timely work and rest, diet rules to prevent this disease.

Complication

Red pityriasis complication Complications

Patients usually have acne-like lesions such as cystic nodules and pustules, and the association of this disease with the tumor may be accidental.

Symptom

Red pityriasis symptoms of common hair Common symptoms Papular scaly spots, facial flushing, dry skin, secondary infection, erythema, scaly, cleft palate, appetite, withdrawal, sweat reduction

Beginning from the scalp and face, it is characterized by thick scalp scalp scales, flushing of the face, with more fine scaly, similar to dry seborrheic dermatitis, followed by the neck, torso, and extremities. Especially on the back of the first and second knuckles, wrist joints, elbow joints, large miliary skin, normal skin color, reddish or reddish brown hair follicle keratotic hard papules, multi-integrated tablets, looks like goose bumps, touch Stinging, the center of the pimples has small horny plugs, and there are bristles running through, the base is infiltrated with flushing, and the horny plugs are deep into the hair follicles, so it is not easy to remove. The lesions are characteristic and symmetrically distributed, rarely appearing in Face and face, continue to develop can become a patchy patch of varying size and clear boundaries.

Severe cases can affect the scalp and the whole body skin, diffuse erythroderma, often visible island-like normal skin, such lesions resemble psoriasis or lichen planus, but the edges are still visible isolated follicular papules, sometimes scratching New damage can be seen on the traces. The patient feels dry and nervous skin, prone to chapped, excessive keratosis of the palmar palpebral, hyperplasia or secondary erythroderma, fever, reduced sweating, loss of appetite, weight loss, secondary infection Such as systemic symptoms, summer sun exposure can often be aggravated.

Nail and hair are also often affected, nails are turbid and thick, and there are crepe on the surface, but there is no characteristic punctate depression in psoriasis, excessive keratinization under the nail, sparse hair, and hairy cleft palate, often accompanied by Grooved tongue, a small number of patients in the oral mucosa, such as hard palate, may have white line or spotted rash, individual with conjunctivitis, corneal opacity, or the formation of dendritic corneal ulcer.

Familial type is slow, the symptoms are mild, but often lifelong unhealed, and the acquired type is more acute, can be relieved periodically, and partially relieves or heals after 2 to 3 years.

It has recently been found that this disease can be associated with HIV infection. These patients usually have acne-like lesions such as cystic nodules and pustules. It has been reported that adolescent cases are accompanied by gamma globulin hypoplasia and rickets. Relevance may be accidental.

Examine

Red pityriasis check

Laboratory tests: Some patients found a decrease in plasma vitamin A, and some patients had eosinophilia.

Histopathological examination: the main lesions were diffuse hyperkeratosis in the epidermis, the hair follicles were a little keratinized, the follicular horns were formed, the granules and acanthosis were thick, the basal cells were liquefied and degenerated, the epidermis was short and thick, and the dermis was dilated. There are lymphocytes around the blood vessels and hair follicles, and most mast cells and plasma cells infiltrate.

Diagnosis

Diagnosis of hair red pityriasis

The disease has a special brown-red hair follicle keratotic papules, which occur in the back of the fingers, the neck side and the extremities of the extremities. The skin is thick and rough, with mild or moderate scaly, excessive palmar keratosis, and histopathological features. Diagnosis is generally not difficult.

Need to be differentiated from the following diseases.

1. Psoriasis with multiple layers of silvery white scales, stripping scales, a bit of bleeding, the rash is not consistent with the hair follicles, the palmar part is generally unchanged, the histology is very different, there is Munro micro in the psoriatic stratum corneum Abscess, keratosis is prominent, the epidermis plate on the nipple is thin, and the epidermis is slender.

2. The lichen planus rash is a purple or dark red shiny polygonal flat papule with wickham pattern, no follicular keratin plug, rarely involving the head and palmar.

3. Hair moss occurs in the extremities of the extremities, with a small cystic follicle, no inflammation, long-term existence, no fusion, and the finger back is not involved.

4. Seborrheic dermatitis lesions are more common in the fatty area, no follicular keratinous papules, and tend to exude.

5. Vitamin A deficiency lesions are keratinized hair follicle papules, but no inflammation, no erythroderma, no change in palmar, often accompanied by night blindness, dry eye and corneal softening, dark adaptation.

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