Porokeratosis
Introduction
Introduction to poroke keratosis Porokeratosis is a special keratosis characterized by the appearance of a corneallamella in histology. The conical layer is a thin cylindrical tightly arranged parakeratotic cell. Distributed throughout the stratum corneum. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: Mildew
Cause
Causes of porokeratosis
(1) Causes of the disease
The etiology is unknown, autosomal dominant inheritance is seen in PM, PP-PD, DSp and DSAP, LP is found in single-sex twins, clinical manifestations and histopathological similarities, and different clinical types of the same patient or different patients in the same family, suggesting Different clinical manifestations have a common genetic background.
(two) pathogenesis
The classification of this disease is based on its pathological features - conical lamellar and related clinical manifestations, but it should be noted that conical lamellar layers are also found in many diseases unrelated to this disease, such as inflammation, hyperplasia and neoform formation, Reed and Leone It is suggested that the epidermal cell mutant clones of this cause can be expanded along the periphery to form a conical layer at the boundary between the clonal population and normal keratinocytes. Abnormal cloning may be hereditary, and other causes lead to clinical symptoms. One of the causes is ultraviolet light, which is related to DSAP. Because of immunosuppression, it is common in organ transplants and HIV-infected patients. It is associated with DSAP and DSP. Severe immunosuppression is reduced by immune surveillance, which is beneficial to the appearance of abnormal keratinocyte clones, which eventually leads to malignant transformation.
Prevention
Sweat keratosis prevention
(1) Pay attention to skin care and hygiene to prevent secondary infections.
(2) Eat more fresh vegetables and fruits.
(3) Avoid using irritating external medicine.
(4) Prohibiting the marriage of close relatives.
Complication
Complications of porokeratosis Complications
The disease is mainly characterized by excessive keratinization of the skin, so the integrity of the skin may be destroyed due to excessive keratinization, and it is easy to combine bacteria and fungal infections, which should cause clinical attention, often seen in low constitution, or long-term use of immunosuppressants. Patients, once infected, should actively carry out anti-bacterial and anti-fungal treatment to prevent the spread of infection and invade the blood system to cause sepsis.
Symptom
Symptoms of porokeratosis common symptoms papules itching pores block severe pain
The basic damage is a well-defined parakeratosis with a centrally atrophied ring, line or spot-like shape. There are five clinical types (Table 1): the classic Mibelli type (PM), the disseminated superficial type (DSP) and the broadcast. Dispersive actinic superficial phenotype (DSAP), palmoplantar disseminated (PPPD), linear (LP) and spotted (often associated with PM and LP).
1.Mibelli type: the damage begins with small, brown, keratinized papules, gradually expanding to form regular and annular plaques with boundary clearing, high keratinized borders, often above 1 mm, including linear grooves, The lesions manifested as hyperkeratosis and warts, the center is often atrophic, hairless and lack of sweating, accompanied by hyperpigmentation or loss, several lesions ranging in diameter from a few millimeters to several centimeters, with limbs, hips and The external genitalia are common, but also found in the face and mouth, which can affect the palmar.
This type begins in childhood, and the damage slowly expands after several years, often asymmetry. This type is characteristic, limited, mostly unilateral, with large damage, clear boundaries and diagnostic grooves. More common in men.
2. Disseminated superficial and disseminated actinic superficial: DSP is more common, mainly involving the limbs, bilateral symmetry, damage is also seen in the armpit, groin, perineum, palmar and mucosa, about In 50% of cases, the damage is seen in the exposed area (DSAP), which is aggravated in summer, the damage is small, superficial, single, clustered, and even hundreds of visible on the extremities of the extremities, the damage begins with small keratosis Pimples, mostly with central fovea, 1 to 3 mm in diameter, more dry, may have erythema, pigmentation or normal skin tone, gradually increasing superficial ring lesions, with mild central atrophy, with intermittent apical grooves The dykes of the dikes, DSP and DSAP are relatively common, often occurring at the age of 30 to 40 years old, and have developed slowly over the years. Although women are more common, the proportion of males and females in the family is half, and the ultraviolet exposure is too long, such as patients with psoriasis. Photochemotherapy and phototherapy can cause DSAP to aggravate or prolong the course of the disease. DSAP is found in areas with strong sunshine and is rare in blacks.
3. Dispensing type of palmar: a small superficial relatively single damage, the boundary is clear, the surrounding is a ridge-like bulge, the height is no more than 1mm, the volarization of the palmar part is more extensive, and the longitudinal groove along the ridge is more obvious. The damage is first seen in the palm of the hand, and then spread to the limbs, trunk and other parts, including non-exposed parts, the number is large, itching and tingling, the mucosal damage is small, ring-shaped or scorpion-shaped, milky white and asymptomatic, The number is more than twice as high as that of women, and it usually begins in adolescence and early adulthood.
4. Line type: can be expressed as unilateral, linear and extensively involved, similar to linear scorpion epidermis, with the same damage as the Mibelli type, including mossy papules, small ring lesions, hyperkeratotic plaques with central atrophy and features The surrounding ridges are bulging, distributed in groups, lined along the limbs, and the distal end is easily affected. It can be distributed in the trunk, which can affect the unilateral limbs. It is also seen on the ipsilateral face and trunk, starting from infants or children. The genetic characteristics are not yet clear, and may be associated with other clinical types, with reports of deterioration.
5. Spot type: often associated with Mibelli type or line type, is a large number of small, discontinuous spots seen in the palmar sac, excessive keratinization, accompanied by fine high edges, damage can be arranged in a line, also Can be integrated into plaque-like, clinical attention must be paid to the identification of spotted palmoplantar keratosis.
Examine
Examination of porokeratosis
Histopathology: The center of the ring lesion showed mild stratum corneum thickening, normal granule layer, atrophy of the spinous layer, high keratinization at the sweat tube mouth and the bank edge, the granule disappeared, the spinous layer thickened, and the dermis layer had Lymphocyte-based inflammatory infiltration, collagen fibers and appendages atrophy, it is best to cut the ridge-like bulge edge with a central groove for pathological examination, the central sulcus shows a keratin-filled epidermal depression, which has an angle The incomplete column, the so-called conical plate layer, has abnormal epidermal cells at the base of the keratinized column, which is the most characteristic pathological change of all types of this disease.
Diagnosis
Diagnosis and differentiation of porokeratosis
diagnosis
According to the clinical manifestations, the general diagnosis is not difficult. If necessary, biopsy can be performed to confirm that the pathological image of the disease has diagnostic value.
Differential diagnosis
Differential diagnosis sometimes needs to be distinguished from lichen planus, sputum, sputum sputum, keratosis, epidermal carcinoma, skin carcinoma in situ, and penetrating elastic fiber disease.
1. Annular lichen planus: The purple-red flat papules are arranged in a ring shape; the edges are smooth and not rough; subjective itching, pathological changes can be identified.
2. sputum sputum: skin lesions are scorpion-like, linearly arranged, there are at birth, pathological changes are easy to identify.
