Medical Subjects: Skin

What Is Porokeratosis

What disease is porokeratosis?

Porokeratosis (PK) is a chronic hereditary skin disease with autosomal dominant inheritance. It is characterized by the presence of one or more atrophic macula or patches, with a distinct hyperkeratotic ridge-like eminence at the edge (called an angular template).

Treatment includes surgery and topical medications, or oral treatment with vitamin A derivatives may be considered. Various treatments have limited efficacy and cannot prevent recurrence. Porokeratosis has the possibility of malignant transformation. Once diagnosed, it should be closely followed up and regularly reviewed.

Is porokeratosis common?

Porokeratosis is rare and the exact incidence is unknown.

Is porokeratosis easy to malignant change?

Studies have reported that the malignant transformation rate is 7%–11%.

What are the types of porokeratosis?

The most common variants of porokeratosis include:

  • Disseminated superficial actinic porokeratosis (DSAP );

  • Disseminated superficial porokeratosis (DSP );

  • Mibelli porokeratosis (OMIM );

  • Linear porokeratosis;

  • Palmoplantar disseminated porokeratosis (PPPD );

  • Punctate porokeratosis (sometimes considered a variant of PPPD).


What Is The Cause Of Porokeratosis

What causes porokeratosis?

Most porokeratosis is hereditary disease in the form of autosomal dominant inheritance; Some are sporadic cases with no family history, and genetic factors, sun exposure, and the immunosuppressed state of the body may all be involved in the occurrence of the disease.

Is porokeratosis contagious? How is it contagious?

It's not contagious.

Does porokeratosis meet heredity? How?

Most of them are inherited, with autosomal dominant inheritance.


What Symptom Does Porokeratosis Have

What are the common clinical manifestations of porokeratosis?

Porokeratosis generally has no obvious symptoms but pruritus, mainly skin lesions. According to clinical classification, skin lesions vary in form:

  • Disseminated superficial actinic porokeratosis (DSAP):

  • It most commonly occurs between the ages of 30 and 40 years old, with autosomal dominant inheritance, and is related to sunlight exposure.

  • Features of skin lesions: They were symmetrically distributed at the light exposure sites, and there were several to hundreds of annular skin lesions with the diameter of 1 cm, which could fuse into multiple rings and gradually disappeared in winter;

  • 1/3 Conscious itching.

  • Mibelli type porokeratosis:

  • Is the second most common type of porokeratosis; It occurs in multiple childhood, in more males than females, and is usually associated with immunosuppression.

  • Small, usually asymptomatic or slightly pruritic papules at first and slowly enlarging over several years. May grow more rapidly in an immunosuppressed state. The formed skin lesions are usually several centimeters in diameter, and a few can grow to 10–20 cm. These particularly large lesions are known as "giant porokeratosis".

  • Skin lesions can occur in any part of the body, including the palm, plantar, genital or mucosal.

  • Disseminated superficial porokeratosis (DSP);

  • The onset of the disease was early (more than 5–10 years old), and it had nothing to do with the light.

  • Characteristics of skin lesions: erythema or pigmented keratopapules on the trunk, genital organs and palms and soles, with central atrophy and gradually enlarged periphery showing superficial ring;

  • 1/3 Conscious itching;

  • Linear porokeratosis:

  • Rarely, at birth or in childhood;

  • Skin lesions were characterized by eccentrically dilated hyperkeratotic papules, which were the most malignant to squamous cell carcinoma;

  • Punctate porokeratosis:

  • Most adults suffer from the disease;

  • Features of skin lesions: keratotic spots limited to the palmoplantar region, with slightly elevated edge and soft touch;

  • Palmoplantar disseminated porokeratosis;

  • Onset in adolescence or adulthood;

  • Features of skin lesions: First appeared in the palmoplantar region, and gradually spread to the whole body; bilaterally symmetrically distributed brownish-red keratinizing papules with a diameter of 1–2 mm.

How does porokeratosis develop?

And has a certain malignant transformation rate, and the course of disease varies.


How To Check For Porokeratosis.

What is the diagnostic basis of porokeratosis?

When doctors diagnose porokeratosis, they mainly refer to family history, skin lesions and pathological manifestations.

What tests should be done for porokeratosis?

Generally, patients will be asked to do skin histopathological examination, and the keratosis bulge will be taken for examination, showing the characteristic changes: epidermal dyskeratosis, which is a "model of corns".

Which diseases are porokeratosis easily confused with?

Because skin lesions lack specificity, porokeratosis may be confused with verruca vulgaris, lichen planus and other diseases. Doctors need to differentiate by skin histopathology.


How To Prevent Porokeratosis.

Can porokeratosis be prevented?

Because it is a hereditary disease, it can not be prevented, so skin care and hygiene should be paid attention to in life to prevent secondary infection. In addition, family members need genetic counseling before marriage, and close relatives are prohibited from getting married.


How To Treat Porokeratosis.

Which department should I see for porokeratosis?

Dermatology department.

What are the treatments for porokeratosis?

Most cases of porokeratosis are benign and do not require treatment. However, when skin damage is symptomatic or unsightly, patients may wish to receive treatment. The treatment options of porokeratosis include: external drug treatment, destructive treatment, surgical resection and systemic vitamin A acid treatment;

  • Cryotherapy, electro-drying, curettage, or surgical resection are recommended for patients who have only a few minor skin lesions, require rapid treatment of symptoms or improve their aesthetic appearance, and can receive treatment-related pigmentation changes or scars.
  • For patients with large or large number of skin lesions, or patients who are worried that the above intervention scheme may produce scars, it is recommended to use external medication. It can be used externally with 5- fluorouracil (5-FU) or imiquimod or to treat localized skin lesions. Topical vitamin A acid and vitamin D analogues are less irritating, but may require longer treatment.
  • Other methods to destroy skin lesions include skin grinding, laser treatment and photodynamic therapy.
  • Generally, only severe cases are given systemic vitamin A acids. It is very likely to recur after stopping treatment.

Can porokeratosis be completely cured?

No, all kinds of treatment methods have limited curative effect and cannot prevent recurrence.


What Should Porokeratosis Patients Pay Attention To In Their Lives?

What should patients with porokeratosis pay attention to in their diet?

Because the etiology of porokeratosis is hereditary, diet and exercise have no effect on porokeratosis. But adhering to a healthy lifestyle is good for anyone's health.

What should porokeratosis patients pay attention to in their lives?

Pay attention to skin care and hygiene to prevent secondary infection. Eat more fresh vegetables and fruits.

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