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Xeroderma pigmentosum or XP, is a very rare inherited disease that causes extreme sensitivity to the sun’s ultraviolet rays.


Xeroderma pigmentosum or XP, is a very rare inherited disease that causes extreme sensitivity to the sun’s ultraviolet rays. Unless patients with XP are protected from sunlight, their skin and eyes may be severely damaged. This damage may lead to cancers of the skin and eyes. XP has been identified in people in every ethnic group all over the world.

Many persons with XP will get an unusually severe sunburn after a short sun exposure. The sunburn will last much longer than expected, perhaps for several weeks. This type of sunburn will usually occur during a child’s first sun exposure, and it may be a clue to the diagnosis of XP. However, some people with XP will not get a sunburn more easily than others, and the disease will be undetected until unusual skin changes appear over time.

Abnormalities that occur in individuals with XPA, but are unlikely to be related to UV damage, include neurological abnormalities and internal cancers. At least 25 to 30% of individuals with XPA have progressive neurological issues that may include hearing loss, difficulty swallowing and talking, movement problems, seizures, and intellectual disability. The risk for internal cancers is thought to be linked to environmental carcinogens, like cigarette smoke and other, potentially uncontrollable, exposures.


Dermatologist Moriz Kaposi originally reported XP in 1874 after observing wrinkling, checkered pigmentation, tiny dilatations of the arteries, skin contraction, and the development of skin-based malignancies in four individuals with thin, dry skin. Following reports, the disease’s spectrum was enlarged to encompass neurologic problems, as well as a severe variant with dwarfism, gonadal hypoplasia, and learning disabilities, in addition to the classic symptoms of XP. The disease pathogenesis was identified as congenital excessive sensitization of the skin to ultraviolet radiation of the sun as early as 1926, with clear value in sun exposure prevention methods.


XP has been found in all continents and across all racial groups. Consistent with autosomal recessive inheritance, males and females are similarly affected. Estimates made in the 1970s suggested an incidence in the USA of 1 in 250,000 and in Japan of 1 in 20,000. A more recent survey in Western Europe suggests approximately 2.3 per million live births. Anecdotally, the incidence in North Africa and the Middle East, where there is a high level of consanguinity, is substantially higher.

Symptoms of Xeroderma pigmentosum

Symptoms usually appear by the time a child is 2 years old.

Skin symptoms include:

Sunburn that does not heal after just a little bit of sun exposure

Blistering after just a little bit of sun exposure

Spider-like blood vessels under the skin

Patches of discolored skin that get worse, resembling severe aging

Crusting of the skin

Scaling of the skin

Oozing raw skin surface

Discomfort when being in bright light (photophobia)

Skin cancer at a very young age (including melanoma, basal cell carcinoma, squamous cell carcinoma)

Eye symptoms include:

Dry eye

Clouding of the cornea

Ulcers of the cornea

Swelling or inflammation of the eyelids

Cancer of eyelids, cornea or sclera

Nervous system (neurologic) symptoms, which develop in some children, include:

Intellectual disability

Delayed growth

Loss of hearing

Muscle weakness of the legs and arms

Xeroderma pigmentosum causes

Xeroderma pigmentosum is an autosomally recessive inherited disease, which means that a faulty xeroderma pigmentosum gene comes from each parent. Carriers of the xeroderma pigmentosum trait have one xeroderma pigmentosum gene and one normal gene and do not show signs or symptoms of the disease.

The signs and symptoms of xeroderma pigmentosum are a result of an impaired nucleotide excision repair (NER) system. Two types of NER have been identified:

Genome (GG)-NER

Transcription-coupled (TC)-NER.

At least seven different gene abnormalities or complementation groups have been described in different families (XPA to XPG) resulting in varying disease severity.

XPA and XPC are relatively common

XPE is fairly rare

XPG is severe

XPF is mild.

In addition to the genetic abnormality, the immunosuppressive effects of exposure to ultraviolet radiation (UV) contribute to the disease, for example by depleting Langerhans cells from the epidermis.

Xeroderma pigmentosum

Types of Xeroderma pigmentosum

There are 8 different genetic types of Xeroderma Pigmentosum depending on the affected gene: XPA, XPB (or ERCC3), XPC, XPD (or ERCC2), XPE (or DDB2), XPF (or ERCC4), XPG (ERCC5) and XPV (or POLH)10, each one of them affecting different mechanisms of DNA repair (making them more severe or not) in the protection of the aggressions caused by UV light.XP typeAffected gene / locusClinical featuresXP-AXPA / 9q22.3It represents 25% of the patients with XP.

It is the most serious form of the disease because its DNA repair capacity is more diminished and its sensitivity to UV light highly increased. Patients present a diverse range of symptoms, including the development of numerous skin cancers at an early age and serious neurological abnormalities.XP-BXPB (ERCC3) / 2q21It is the least frequent of the XP types. It presents increased sensitivity to UV light.

Patients may develop numerous skin cancers at an early age, some mild neurological abnormalities and they can present characteristics of Cockayne syndrome.XP-CXPC / 3p25It represents 25% of the patients with XP.

It is considered the classical form of XP, the most frequent form in the Caucasian population. It is the disease variant with the highest capability to repair DNA even it shows increased sensitivity to UV exposure.

Patients may develop severely atypical and dense lentigines at exposed areas as well as ocular anomalies but no neurological abnormalities.XP-DXPD (ERCC2) / 19q13.2-q13.3It represents 15% of the patients with XP. It presents increased sensitivity to UV light. Patients with this variant may develop numerous skin cancers at an early age.

About neurological defects, they may present in some cases severe neurological defects with progressive degeneration such as sensorineural deafness, ataxia and mental retardation.XP-EXPE (DDB2) / 11p11-p12 ; 11q12-q13Low frequency. A less aggressive form of XP, it is limited to skin problems. Patients present the development of cutaneous cancers at a later age and they do not present neurological symptoms.XP-FXPF (ERCC4) / 16p13.3Low frequency.

A less aggressive form of XP, most of the cases in the Japanese population. It presents the development of cutaneous cancers at a later age and it does not present neurological nor ocular symptoms.XP-GXPG (ERCC5) / 13q32-q33Low frequency but aggressive. Increased sensitivity to UV light. Development of numerous skin cancers at an early age and it presents neurological abnormalities in some cases.XP-variantXPV (POLH) / 6p21.1

It represents 20% of the patients and it is very similar to XP-C. Increased sensitivity to UV exposure but less photosensitivity than other forms of XP. Higher risk of cutaneous cancers development after 30 years old and it does not present neurological symptoms nor ocular problems.

Risk factors

XP is a hereditary disease. The only people at risk are those who have a parent, or parents, who either have the disease, or are carriers of the disease.

XP is more prevalent in certain isolated, geographic areas. This may, in part, be caused by consanguinity. This means that both parents are blood relatives, such as cousins. If parents share a genetic background, their chances of passing XP on to their children increase.

The most common complications of XP are skin cancer, neurologic abnormalities, and eye abnormalities.

Malignancies are also common. Repeated surgeries to remove tumors can result in disfigurement, but can be avoided by taking precautions against sun exposure.

Those with XP need to take extreme measures to protect every surface of the body from UV light at all times. These precautions include:

wearing protective clothing, such as long sleeve tops, pants, and wide-brimmed hats

applying broad spectrum sunscreen

using UV-absorbing glasses with shields

Xeroderma pigmentosum Complications

There are many complications of xeroderma pigmentosum, which this activity discussed previously.

The median age for the development of their first non-melanoma skin cancer is about 9. Patients may develop dozens to hundreds of non-melanoma skin cancers per year.

Patients with xeroderma pigmentosum have a more than 10000 fold risk of developing non-melanoma skin cancer compared to the general population.

The median age for the development of their first malignant melanoma is about 22.

Patients with XP have a more than 2000 fold risk of developing malignant melanoma compared to the general population.

The median age of death in patients with XP without neurodegeneration is about 37 years old. The median age of death in patients with XP with neurodegeneration is younger at about 29 years old.

While the most common cause of death amongst patients with XP is metastatic malignant melanoma or invasive squamous cell carcinoma, the second most common cause is due to neurodegeneration.


Clinical history and physical examination

In most cases, the initial clinical diagnosis can be made on the basis of extreme sensitivity to sun exposure, or by the appearance of freckles on the face at an unusually early age. A history of consanguinity between the parents may be an additional clue.

Confirmatory Tests for Xeroderma Pigmentosum

Tests to detect defective DNA repair

When there is a high index of suspicion, a piece of skin is removed (skin biopsy) using a local anesthetic and sent for a confirmatory test. These tests detect defective DNA repair and are offered in several countries.

A type of cells found in the skin termed fibroblasts that are hypersensitive when exposed to UV radiation can be studied with the help of a microscope to see how effective the DNA repair mechanism is. A blood sample can also be used to perform this test.


A skin biopsy may show findings of hyperkeratosis and increased pigmentation along with other findings which may confirm the diagnosis.

Genetic Testing

Molecular testing and gene sequencing can be done to identify the specific defect.

Prenatal diagnosis

Prenatal diagnosis may be done if there is already an affected sibling or one of the parents is affected by the condition. The DNA repair tests can be performed on amniotic cells or chorionic villi derived cells. Molecular analysis can also be done in the presence of a known mutation.


Strict lifetime sunlight avoidance is key to reducing the risk of associated sequalae.

Individuals with XP need to take precautions to minimise any sun exposure, including:

Wearing SPF 50+ and sun-protective clothing such as long sleeves, wide-brimmed hat and wraparound sunglasses.

Ensuring access to SPF 50+ at all times.

Avoiding outdoor occupations or recreational activities.

Avoiding sun exposure through car or building windows, either through drawing curtains and/or applying UV protective tinting to windows.

Using lightbulbs which emit lower levels of UV.

Avoiding going outdoors during the middle of the day, and planning travel and activities in periods of low sunlight.

Awareness of your own skin is very important if you suffer from XP, to detect skin cancers early.

It is also worse in people who smoke, and therefore smoking cessation is crucial.

Individuals with XP must also take a vitamin D supplement, as they will be avoiding the sun.


Experts recommend genetic counseling for persons with a family history of xeroderma pigmentosum who wish to have children.

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Police inspector resign alleges jihadist plot against indigenous land.

Nigerian Inspector of Police attached to IGP Special Task Force on Petroleum and Illegal Bunkering Unit, Calabar Sector, Ekemezie Hycient has formally tendered his resignation from the force.

The Inspector with force number 318093 in a letter addressed to the Force headquarters, Louis Edet House, Shehu Shagari Way, Area 11 Garki, Abuja said he quite to serve his fatherland because the Nigeria State has been so polarized that foreign machinery and terrorists have surrounded the whole country, killing the indigenous owners of the land in the name of their Jihad especially the Indigenous People of Biafra, IPOB.

Ekemezie alleged that there’s the systematic extermination of the indigenous population that makes up the country called Nigeria with the help of the Nigerian Government.

Adding that, Mazi Nnamdi Kanu is undergoing political persecution because he spoke and stood against this shenanigan of the present Government in the name of Islamizing the whole country and handing over the indigenous people’s land to the Fulanis.

The officer who joined the Nigeria Police force on 1 July 2007, with fifteen years of experience in the force accused the Nigerian security forces of killing innocent Nigerians, leaving the terrorists untouched.

The Nigeria security forces go about killing innocent people leaving the Fulanis that are killing people everywhere, I can no longer continue to serve the Government that cannot guarantee the safety of its citizens.

I call on the Federal Government of Nigeria to free Mazi Nnamdi Kanu for he has committed no offense known to law because self-determination is not a crime, and a call for referendum is not a call for war.

It is also a democratic process just like an election is. Hence, this resignation. Thanks for your consideration, he wrote.

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Terrorist treathen to kidnap Mr. President.

Aisha Yesufu, a human rights activist has reacted to the threat by terrorists to abduct President Muhammadu Buhari.

Yesufu described Buhari as an absolute disgrace.

He wondered why there was cowardly silence over the threat to kidnap the President.

In a tweet, the activist lamented that the terrorists were not under fire after threatening to kidnap Buhari.

Yesufu tweeted: Muhammadu Buhari @MBuhari is an absolute disgrace! Terrorists dared to say they will abduct the Commander-in-Chief of the Federal Republic of Nigeria and there is cowardly silence everywhere.

The terrorists are not currently under fire. Kai! What an utter waste of existence!”

DAILY POST had earlier reported that the terrorists made the threat in a fresh video where they were captured flogging some of their captives, especially the male victims.

The victims were part of the 60 passengers abducted from the Abuja-Kaduna bound train on March 28, 2022.

Since the kidnapping, some of those kidnapped have been released, while others were still being held captive by the terrorists.

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Court ordered DSS and other security agency to pay damages to Mazi Nnamdi Kanu Lawyer.

Justice Hyeladzira Nganjiwa of a Federal High Court in Anambra  has ordered the Department of State Services and other security agencies to pay N102 million damages to Ifeanyi Ejiofor,  the lawyer to the detained leader of the Indigenous People of Biafra, Nnamdi Kanu, for invading his house.

The court said the invasion of his house at Oraifite, Anambra State,  on June 6, 2021 by security agents amounted to a violation of his fundamental rights.

The court also went ahead to award him N5 million being the cost of his Toyota Camry car which was burnt by  security agencies.

Ejiofor had in a fundamental rights enforcement suit with No: FHC/AWK/CS/56/2021, dragged the government to court to challenge the invasion of his ancestral home in Oraifite, in the state on June 6, 2021, alleging that an aide of his was killed during the exercise.

The eight defendants in the suit were the Nigeria Police Force; the Inspector General of Police; the State Security Service; the Director-General, State Security Service; the Nigerian Security And Civil Defence Corps; the Nigerian Army; the Chief of Army Staff and Chukwuka  Ofoegbu.

Delivering judgment, Justice Nganjiwa said the brutal invasion by the security agents, was oppressive being a gross violation of the rights to life, dignity of human person, fair hearing, right to private and family life of the plaintiff.

He said it was illegal, oppressive and unlawful, to take away and burn Ejiofor’s Toyota Car with registration number: YAB 60 CB.

He said it was wrong for them to take away the dead body of his domestic staff, Samuel Okoro, and other vital documents and valuables seized from his house by government agents.

The court further made an order restraining the defendants or their agents from further harassing him, threatening, and/or taking further steps in an attempt to terminate his life and/or destroying the properties of the plaintiff.

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Kanu to be released unconditional.

The UN Human Rights Council Working Group on Arbitrary Detention has brought charges against the governments of Nigeria and Kenya for Mazi Nnamdi Kanu’s wrongful arrest, extraordinary rendition, torture, and ongoing detention. Kanu is the leader of the Indigenous People of Biafra (IPOB).

The UN subsequently demanded that Nigeria’s government immediately free Kanu unconditionally and make restitution for the wilful violation of his fundamental human rights.

The inquiry and prosecution of the government officials who tortured the IPOB Leader were also supported by this statement.

After conveying its conclusions about Kanu’s case, the UN panel further urged Nigeria to report back on the steps taken to implement all of its recommendations within six months.

It sent Kanu’s case to the Special Rapporteur on torture and other forms of cruel, inhuman, or degrading treatment or punishment for further investigation.

The UN Working Group also pledged to take additional steps to ensure that the recommendations are followed, pointing out that both Kenya and Nigeria have signed the Convention and should follow suit.

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Nigeria activist Aisha Yesufu react to the killing of alleged bike thief’s.

Aisha Yesufu has taken out her time to condemn the burning of two individuals who allegedly stole a bike, asking to know the difference between terrorists and those who killed and burnt these two individuals.

On several occasions, innocent Nigerians have lost their lives due to jungle justice, in different parts of the country. While reacting to the sad incident, Aisha Yesufu described the action of these individuals as barbaric, stating it clearly that those who carried out this dastardly act are also terrorists.

She made this tweet while replying a twitter user who claimed that two guys were set ablaze at Akabo under bridge, for stealing a bike.

This is really sad and unfortunate. No matter what they have done, it is very wrong for us to take laws into our hands, by burning people because of one reason or the other.

No matter what they have done, we should always allow law to take its course. Let’s say no to jungle justice.

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Rampaging terrorist blocked and kidnapped travelers in Kogi state.

According to Homepage news channel it was learnt that Terrorist block road and kidnapped travelers along Abuja-Lokoja highway.

According to a report by Daily Post, the incident took place Wednesday evening at Ochonyi-Omoko village along the Abuja-Lokoja highway in Kogi State.

After the gunmen succeeded in forcing the bus to stop, they went ahead to kidnap the passengers at gunpoint and whisked them into the bush.

The sad incident happened around 8pm on that fateful night. One of the passengers who managed to escape narrated the incident.

The passenger identified as Benjamin Isaac revealed that he and few others were lucky to have escaped from being abducted by the bandits when the gunmen were leading other victims into the bush.

The incident was also confirmed by a member of the vigilante group who spoke on the condition of anonymity. He said that he got to know about the attack while he was praying nearby.

After concluding his prayer, he mobilized other members of his group and started looking for the gunmen having seen the abandoned car from where some of the travelers were abducted.

The search for the bandits was still going on with a view to catching up with them and rescuing the victims.

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