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Dowling Degos Disease: Treatment, Cost and Side Effects

Last Updated: Jan 20, 2025

What is Dowling Degos Disease ?

Dowling Degos Disease is a rare kind of skin disease which generally occur during the adulthood age between 15 to 59 years of age. It is generally characterized by spots over neck cheek and sometimes in another part of the body which involves numerous small round pigmented macules even over groins and face. The first time the disease was described by Kohlmeier it was then recognized by Robert Degos. The origin of such disease is basically unknown. The condition of this disease is inherited in an autosomal dominant pattern. The awareness of such kind of disease is not very much. The main complaint was found among patient was cosmetic disfigurement which occurred because of pigmented spots and pits. It is also known as “pigmented reticulate anomaly of the flexures”. The spots may have developed colors of black and brown. The diagnosis of such rare disease is very difficult and often very challenging for the physicians and the doctors. Physicians associated with health care looks upon patient medical history, symptoms and laboratory test result for diagnosis. The Genetic Testing Registry or in short (GTR) is used to test such genetic condition.

How is the treatment done?

As the name indicates that Dowling disease is rare the treatment is also very rare previously there was no such effective treatment was discovered by the physician. Treatment was done on symptomatic base. Recent research has helped in the development of a treatment with the use of eculizumab and Treprostinil. The first one is very expensive and needed to be infused every fourteen days. The second one has been much more effective. Laser treatment called Er.YAG in years has also been successful. People who are diagnosed with such rare skin disease with many hyperpigmented macules on the face and inguinal fold can undergo this treatment with prior consultation with doctors. Laser therapy needs to follow some procedures which are continued for one whole year. This treatment was given in every three weeks followed by fractional Co

  • After five sessions of therapy, the patient showed a lot of improvement like reduction of spots etc.

    Laser treatment might have an adverse effect on the people living in Asian countries. People with darker skin can also face post inflammatory hyperpigmentation. Laser treatment was first carried on by a Korean woman and the outcome was effective. The percentage of success rate is higher in laser therapy than other oral medicines but there might be some immediate and midterm effect on the patient depending upon the hormonal balance in the patient body like erythema, pain and swelling might occur. The treatment is also dependent on age and health of the patient

  • Who is eligible for the treatment?(When is the treatment done ?)

    Any person who is suffering from this disease and who has family history of the mutation of KRT5, POFUT1, or POGLUT1 gene report mostly to the cases of Dowling-Degos disease.Such patients should get tested for the disease.

    Who is not eligible for the treatment?

    Any person who has no such mutation cases in the family history and who lacks any symptomps like dark dots in skins etc need not go for the treatment. The doctor shall advise for the treatment eligibility

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    Are there any Side Effects?

    Since this is a genetic disorder the treatment are less effective. Only surgery can be a case which can give rise to some complications. Every surgery has a certain side effect but it is difficult to say that the results of the treatment might not be permanent because of its genetic nature.

    What are the post-treatment guidelines?

    The post-treatment guidelines are very similar to all other surgeries like taking rest is important for the first few days, physicians might advise painkillers for discomfort. Use of sunscreen and tropical adapalene are advised to continue between the sessions.

    How long does it take to recover?

    As the treatment is similar to other surgeries it is a long-term process and patient have to sit for numerous sessions as the disease is not very common. They should be handled with great care because it takes quite a long time to recover. As the disease is rare so there is a high risk of relapsing the same.

    What is the price of the treatment in India?

    The Dowling Degos disease is a rare kind of disease and is a genetic disorder the discovery of medicines of such rare skin disease was also very tough so the price naturally is a little bit expensive than any other normal laser treatment. Genetic testing can be in the range of 13,000 to 20,000 INR. Apart from that the cost of treatment shall be based on the consultation fees which can range from 500 to 2000 INR.

    Are the results of the treatment permanent?

    Since this is a genetic disorder its treatment is still not effective and thus the results are not permanent in nature.

    What are the alternatives to the treatment?

    There is no such an alternative treatment of this kind of skin disease many other ways have been tried which involves depigmenting agents such as hydroquinone and systematic and tropic retinoids but it was laser treatment only which was successful and this modern technique can be viewed as an alternative therapeutic option.

    References

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    Written ByDr. Neha Anand Gadodia MBBS Bachelor of Medicine and Bachelor of Surgery,DNB - Dermatology & VenereologyDermatology
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