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      D-penicillamine-induced pseudo-pseudoxanthoma elasticum and extensive elastosis perforans serpiginosa with excellent response to acitretin

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          D-penicillamine (DPA)-induced pseudo-pseudoxanthoma elasticum (PXE) and elastosis perforans serpiginosa (EPS) has been reported in the past, but most of the treatment modalities used before have a suboptimal response. We report a case of DPA-induced pseudo-PXE with extensive EPS who had an excellent rapid response to acitretin. To the best of our knowledge no such report has been published in the past, even though there is a single report of effectiveness of isotretinoin in elastosis perforans serpiginosa.


          One similar case but with a different medication (reference 13).

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          Most cited references 14

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          A review of acitretin, a systemic retinoid for the treatment of psoriasis.

           John Koo,  Chai Lee (2005)
          Acitretin is a second-generation, systemic retinoid that has been approved for the treatment of psoriasis since 1997. It can be considered one of the treatments of choice for pustular and erythrodermic psoriasis. However, the efficacy of acitretin as a monotherapy for plaque psoriasis is less, although it is often used in combination therapy with other systemic psoriasis therapies, especially ultraviolet B or psoralen plus ultraviolet A phototherapy, to increase efficacy. Such combination treatments may potentially minimise toxicity by using lower doses of each of the two agents. All systemic retinoids are potent teratogens. The most common side effects are mucocutanous effects such as cheilitis and hair loss, which are dose-dependent. Acitretin is not immunosuppressive, is generally safe for long-term use and has no time limit restrictions, which makes it useful in combination therapy and for maintenance therapy.
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            Penicillamine revisited: historic overview and review of the clinical uses and cutaneous adverse effects.

            Penicillamine is a well-known heavy metal chelator, classically used in the treatment of Wilson disease, rheumatoid arthritis, and cystinuria. From a dermatologic standpoint, penicillamine was found to be useful in the treatment of systemic sclerosis. The successful therapeutic uses of penicillamine have been hindered by its numerous adverse effects, both cutaneous and extra-cutaneous. It is a unique drug since it provokes a diversity of dermatologic manifestations that include (1) acute hypersensitivity reactions, (2) dermopathies characterized by elastic fiber abnormalities including elastosis perforans serpiginosa and pseudo-pseudoxanthoma elasticum, (3) autoimmune disorders such as pemphigus and penicillamine-induced lupus erythematosus-like syndrome, and (4) miscellaneous dermatoses that result from undefined mechanisms. These cutaneous adverse effects may correlate with the dosage and duration of penicillamine therapy as well as the disease being treated.
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              Imiquimod therapy for elastosis perforans serpiginosa.


                Author and article information

                Ann Saudi Med
                Ann Saudi Med
                Annals of Saudi Medicine
                King Faisal Specialist Hospital and Research Centre
                Jan-Feb 2019
                31 January 2019
                : 39
                : 1
                : 56-60
                [a ]Department of Dermatology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
                [b ]Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
                Author notes
                Correspondence: Dr. Muzamil Amin Chisti, MBC 46 Department of Dermatology, King Faisal Specialist Hospital and Research Centre, PO Box 3354, Riyadh 11211, Saudi Arabia, T: +966114424608, janphiny@ 123456gmail.com , ORCID: http://orcid.org/0000-0002-6877-2715
                Copyright © 2019, Annals of Saudi Medicine

                This is an open access article under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND). The details of which can be accessed at http://creativecommons.org/licenses/by-nc-nd/4.0/

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