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Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 53-54

Non-steroidal anti-inflammatory drugs use in pachydermoperiostosis and gastrointestinal complications

1 Department of Internal Medicine, Rashid Hospital, Dubai Health Authority, Dubai, UAE
2 Department of Medical Education and Research, Dubai Health Authority, Dubai, UAE
3 Department of Gastroenterology, Rashid Hospital, Dubai Health Authority, Dubai, UAE

Date of Submission21-Sep-2021
Date of Acceptance17-Oct-2021
Date of Web Publication25-Mar-2022

Correspondence Address:
Omar Yousef Al-Assaf
Department of Internal Medicine, Rashid Hospital, Dubai Health Authority, Dubai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/hmj.hmj_63_21

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Introduction: Pachydermperiostosis (PDP) is a rare multi-organ disease. In which non-steroidal anti-inflammatory drugs are used as the first line treatment. Case Report: A young male known to have PDP presented to our hospital with upper gastrointestinal bleeding. On endoscopy he was found to have multiple ulcers. Discussion: We discuss the possible association between the two diseases and possible approaches in such cases. Furthermore, our case suggests the need for a well-recognized screening program with routine upper GI endoscopies.

Keywords: Gastroenterology, pachydermoperiostosis, rheumatology

How to cite this article:
Darwish I, Al-Assaf OY, Al-Ashoor M, Al-Baghdadi N. Non-steroidal anti-inflammatory drugs use in pachydermoperiostosis and gastrointestinal complications. Hamdan Med J 2022;15:53-4

How to cite this URL:
Darwish I, Al-Assaf OY, Al-Ashoor M, Al-Baghdadi N. Non-steroidal anti-inflammatory drugs use in pachydermoperiostosis and gastrointestinal complications. Hamdan Med J [serial online] 2022 [cited 2022 May 28];15:53-4. Available from: http://www.hamdanjournal.org/text.asp?2022/15/1/53/340824

  Introduction Top

Pachydermoperiostosis (PDP) is a rare genetic disease that mostly carries an autosomal dominant pattern of inheritance and affects multi-organs such as skin, bones, joints, muscles and the gastrointestinal (GI) system.[1] The key dermatologic features include skin thickening on the face and scalp with distinctive forehead furrowing, hyperhidrosis, and seborrhoea. While the skeletal features are characterized by digital clubbing, periostosis and joint enlargement with associated arthritis and arthralgia.

The disease afflicts predominantly males, male: female ratio of 9:1, with a sudden and bimodal distribution of onset, peaking at the 1st years of life and adolescence. Studies have linked the disease to two genes HPGD and SLCO2A1, involved in prostaglandin metabolism. These genes appeared in PDP patients either sporadically or were inherited in an autosomal recessive or dominant manner.[1] Even though the pathophysiology of the disease is yet to be fully understood, it has been linked with aberrant prostaglandin E2 (PGE2) metabolism. Multiple studies have found PDP patients with elevated urinary PGE2 levels.[2]

In this case, we report a young male known to have PDP presenting with an upper GI bleed. We present the clinical findings and possible associations and considerations between the two diseases.

  Case Report Top

A 25-year-old male, known case of PDP, for which he takes sulfasalazine and etoricoxib daily, presented with progressive tiredness for 1 week associated with melena for 2 days. The review of symptoms is negative. On physical examination, the patient was haemodynamically stable with no orthostatic hypotension. On thorough physical examination, he was found to have features of PDP including; clubbing of the fingers and toes, forehead furrowing and hyperhidrosis [Figure 1]a, [Figure 1]b, [Figure 1]c. Another systemic examination was unremarkable.
Figure 1: (a-c) Physical examination of the patient

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Laboratory work-up showed a hemoglobin level of 6.8 g/dL. The patient was admitted as a case of upper GI bleed, he was started on pantoprazole infusion, transfused with two units of packed red blood cells, and underwent urgent upper endoscopy.

The upper GI endoscopy showed a prepyloric hyperemic mucosa of the stomach with scattered erosion and one small, healed ulcer. In addition, the duodenum showed bulbar deformity and erosions with three small, healed ulcers. The post-endoscopy patient was discharged in a stable condition, symptom-free, tolerating orally and repeat hemoglobin level was 9.1 g/dL.

  Discussion Top

PDP is relatively rare, constituting 3%–5% of all hypertrophic osteoarthropathy. It usually carries a good prognosis. Multiple therapeutic modalities are used depending on presenting symptoms and severity. Studies have shown that most PDP cases were treated with non-steroidal anti-inflammatory drugs (NSAIDs), with around 70% achieving clinical improvement of symptoms of arthritis and arthralgia.[3] NSAIDs inhibit the cyclooxygenase enzyme, thereby inhibiting prostaglandin synthesis. However, many patients develop refractory arthritis and arthralgia that require additional treatment. Case studies have shown some improvement with methotrexate, aescin and etoricoxib.[4],[5] Li et al. have shown treatment with etoricoxib, a selective cyclooxygenase-2 (COX-2) inhibitor, resulted in improvement in pachyderma, digital clubbing and joint swelling.[5] Select cases have also utilised arthroscopic synovectomy for arthritis with joint effusion.[4] Facial skin thickening has been treated cosmetically with both plastic surgery and botulinum toxin injections.

As the mainstay treatment of PDP constitutes NSAIDs and steroids, this puts patients at a greater risk for GI complications, which could manifest as peptic ulcers, chronic gastritis, Crohn's disease, juvenile polyposis and malignancies.[1] This raises the concern regarding putting limitations on the frequent use of NSAIDs and steroids and raises multiple points of discussion whether they should be used as a first-line therapy in some selected patients with a documented GI complication. Another aspect that must be considered is whether patients with chronic use of NSAIDs and an underlying PDP should undergo frequent upper GI endoscopies to evaluate the upper digestive tract. In addition, in this subset of patients, the initiation of a lifelong proton-pump inhibitor (PPI) might be essential in addition to the patient's PDP regimen that includes NSAIDs.

As PGE2 plays an essential role in the pathogenesis of PDP, NSAIDS and steroids would undoubtedly be a cornerstone in the treatment of PDP. As a consequence, this increases the likelihood of erosive gastric or duodenal disease, as has been reported in this case. Unfortunately not enough data is available in the literature regarding the adjustments of the treatment with a co-existent GI tract complication, however, some cases published the success of using a selective COX-2 inhibitor etoricoxib and Aescin,[4] as mentioned earlier, which will impose a lesser risk of GI tract complications. The addition of a lifelong PPI is another point of discussion that we could propose as the literature is deficient in such aspect and whether it will add a benefit in the reduction in the incidence of peptic ulcer disease in people with PDP.

Periodic upper GI endoscopies are also another point of debate, multiple case reports have suggested an association between gastric adenocarcinoma and PDP.[6] Moreover, it is well established that PDP is associated with peptic ulcer disease, as it was reported in our case. This indicates that the establishment of a well-recognized screening program with routine upper GI endoscopies would be of great value. There is a lack of data regarding how to approach PDP with associated GI complications. Therefore, more studies are needed to explore better treatment options and surveillance approaches to this rare disorder.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Castori M, Sinibaldi L, Mingarelli R, Lachman RS, Rimoin DL, Dallapiccola B. Pachydermoperiostosis: An update. Clin Genet 2005;68:477-86.  Back to cited text no. 1
Giancane G, Diggle CP, Legger EG, Tekstra J, Prakken B, Brenkman AB, et al. Primary hypertrophic osteoarthropathy: An update on patient features and treatment. J Rheumatol 2015;42:2211-4.  Back to cited text no. 2
Shakya P, Pokhrel KN, Mlunde LB, Tan S, Ota E, Niizeki H. Effectiveness of non-steroidal anti-inflammatory drugs among patients with primary hypertrophic osteoarthropathy: A systematic review. J Dermatol Sci 2018;90:21-6.  Back to cited text no. 3
Zhang H, Yang B. Successful treatment of pachydermoperiostosis patients with etoricoxib, aescin, and arthroscopic synovectomy: Two case reports. Medicine (Baltimore) 2017;96:e8865.  Back to cited text no. 4
Li SS, He JW, Fu WZ, Liu YJ, Hu YQ, Zhang ZL. Clinical, biochemical, and genetic features of 41 han chinese families with primary hypertrophic osteoarthropathy, and their therapeutic response to etoricoxib: Results from a six-month prospective clinical intervention. J Bone Miner Res 2017;32:1659-66.  Back to cited text no. 5
Ikeda F, Okada H, Mizuno M, Kawamoto H, Okano N, Okazaki H, et al. Pachydermoperiostosis associated with juvenile polyps of the stomach and gastric adenocarcinoma. J Gastroenterol 2004;39:370-4.  Back to cited text no. 6


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