|Year : 2021 | Volume
| Issue : 1 | Page : 42-43
Pachydermodactyly: Case Report
Jumana Aldhalaan1, Ruaa Alharithy2
1 College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
2 Department of Dermatology, Security Forces Hospital, Riyadh, Saudi Arabia
|Date of Submission||23-Oct-2019|
|Date of Acceptance||11-Dec-2019|
|Date of Web Publication||04-May-2021|
Dr. Ruaa Alharithy
Division of Dermatology, Security Forces Hospital, Riyadh, Salah Ad Din Al Ayyubi Road, Riyadh, 12625
Source of Support: None, Conflict of Interest: None
Pachydermodactyly (PDD) is a rare benign form of digital fibromatosis characterized by a progressive painless fusiform swelling of the periarticular soft tissue, surrounding the dorsolateral aspects of the proximal interphalangeal joint. PDD most commonly affects young adolescent males and is frequently unrecognized and misdiagnosed as inflammatory arthritis. In this report, we present a 17-year-old male with a 1-year history of progressive finger swelling in both of his hands diagnosed as PDD. We also highlight PDD's clinical and histologic features with the aim to enhance physician's awareness of this benign condition to cut unnecessary diagnostic tests and interventions.
Keywords: Digital fibromatosis, pachydermodactyly, proximal interphalangeal joints
|How to cite this article:|
Aldhalaan J, Alharithy R. Pachydermodactyly: Case Report. J Dermatol Dermatol Surg 2021;25:42-3
| Introduction|| |
Pachydermodactyly (PDD) is a rare benign form of digital fibromatosis characterized by a progressive painless fusiform swelling of the periarticular soft tissue, surrounding the dorsolateral aspects of the proximal interphalangeal (PIP) joint. Multiple variants of PPD have been reported in the literature. Its etiology is poorly understood. However, microtrauma and habitual hand movements are potential triggers. PDD most commonly affects young adolescent males and is frequently unrecognized and misdiagnosed by physicians. Herein, we report a 17-year-old male with a 1-year history of progressive finger swelling in both of his hands diagnosed as PDD with emphasis on its clinical and histologic features.
| Case Report|| |
An otherwise healthy 17-year-old male presented with a 1-year history of swelling around the PIP joint of both of his hands that has been progressively increasing. He reported that the swelling initially began in his right hand with subsequent progression to his left side after several months. There is no history of trauma, pain, morning stiffness, or limitation in movement. The patient is known to be an avid video-game player but otherwise denies any habitual hand manipulation or similar findings in any of his family members. On examination, there was an isolated bulbous fusiform swelling along the radial and ulnar aspects of the second, third, and fourth PIP joint of bilateral hands [Figure 1]. There was no erythema, tenderness, effusion, or compromise in range of motion. No other joints were involved. Laboratory investigations, including complete blood count, inflammatory markers, uric acid, rheumatoid factor, and full autoimmune workup, were all unremarkable. A plain radiograph and magnetic resonance imaging showed soft tissue swelling with no evidence of joint involvement. A skin biopsy taken from the right fourth finger revealed a hyperkeratotic stratum corneum and mildly papillomatous epidermis with a mild increase in dermal fibroblasts [Figure 2]. Based on the clinical and histologic findings, a diagnosis of PDD was made. Two trials of intralesional triamcinolone acetonide injections (0.6 cc of 2.5 mg/cc followed by 0.7 cc of 5 mg/cc) at a 2-month interval were performed with no improvement. The patient has been following up at our clinic for 2 years without any progression of the swelling.
|Figure 1: (a) Pachydermodactyly: Right hand. Increased volume and thickening surrounding the proximal interphalangeal joints of the second, third, and fourth digits (R>L). (b) Pachydermodactyly: Left hand. Increased volume and thickening surrounding the proximal interphalangeal joints of the second, third, and fourth digits (L)|
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|Figure 2: (a) Hyperkeratotic stratum corneum and mildly papillomatous epidermis (H and E, ×40). (b) Mildly increased dermal fibroblasts (H and E, ×200)|
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| Discussion|| |
PDD (pachy: thick, dermis: skin, dactylos: digits) was first described in medical literature in 1973 by Bazex and coined in 1975 by Verbov., It is a rare form of digital fibromatosis typically presenting as a progressive painless swelling of the dorsolateral soft tissue overlying the PIP joints of the second, third, and fourth fingers, with sparing of the thumb, defined as classic PDD. Other variants of PDD have been described in the literature; monopachydermadactyly (localized to one finger), transgradients (extending into the metacarpophalangeal joint), familial, and syndromic (tuberous sclerosis and Ehlers–Danlos). It predominantly affects healthy adolescent males, with a male-to-female ratio of 4:1. The exact pathophysiologic mechanism is yet to be understood, but it is believed to be precipitated by microtrauma from repetitive mechanical stimulation and habitual movements. Hence, PDD has been frequently described in athletes, musicians, and those with obsessive–compulsive and anxiety disorders. Histopathologic features of PPD include epidermal acanthosis and hyperkeratosis with increased dermal collagen deposition and fibroblast proliferation in a haphazard manner. Furthermore, hormones are believed to play a role in the pathogenesis as androgens have shown to be a trophic factor for fibroblast proliferation and collagen production, which explains the histopathologic and demographic features. The diagnosis of PDD is made by its clinical features with a lack of evidence of intra-articular or bony involvement on imaging and negative laboratory testing. Due to PDD's benign nature, treatment mainly focuses on cosmetic improvement. To date, there is variable response to treatment in the literature. The most helpful option is behavioral adjustment to eliminate mechanical stimulation and possible psychiatric evaluation in those with compulsive habitual movements. Intralesional triamcinolone and oral tranilast were shown to have favorable response in the literature., Surgical excision is an option for those who seek cosmetic benefit. PDD has shown to stabilize or even regress within a few years with no treatment.
In conclusion, we believe that it is crucial for clinicians to recognize this benign asymptomatic condition and consider it in the differential diagnosis of any adolescent male with asymptomatic finger swelling to avoid unnecessary referrals, investigations, treatments, and patient anxiety.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Authors would like to thank Dr. Tariq Alzaid, consultant pathologist, for for providing us with the biopsy images and interpretation.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bazex A, Dupre A, Teillard J. Digital pachyderma of the phalanges with dermal conjunctival hyperplasia and hypodermic aplasia. Bull Soc Fr Dermatol Syphiligr. 1973;80:455-8.
Verbov J. Letter: Pachydermodactyly: a variant of the true knuckle pad. Arch Dermatol. 1975;111:524.
Vale LR, Coeli FR, Michalouay N, Hassun KM, Porro AM. Transgrediens pachydermodactyly: Report of a case. An Bras Dermatol 2009;84:190-3.
Beltraminelli H, Itin P. Pachydermodactyly – just a sign of emotional distress. Eur J Dermatol 2009; 19: 5-13
Acar A, Dirican F, Yaman B, Tamsel I, Keser G, Karaarslan I. Swollen digits due to pachydermodactyly resembling inflammatory arthritis. European Journal of Rheumatology. 2019;6:57-9.
Ohno H, Kowatari Y, Owaki M, Ohta J, Nakajima N, Yoshioka K, et al
. Effects of androgens on cultured cells derived from canine anterior cruciate ligament. Okajimas Folia Anat Jpn 2012;89:35-8.
Iraci S, Bianchi L, Innocenzi D, Tomassoli M, Nini G. Pachydermodactyly: A case of an unusual type of reactive digital fibromatosis. Arch Dermatol 1993;129:247-8.
Plana Pla A, Bassas Vila J, Toro Montecinos MA, Ferrandiz Foraster C. Pachydermodactyly successfully treated with triamcinolone injections. Actas Dermosifiliogr 2014;105:319-21.
Higuchi C, Tomita T, Yoshikawa H. Pachydermodactyly treated with tranilast in a young girl. Case Rep Orthop 2014;2014:132854.
Fleeter TB, Myrie C, Adams JP. Pachydermodactyly: A case report and discussion of the pathologic entity. J Hand Surg Am 1984;9:764-6.
Curley RK, Hudson PM, Marsden RA. Pachydermodactyly: A rare form of digital fibromatosis-report of four cases. Clin Exp Dermatol 1991;16:121-3.
[Figure 1], [Figure 2]