|IMAGES IN RHEUMATOLOGY
|Year : 2018 | Volume
| Issue : 4 | Page : 282-283
Reena Kumari Sharma1, Mudita Gupta1, Samriti Sood1, Archit Gupta2
1 Department of Dermatology, Venereology and Leprosy, IGMC, Shimla, Himachal Pradesh, India
2 General Surgery, IGMC, Shimla, Himachal Pradesh, India
|Date of Web Publication||18-Nov-2018|
Dr. Mudita Gupta
Department of Dermatology, Venereology and Leprosy, IGMC, Shimla, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Keywords: Benign, digital fibromatosis, pachydermodactyly
|How to cite this article:|
Sharma RK, Gupta M, Sood S, Gupta A. Pachydermodactyly. Indian J Rheumatol 2018;13:282-3
Pachydermodactyly (PDD) is a rare disease with thickening over the lateral aspect of fingers and swelling at the level of proximal interphalangeal (PIP) joints. PDD was first described by Bazex et al., in 1973, and the term “PDD” was coined by Verbov, literally meaning thickened skin of fingers. There are only about 150 cases reported in literature worldwide. We present a case of a 14-year-old boy with PDD.
A 14-year-old boy presented with painless swelling and thickening of fingers of both hands at the level of PIP joints for the past 2 years. There was no history of morning stiffness, joint pain, any functional impairment, oral ulcer, seizures, behavioral abnormalities, or any trauma. He denied any history of rubbing or interlacing the fingers. He had no history of similar complaint in the family. On examination, he was a healthy adolescent male without any systemic complaints. There were bulbous swellings of 2nd–4th fingers of bilateral hands with thickening and roughening of fingers. The swellings were more prominent over the dorsolateral aspects of the fingers [Figure 1]. Blood investigations including complete blood count with differential count were normal. Antinuclear antibody (ANA), rheumatoid factor, and thyroid functions were normal. Erythrocyte sedimentation rate was 8 mm/1st h. X-ray of hand showed soft-tissue swelling of 2nd–4th fingers surrounding PIP joints without any bone or joint abnormality [Figure 2]. On psychiatric consultation, no abnormality was detected. Final diagnosis of PDD was made. He was treated with intralesional injections of triamcinolone 20 mg/ml and under follow-up. He was also advised to wear gloves and avoid friction.
|Figure 1: Hands of the child showing thickening of skin over dorsolateral aspect of the 2nd to 4th fingers at the level of proximal interphalangeal joints of bilateral hands and sparing of thumbs|
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|Figure 2: X-ray showing soft-tissue swelling only without involvement of bones or joints|
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PDD is a rare form of digital fibromatosis, most commonly seen in adolescent boys. It is usually caused by repeated trauma to the sides of the fingers, by interlocking fingers, or continual hand rubbing.,
Mechanical cause in a genetically predisposed person is thought to be an important factor in the pathogenesis of PDD. Association with tuberous sclerosis and presence of familial variant suggest the role of genetics. Onset at puberty and predominance in males suggest the role of androgens which may act through increased fibroblast activity and collagen synthesis.
PDD presents as asymptomatic noncircumferential plaque with a fusiform swelling, most prominent over the radial and ulnar aspect of fingers. The skin over the 2nd–4th PIP joints is primarily affected, rarely the fifth, with almost always sparing of thumbs. There is no associated tenderness or arthritis. Bardazzi et al. classified PDD into five main types as follows: (1) classic PDD (idiopathic or trauma induced) and primarily described in male patients with several affected fingers; (2) localized or mono-PDD (idiopathic or trauma induced); (3) PDD transgrediens, when cutaneous changes extend to the palms or metacarpophalangeal joints; (4) familial PDD (classic or transgrediens); and (5) PDD associated with tuberous sclerosis which may be painful. Our patient was classic PDD. The other conditions which need to be ruled out before diagnosing of PDD are connective tissue diseases, obsessive-chewing pads, Asperger syndrome, collagenous plaques of the hands, juvenile digital fibromatosis, progressive nodular fibrosis of the skin, thyroid acropachy, pachydermoperiostosis, Garrod's pads in violinists, acropachydermodactyly in psoriasis, paraneoplastic acropachydermodactyly, and knuckle pads.
Connective tissue diseases have joint involvement and ANA is usually positive. Asperger syndrome and obsessive chewing pads have associated behavioral abnormalities. The site involved in collagenous plaques of hand is usually on the radial aspect of index finger and medial aspect of thumb, while in PDD, thumb is spared. Nodular lesions are present anywhere on the fingers in juvenile digital fibromatosis and all over the body in progressive nodular fibrosis of the skin. Thyroid acropachy, psoriatic acropachydermodactyly, and paraneoplastic acropachydermodactyly will have features of primary disease. Pachydermoperiostosis is associated with clubbing and pulmonary involvement. Garrod's pads are seen in violinists. The closest differential diagnosis is knuckle pads which are seen mainly over the extensor surfaces of metacarpophalangeal and PIP joints.
Treatment of PDD consists of stopping any compulsive mechanical skin irritation and cessation of manipulation, which may lead to natural regression without the need of rheumatology workup. Topical corticosteroids and intralesional triamcinolone have been found to be effective. Psychological support and psychiatric therapy may be required in some cases.,
In conclusion, PDD is a rare but benign disease. Both dermatologists and rheumatologists should be aware of the condition to avoid unnecessary investigation and treatment burden to the patient.
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.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Bazex A, Dupre A, Teillard J. Pachydermie digitale des premières phalanges par hyperplasie conjonctive dermique et aplasie hypodermique. Bull Soc Fr Dermatol Syphiligr. 1973;80:455–8.
Dallos T, Oppl B, Kovács L, Zwerina J. Pachydermodactyly: A review. Curr Rheumatol Rep 2014;16:442.
Żuber Z, Dyduch G, Jaworek A, Turowska-Heydel D, Sobczyk M, Banach-Górnicka M, et al.
Pachydermodactyly – A report of two cases. Reumatologia 2016;54:136-40.
Chen CK, Shyur SD, Chu SH, Huang LH, Kao YH, Liu LC, et al.
Pachydermodactyly: Three new cases in Taiwan. J Microbiol Immunol Infect 2015;48:340-4.
Bardazzi F, Neri I, Raone B, Patrizi A. Pachydermodactyly: Seven new cases. Ann Dermatol Venereol 1998;125:247-50.
Sinha NK, Ling SP, Nema SK, Pai DR. Pachydermodactyly does not need rheumatologic work-up. J Postgrad Med 2013;59:335-6.
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Plana Pla A, Bassas Vila J, Toro Montecinos MA, Ferrandiz Foraster C. Pachydermodactyly successfully treated with triamcinolone injections. Actas Dermosifiliogr 2014;105:319-21.
[Figure 1], [Figure 2]