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| CASE IN POINT: MUSCULOSKELETAL CLINICS |
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| Year : 2015 | Volume
: 2
| Issue : 2 | Page : 105-106 |
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Pachydermodactyly
Sumalatha K Bhaskaraiah1, Dheeraj Adiga2, Ramita Sardana3, Upinderpal Singh3
1 Department of Physical Medicine and Rehabilitation, Sanjay Gandhi Institute of Trauma and Orthopaedics, Bengaluru, Karnataka, India
2 Department of Physical Medicine and Rehabilitation, Manipal Hospital, Bengaluru, Karnataka, India
3 Department of Physical Medicine and Rehabilitation, AIIMS, New Delhi, India
| Date of Web Publication | 28-Dec-2015 |
Correspondence Address:
Sumalatha K Bhaskaraiah
Department of Physical Medicine and Rehabilitation, Sanjay Gandhi Institute of Trauma and Orthopaedics, Byrasandra, Jayanagar East, Bengaluru - 560 011, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2349-0977.172683
How to cite this article:
Bhaskaraiah SK, Adiga D, Sardana R, Singh U. Pachydermodactyly. Astrocyte 2015;2:105-6 |
A 23-year-old male student, from Delhi, presented with swelling around the joints in the fingers of both hands for the past 3 years. Onset was insidious and nonprogressive with no history of pain, early morning stiffness, and redness in the joints. There was no history of trauma, activities involving repetitive finger rubbing movement, similar complaints in other joints or in any other family member, and any difficulty with the activities of daily living. Before presenting to us, he was diagnosed as having rheumatoid arthritis and was treated with methotrexate (7.5 mg once a week for 4 months) and prednisolone (10 mg once a day for 3 months); however, the treatment was discontinued because no symptomatic improvement was observed.
Physical examination revealed symmetric soft tissue swelling around the proximal interphalangeal (PIP) joints of the second to fifth fingers, with slight epidermal thickening [Figure 1]. There was no deformity, rise of temperature, or tenderness over the swellings. The swelling was soft to firm in consistency, with a negative fluctuation test. The range of motion of the joints was normal. Rest of the musculoskeletal and general examination showed no abnormality. | Figure 1: Swelling around the PIP joints in the second to fifth fingers.
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Routine blood investigations and rheumatoid factor and antinuclear antibody tests were unremarkable. Skiagrams of the hands revealed soft tissue swelling [Figure 2] without joint space narrowing, bony erosions, periarticular osteopenia, or other structural abnormalities. Patient refused for investigations such as magnetic resonance imaging (MRI) and soft tissue biopsy.
On review of literature, it was observed that his clinical presentation was similar to that of pachydermodactyly (PDD). He was counseled regarding the benign condition of the disease.
He was recommended intralesional injection of triamcinolone; however, the patient refused treatment considering the benign nature of the disorder.
PDD, from the Greek words pachy (thick), dermo (skin), and dactylos (finger), was first reported by Bazex et al. in 1973[1] and named by Verbov [2] in 1975. It is a rare, benign form of digital fibromatosis affecting the PIP joints of the fingers.[3] Approximately 100 cases of PDD have been reported worldwide [4] and a few from India.[5]
Typical presentation includes asymptomatic swelling of the soft tissues over the PIP joints without any damage to the joint structures.[1] No other joints are affected. It affects otherwise healthy young individuals (mean age: 21.2 years, male/female ratio: 3/2).[2] The evolution of joint swelling is insidious, usually symmetrical, and often predominant in one or a few joints.[6] PDD has occasionally been reported to involve the metacarpal phalangeal joints or the palm or dorsum of the hand, termed as pachydermodactyly transgrediens.[7]
The etiology is unknown. Most cases have no familial link or associated comorbidity. Repetitive minor trauma caused by habitual or compulsive habits of interlacing or rubbing of the fingers has been reported as a cause of PDD.[5],[8]
The diagnosis is clinical and is supported by radiological studies. Bone scintigraphy, ultrasonography, and MRI reveal soft tissue swelling with no bone or articular abnormalities. All laboratory tests are unremarkable. Serology results are negative for antinuclear antibody and rheumatoid factor.[6] Histopathologic examination reveals an increase in dermal collagen, with variable degrees of overlying hyperkeratosis and acanthosis.[5] Collagen isolated from the involved sites shows a structure consistent with that of collagen types III and V; this differs from the collagen profile of the normal skin.[9]
Differential diagnoses include inflammatory arthritis such as rheumatoid arthritis, psoriatic arthritis, and knuckle pads.[5]
PDD has a benign evolution. Intralesional administration of triamcinolone hexacetonide or subcutaneous resection of the excess tissues can improve cosmesis,[10] but topical steroid therapy is ineffective. Psychotherapy may be an option in those with compulsive behavior.[8] Follow-up is essential to assess progress.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | |  |
| 1. | Bazex A, Dupré A, Teillard J. Pachydermie digitale des premières phalanges par hyperplasie conjonctive dermique et aplasie hypodermique. Bull Soc Fr Dermatol Syphiligr 1973;80:455-8.  |
| 2. | Verbov J. Letter: Pachydermodactyly: A variant of the true knuckle pad. Arch Dermatol 1975;111:524. [ PUBMED] |
| 3. | Seo SH, Sung HW. A case of pachydermodactyly. Ann Dermatol 2011;23:258-61. |
| 4. | Beltraminelli H, Itin P. Pachydermodactyly—Just a sign of emotional distress. Eur J Dermatol 2009;19:5-13. |
| 5. | Kumar P, Gharami R. Pachydermodactyly: A classical case of this rare disease. J Turk Acad Dermatol 2009;3:93401L. |
| 6. | Ye S, Chen SL, Dong YQ, Lin F, Guo Q, Bao CD. Pachydermodactyly: Six new cases from China. J Clin Rheumatol 2005;11:72-5. |
| 7. | Sola A, Vazquez-Doval J, Sola J, Quintanilla E. Pachydermodactyly transgrediens. Int J Dermatol 1992;31:796-7. |
| 8. | Dupin N, Gautier MS, Rabary G, Auffret N, Beltzer-Garelly E, Binet O. Pachydermodactyly. Ann Dermatol Venereol 1994;121:632-4. |
| 9. | Kang BD, Hong SH, Kim IH, Kim WK, Oh CH. Two cases of pachydermodactyly. Int J Dermatol 1997;36:768-72. |
| 10. | Costa MM, Romeu JC, da Costa T. Pachydermodactyly a rare cause of finger joint swelling. J Rheumatol 1995;22:2374-5. [ PUBMED] |
[Figure 1], [Figure 2]
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