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Pachydermodactyly

Authors: Dr Dushyanth Gnanappiragasam, Dermatology Specialist Registrar; Dr Karen Harman, Consultant Dermatologist, University Hospitals of Leicester NHS Trust, United Kingdom. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. January 2020.


What is pachydermodactyly?

Pachydermodactyly is a form of benign digital fibromatosis which causes progressive asymptomatic periarticular thickening, most commonly around the proximal interphalangeal joints. Some subtypes may have palmar and metacarpophalangeal (MCP) joint involvement.

Pachydermodactyly

Who gets pachydermodactyly?

Pachydermodactyly most commonly occurs in adolescents and young men. The affected male to female ratio is 4:1 [1,2].

What is the cause of pachydermodactyly?

The exact cause of pachydermodactyly is not fully understood. It has been linked to repetitive mechanical stimulation in genetically predisposed individuals. Hormonal factors may also have an influence as illustrated by the predominance in male individuals and symptoms appearing at the age of puberty.

Common triggers identified have been habits such as interlacing, stretching, rubbing and cracking of the fingers, and increased mechanical irritation related to professional sporting and musical activities.

Associations with tuberous sclerosis and Ehlers-Danlos syndrome are reported [1,2].

The pathophysiology is thought to be due to deposition of abnormal collagen in the dermis, especially loose strands of type III and type V collagen.

What are the clinical features of pachydermodactyly?

Pachydermodactyly is usually painless. Thickening around the 2nd and to 4th proximal interphalangeal (PIP) joints is most frequently seen. Some patients may also have overlying hyperkeratosis, lichenification, and hyperpigmentation.

What is the differential of diagnosis for pachydermodactyly?

Other conditions that may be considered in the differential of diagnosis of pachydermodactyly include:

How is pachydermodactyly diagnosed?

The diagnosis of pachydermodactyly is largely clinical.

Patients are often investigated to rule out inflammatory arthropathies with:

  • Rheumatoid factor (RF)
  • Anti-cyclic citrullinated peptide antibody (Anti-CCP)
  • Antinuclear antibody (ANA)
  • Extractable nuclear antigens (ENA)
  • C-reactive protein (CRP)
  • Plain radiograph, to assess soft tissue swelling, periarticular osteopenia, periostosis, erosions, cysts, or osteophytes
  • Ultrasound scan, to look for synovial hypertrophy, vascularity, and bony erosions
  • In some instances, magnetic resonance imaging (MRI), to exclude joint effusion, synovitis, or tendinitis.

Features seen in skin biopsies include [1,3]:

  • Increased fibroblast proliferation
  • Abnormal collagen deposition in the mid and deep dermis
  • Increased mucin deposition around the collagen fibres entrapping the eccrine sweat glands
  • Sometimes, hyperkeratosis, and acanthosis.

Inflammation is typically absent.

What is the treatment for pachydermodactyly?

Pachydermodactyly may reverse or resolve with cessation of mechanical stimulation if any.

Intralesional corticosteroid (triamcinolone) and surgical resection have been trialled in severe cases with some benefit.

What is the outcome for pachydermodactyly?

Overall, pachydermodactyly remains stable. Permanent joint deformities or damage are rarely observed.

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References

  1. Dallos T, Oppl B, Kovács L, Zwerina J. Pachydermodactyly: A Review. Curr Rheumatol Rep 2014; 16: 442. DOI: 10.1007/s11926-014-0442-7. PubMed
  2. Paravina M, Stanojević M, Jovanović D, Ljubisavljević D. Pachydermodactyly: A Case Report and Literature Review. Serbian J Dermatol Venereol 2014; 6: 174-185. DOI: 10.2478/sjdv-2014-0015. Available at: content.sciendo.com/view/journals/sjdv/6/4/article-p174.xml?lang=en (accessed 13 October 2019)
  3. Barnes LA, Bae GH, Lewis MA, Rieger KE. Pachydermodactyly: Case report including clinical and histopathologic diagnostic pitfalls. J Cutan Pathol 2018; 45: 949–53. DOI: 10.1111/cup.13359. PubMed

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