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Teenage male with ‘swelling’ on the fingers
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A 15-year-old boy presents to your office with “swelling” of multiple fingers. He first noticed enlargement of some fingers approximately 1 year before his appointment. He denies pain or functional limitation. He has seen three other doctors to discuss the changes on his hands. His hobbies include practicing martial arts, which includes sessions with a punching bag; he denies playing an instrument or doing any manual labor.
On exam, you note thickening of the skin around the proximal interphalangeal (PIP) joints on the second to fourth fingers of the right hand and between the metacarpophalangeal (MCP) joints and PIP joints of the left hand. There is overlying hypopigmentation bilaterally. He also has hypopigmented papules on the second MCP joint bilaterally (Figures 1 and 2).
Can you spot the rash?
Diagnosis: Pachydermodactyly
Carrie C. Coughlin
Marissa J. Perman
Case discussion
Pachydermodactyly is a proliferation of fibrous tissue on the hands. It typically involves the PIP joints.
Differential diagnosis includes knuckle pads, calluses, palmar fibromatosis (Dupuytren’s contracture), epithelioid sarcoma and subcutaneous/deep granuloma annulare. Knuckle pads situated over the knuckles (instead of occurring laterally) can be familial or acquired (trauma/cracking). Dupuytren’s contracture occurs as a proliferation of fibrous tissue on the aponeurosis/palmar fascia. Classically, it is proximal to the fourth finger, and with time can produce contractures, although it can occur more diffusely on the palm. Epithelioid sarcoma, which also occurs most commonly on the hands of young male adults, could be considered if only one finger was affected and the lesion were nodular, as well as ulcerated and/or painful. Subcutaneous granuloma annulare would be firmer than the lesions of pachydermodactyly. If there were associated inflammation and pain of the joints, evaluation for rheumatoid arthritis could be appropriate.
Pachydermodactyly is often an isolated finding in young males. However, it has been reported in patients with Ehlers-Danlos syndrome and patients with tuberous sclerosis.
Figures 1 and 2: Fibromatosis around the PIPs of the second to fourth fingers (top, Figure 1) and between the MCPs and PIPs of the second to fourth fingers (bottom, Figure 2) are typical of pachydermodactyly.
Images: Coughlin CC
We did not perform a biopsy because the patient’s presentation was classic for this disorder. Pachydermodactyly has most often been noted as a reaction pattern to repetitive trauma. Although not the hand-rubbing often reported, our patient’s martial arts practice could have triggered his condition, as he often uses a punching bag to train. Repetitive hand motions, such as stereotyped hand movements, and trauma associated with job functions, such as chicken-handling, have been identified as aggravating factors. As the lesions are not often painful and do not impede function, intervention is optional. However, some patients are distressed by its appearance. Potential therapies have included behavior modification, intralesional steroid injections and surgical excision. Our patient was considering stopping his use of the punching bag, but otherwise was not interested in other therapy.
It is important for physicians to be aware of this entity to facilitate a more timely diagnosis and decrease unnecessary testing.
References:
Aldrich CS. J Am Acad Dermatol. 2010;63:244-251.
Hunt R. Dermatol Online J. 2010;16:5.
Plana Pla A. Actas Dermosifiliogr. 2014;105:319-321.
Sagransky MJ. Arch Dermatol. 2012;148:925-928.
For more information:
Carrie C. Coughlin, MD, is a pediatric dermatology fellow at The Children’s Hospital of Philadelphia. She can be reached at coughlincc@email.chop.edu.
Marissa J. Perman, MD, is an attending physician at The Children’s Hospital of Philadelphia.
Disclosure: Coughlin and Perman report no relevant financial disclosures.