K. Saini, J. Relic, R. Malesu
Introduction: Pachydermodactyly (PDD), describes a rare condition of localised ﬁbromatosis. It predominantly affects adolescent males who usually have no family history and may be associated with repetitive mechanical stimulations
Case study: A 16-year-old, right-hand dominant male pre- sented with a 4-year history of hypertrophy of the soft tissue surrounding his right PIP joints. In addition, swelling of soft tissue over the extensor surface and palm of the right hand, with thickening of the overlying skin was observed. Mild soft tissue and skin changes of the left thumb were also noted. However, the remainder of the left hand was unremarkable. The patient described intermittent pain in his affected PIP joints, with no functional deformity. His general examina- tion was otherwise normal, and he had no personal or family history of concern. Correlation of his clinical presentation, histological and radiographic ﬁndings, resulted in the diag- nosis of PDD. In our patient, the unilateral presentation rep- resents an even rarer atypical variant.
Discussion: There are no universally accepted treatments for PDD. However, improvement with intralesional triam- cinolone injections and surgical resection has been reported 2. Behaviour modiﬁcation if repeated tics like behaviours have been identiﬁed, may be beneﬁcial. Usu- ally, due to its benign course and asymptomatic character, no aggressive treatment is required.
Conclusion: Given its rarity, PDD may be easily misdiag- nosed and confused with joint disorders, rheumatologic diseases or mucinoses 2,3. The correct diagnosis of this dis- ease and its rare subtypes is crucial in sparing patients from unnecessary invasive diagnostic procedures and immunosuppressive therapy.
1. Nicolay JP, Faulhaber J, Kutzner H, Schneider SW. A new sub- type of pachydermodactyly: unilateral pachydermodactyly trans- grediens. Clin Exp Dermatol. 2015;40(6):633–5.
2. Chu H, Song J, Kim do Y. Pachydermodactyly: A Benign Cuta- neous Condition that May Be Misdiagnosed as a Joint Disorder. J Rheumatol. 2016;43(8):1615–6.
3. Barnes LA, Bae GH, Lewis MA, Rieger KE. Pachydermodactyly: Case report including clinical and histopathologic diagnostic pit- falls. J Cutan Pathol. 2018;45(12):949–53.