Clinical features and management of superficial fibromatoses

Abstract

Summary Fibromatoses are a group of benign connective tissue tumors characterized by the infiltrative, aggressive proliferation of well‐differentiated fibroblasts, leading to frequent local recurrence. Within this heterogeneous disease group, superficial fibromatoses show slower growth and more benign infiltration of surrounding tissues than deep fibromatoses. Superficial fibromatoses relevant to dermatology include palmar, plantar, and penile fibromatosis, knuckle pads, pachydermodactyly and infantile digital fibromatosis. They present clinically with subcutaneous nodules or cords that lead to local infiltration and limited mobility of the affected areas. Treatment options vary from watchful waiting, non‐invasive methods such as radiotherapy and intralesional corticosteroid/collagenase injections to radical surgical procedures. Early intervention may disrupt disease progression and may even restore functional ability. These disorders should therefore be recognized and treated early in the course of the disease.

A Case of Knuckle Pad Syndrome in a Middle-Aged Man

“…They typically manifest in the second and third decade of life [4]. They may be seen in a number of inherited syndromes including palmar fibromatosis (Dupuytren's disease), plantar fibromatosis (Ledderhose's disease), camptodactyly, epidermolytic palmoplantar keratoderma, Bart-Pumphrey syndrome, acrokeratoelastoidosis of Costa [2,[5][6][7]. They may also be secondary and acquired through trauma.…”

A case of knuckle pad syndrome in a middle‐aged man

“…They typically present at the PIP joints rather than the MCP joints but can present in any of the small joints of the hands. 2 Less commonly, the feet and knees may be involved.…”

High-Resolution Ultrasonographic Appearances in Superficial Fibromatoses: Palmar and Plantar Diseases

“…Younger lesions appear as hypercellularity, whereas older ones reveal much less cellularity with more prominent collagen. Moderate mitotic activity without malignancy is shown [25]. Figure 2…”

“…Dupuytren's disease is a routine for the dermatologist, because patient with subcutaneous nodule may firstly visit a dermatologist. Kelenjian et al also suggested that superficial fibromatoses relevant to dermatology include palmar, plantar, and penile fibromatosis, knuckle pads, pachydermodactyly and infantile digital fibromatosis [25]. Palmar fibromatosis was described by the French physician Dupuytren and is referred to as Dupuytren disease [23,26,27].…”