Pachydermodactyly

A 16-year-old boy presented to the Pediatric Dermatology Clinic at the Charles C. Harris Skin and Cancer Pavilion with a two-year history of asymptomatic swelling of fingers on both hands. His condition had remained undiagnosed after previous evaluation by several dermatologists and hand specialists. He initially had noticed increased fullness of his proximal left fourth digit. Several months later, he noted swelling of his left fifth digit and right second through fourth digits. The patient reported no pain, pruritus, restriction of movement, morning stiffness, or trauma. He also denied repetitive hand-rubbing movements although his mother stated that he had this habit as a child. Past medical history included allergic rhinitis and asthma. Similar hand findings were not present in any other members of his family.     

厚皮指症

报告1例厚皮指症。患者男,19岁。双手近端指间关节梭形肿胀伴皮肤增厚、粗糙1年,无疼痛,双手指活动不受影响。手部X线检查示双手示指、中指、无名指和小指的近端指间关节周围软组织肿胀。组织病理学检查示表皮角化过度,棘层肥厚,真皮胶原纤维增多,排列不规则,真皮网状层成纤维细胞轻度增生。免疫组化染色结果示真皮网状层轻度增生的梭形成纤维细胞表达波形蛋白(vimentin)。其临床表现及组织病理学检查符合厚皮指症的诊断。     

Two Cases of Pachydermodactyly

We report two cases of pachydermodactyly. Case 1 was a 16-year-old girl who complained of asymptomatic, bulbous, firm swellings which developed insidiously on both sides of the proximal interphalangeal (PIP) joint of her right middle finger. Case 2 was a 14-year-old boy with similar lesions on the sides of the PIP joints of the index and middle fingers of both hands. They both had histories of mild, repetitive mechanical trauma of the fingers. Radiologic findings showed soft tissue swellings without any bony or articular abnormalities. Histopathologic findings from the bulbous swellings revealed marked hyperkeratosis, slight epidermal hyperplasia, and a markedly thickened dermis with a deposition of mucinous material among the collagen fibers. Ultrastructural examinations of both cases showed decreased diameters of collagen fibrils. The lesions temporarily improved with intralesional injection of triamcinolone acetonide. Pachydermodactyly is more commonly found in boys and the affected fingers are more numerous in boys. Both of the present cases had the habit of rubbing and gripping their fingers unconsciously. Mechanical trauma of the fingers around puberty may play an important role in pachydermodactyly.     

A Case of Pachydermodactyly

Pachydermodactyly (PDD) is a rare, benign form of digital fibromatosis and this is characterized by asymptomatic soft tissue swelling that affects the lateral aspects of the proximal interphalangeal (PIP) joints of the fingers. Although the etiology of PDD is unknown, the possibility of repetitive minor trauma by habitual or compulsive habits of interlacing the fingers or rubbing of the fingers has been suggested as a cause by several authors. We experienced a 14-year-old boy who was diagnosed as having PDD by the clinical manifestations and this was supported by a radiological study and the routine laboratory tests. He also had the habit of repetitively manipulating his hands when feeling emotional distress. PDD sometimes can be misdiagnosed as a rheumatic condition. Although an unusual disorder, PDD should be considered in the differential diagnosis of patients who present with digital bulbous swelling.     

厚皮指症1例

患者女,22岁。左手无名指近端指间关节肿胀伴两侧皮肤增厚半年。左手X线示:左手无名指近端关节肿胀,骨质无破坏,关节无异常。皮损组织病理示:表皮角化过度,棘层增厚,真皮胶原纤维增多,排列不规则。诊断:厚皮指症。     

Pachydermodactyly: A Case Report

Pachydermodactyly is an uncommon variant of digital fibromatosis that has previously been related to knuckle pads. It is a benign condition that needs no investigation for an underlying systemic disease. We present a new case of pachydermodactyly and comment on the available literature.     

Pachydermodactyly in Two Young Girls

Abstract: Pachydermodactyly is a benign, superficial fibromatosis frequently observed in young males. We describe the condition in two young girls affected by Ehlers-Danlos syndrome (EDS) and tuberous sclerosis, respectively. We hypothesized that in the patient with tuberous sclerosis, pachydermodactyly is a clinical manifestation of the associated systemic disease, whereas obsessive-compulsive behavior is probably the main cause in the girl with EDS.     

厚皮指症一例

患者,男,30岁。因双手关节梭形肿胀、皮肤增厚7年,于2015年5月18日来我院就诊。7年前患者发现右手中指关节肿胀,呈梭形。伴局部皮肤增厚,皮色及皮温正常,无发热、关节痛、晨僵等症状,逐渐发展至其他手指,偶有麻木感,各关节无疼痛及晨僵,局部皮肤无瘙痒及疼痛等,双手活动未受明显影响。患者从事钢筋工作10余年,反复用手绕钢筋。既往史及家族史无特殊。体格检查：一般情况好,各系统检查无异常。     

Pachydermodactyly: Report of a case

We report a case of pachydermodactyly, a rare entity characterized by acquired diffuse swelling of the lop and sides of the fingers.     

Pachydermodactyly, a new case from Iran

Pachydermodactyly (PDD) is a rare form of benign digital fibromatosis involving the proximal portion of lateral sides of fingers. It is characterized by asymptomatic and symmetrical soft tissue swellings of the radial and ulnar aspects of proximal PIP joints of second to fifth fingers. Herein we report a 15-year-old boy with this condition.     

Fall 42. Nagel-Patella-Syndrom (Turner-Kieser-Syndrom)

Ohne Zusammenfassung     

Pachydermodactyly: case report and review of the literature

BACKGROUND: Pachydermodactyly is a benign form of digital fibromatosis usually presenting in male adolescent patients as asymptomatic nodules on the lateral aspects of the proximal interphalangeal joints of the fingers. It is commonly misdiagnosed as a juvenile rheumatologic condition. OBJECTIVE AND CONCLUSION: Recognition of the features of this disease will assist dermatologists and rheumatologists in making the diagnosis of this rare condition.