PACHYDERMODACTYLY TRANSGREDIENS ANGELES SOLA, M.D., JAVIER VAZQUEZ-DOVAL, M.D., JESUS SOLA, M.D., AND E M I L I O QUINTANILLA, M.D.
Tbe patient was a 24-year-old man, who consulted us because of increased size of tbe proximal interphalangeal area of tbe fingers of botb hands since the age of 14. Physical examination (Fig. 1) revealed cutaneous inflammation of the dorsal and lateral sides of the 2nd, 3rd, and 4th fingers of both hands, while the 5th finger was only slightly affected. Cutaneous changes were mostly localized in the proximal interphalangeal areas with extension up to the metacarpophalangic joints. The skin had a pachydermic appearance with areas of the mild erythema and fine desquamation. There was no previous history of tenderness nor movement limitations of the fingers. There were no other skin cbanges in the hands, feet, or tbe rest of the body. There was no family history of similar skin changes. Radiograpbic studies of tbe bands were essentially normal, except for an increase in soft tissue density in the affected areas. Histologic study of a biopsy obtained from tbe dorsum of one of tbe affected fingers sbowed (Fig. 2) orthokeratosic hyperkeratosis with hypergranulosis, acanthosis, and papillomatosis of the epidermis. Dermal thickness was evident and extended to the perianexial adipose cushion, with collagen deposition in irregular thick bundles, tbere was also mild proliferation of fibroblasts in some areas and small mucin deposits (Fig. 3).
Figure 1. The figure shows a thickness of the skin of the interphalangical joints, with extension to the metacarpophalangcal areas.
There are differences in the histologic findings of patients in the few studies published. The majority of the cases''^ report that the most important characteristics are the presence of dermal thickness with an increase in collagen types III and V, and increase in tbin fiber diameter in the reticular dermis, and a vague demarcation between papillar and reticular dermis. Other authors'" emphasize that hyperkeratosis with acanthosis of the epidermis is the principal histologic lesion. This should include pachydermodactyly in the differential diagnosis of the idiopathic hyperkeratosis, which further separates it from the knuckle pad concept. Our case has a histopathology similar to that reported by Verbov,' in that we observed epidermal hyperplasia with dermal thickness due to an increase in collagen. Nevertheless, this patient is different to other cases reported, because the skin changes also affected the metacarpophalangeal joint as well as the proximal interphalangeal joints. This lesion is basically irreversible, although intralesional infiltration with triamcinolone acetonide has been slightly effective in diminishing inflammation of the affected zones' in one patient. Subcutaneous resection of the excess tissues has likewise given good cosmetic results.** Based on the clinical data and the histopathologic findings, we were able to exclude typical knuckle pad
Pachydermodactyly has been defined as a rare form of digital fibromatosis, characterized by an asymptomatic thickness of tissues surrounding the proximal interphalangeal joints of the 2nd, 3rd, 4th, and occasionally, 5th fingers of the hands. It is usually seen in men after adolescence. It was first described in 1975;' nine cases have since been reported with pachydermodactyly. One of them was associated with carpal tunnel syndrome.^ Pachydermodactyly was initially considered as a variant of atypical knuckle pad, but his idea was later rejected; it was then described as a distinct clinical disease of unknown etiology.^^ From the Departments of Dermatology and Pathology, University Clinic School of Medicine, University of Navarra, Pamplona, Spain. Address for correspondence: Javier Vazquez-Doval, M.D., Department of Dermatology, University Clinic of Navarra, Apdo 192, 31080 Pamplona, Spain. 796
Pachydcrmodactyly l ransgrcdiens Sola ct ill.
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Figure 2. Low power view of the skin, showing marked hyperkeratosis, acanthosis, and papillomatosis. Dermis is thickened by irregular bundles of collagen, (hematoxylin and eosin, original magnification x 40)
Figure 3. tHIigh power field of the deep dermis showing adnexal atrophy and absence of fatty tissue. Collagen is arranged in thick bundles irregularly distributed, (hematoxylin and eosin, original magnification x 400)
or a rare form of hyperkeratosis in our patient. We considered this entity within the concept of pachydermodactyly. This disease exhibits a peculiar presentation characterized by thickness of the dorsal and lateral skin of the fingers with the characteristic histopathology previously described. Because the cutaneous thickness in our patient extended to the metacarpophalangeal areas, we defined this clinical presentation like pachydermodactyly transgrediens as an exceptional form of pachydermodactyly.
Verhov J. Pachydermodactyly: a rare form of the true knuckle pads. Arch Dermatol 1975; 1 1 1:524. Reichert CM, Costa J, Barsky SH, Clay et al. Pachydermodactyly. Clin Orthop 1985; 194:252-257. Curley RK, Hudson PM, Mnrsden RA. Pachydermodactyly. A rare form of digital fihromatosis. Report of four cases. Clin Exp Dermatol 1991; 16:121-123. Fleeter TB, Myrie C, Adams JP. Pachydermodactyly: a case report and discussion of pathologic entity. ] Hand Surg Am 1984; 9:252-257.
Erratum In the July 1992 issue (Vol. 31, No. 7) of the/owrwa/ an error appeared on page 467. In the third paragraph it should have stated that: "Zyderm I: contains 35 mg/niL of collagen (95% type I, 5% type III), saline, and lidocaine." The Editor apologizes for the inaccuracy. 797