Pachydermodactyly and Atrophia maculosa varioliformis cutis

Abstract

Pachydermodactyly is a rare form of superficial digital fibromatosis characterized by progressive asymptomatic thickening of the back and sides of the proximal interphalangeal joints of the fingers. Atrophia maculosa varioliformis cutis is an acquired dermal atrophy, localized on the cheeks. Only a few cases of each pathology have been published. We find it interesting to report the case of a patient with both conditions as these two connective-tissue diseases are very rare. The association is probably fortuitous.

A Case of Atrophia Maculosa Varioliformis Cutis

“…The etiology of AMVC is unknown, although a familial occurrence has previously been reported 2,3 , which suggests that AMVC is an inherited disorder rather than a response to an environmental insult. Cases associated with extrahepatic biliary atresia 4 and pachydermodactyly 5 have been described, but these associations may be coincidental. The diagnosis of AMVC is mainly made on a clinical basis, with patients having facial atrophic lesions with sharply defined edges and an absence of erythema, induration, pigmentary changes or a honeycombed pattern.…”

A Case of Atrophia Maculosa Varioliformis Cutis

“…In the literature, associated diseases include extrahepatic biliary atresia 8 , pachydermodactyly 9 and keratosis pilaris atrophicans 10 . However, there were no associated diseases for our case.…”

Coxins interfalangeanos sobre paquidermodactilia

“…Some authors consider it is due to compulsive neuroses, in which patients rub the fingers of one hand against those of the other. 44,50 PD has already been described associated to Dupuytren's disease, 35 carpal tunnel syndrome, 35 ginecomastia, 37 varioliform macular atrophy, 55 juvenile chronic arthritis, thyroid dysfunction, seborrheic dermatitis and glossitis, 34 tuberous sclerosis, 46,57 Ehlers-Danlos syndrome. 57 However, the most frequent association is with repetitive trauma.…”

“…Alguns autores consideram-na decorrente de ato compulsivo, no qual os pacientes atritam os dedos de uma mão contra os da outra. 44,50 A PD já foi associada à síndrome de Dupuytren, 35 síndrome do túnel do carpo, 35 ginecomastia, 37 atrofia maculosa varioliforme, 55 artrite crônica juvenil, disfunção da tireóide, dermatite seborréica e glossite, 34 esclerose tuberosa, 46,57 síndrome de Ehlers-Danlos. 57 Porém, a associação mais freqüente é com trauma repetitivo.…”