Pachyonychia Congenita

Wright, Carroll S.

doi:10.1001/archderm.1947.01520060081009

Cited by 18 publications

(3 citation statements)

References 4 publications

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“…4,[6][7][8] Control of the hypertrophic nail plates by amputation of the distal phalanx, while effective, is unacceptably extreme. 9 Cosman et al reported nail plate, GME, and pSME followed by full-thickness skin graft coverage with the remaining nail bed developing hyperkeratinization. 10 A more aggressive approach was reported by White and Noone in which the nail plate was excised with GME and then covered with a split-thickness skin graft.…”

Section: Discussionmentioning

confidence: 99%

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Surgical Management of Pachyonychia Congenita in a 3-Year-Old

Sudduth,

Clinker,

Holdaway

et al. 2023

Arch Plast Surg

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Pachyonychia congenita is a rare genetic disorder characterized by hypertrophic nail plates, hyperkeratotic nail beds, and thickened hyponychium of the fingers and toes, impairing manual dexterity and resulting in poor aesthetics. The current body of literature describes various treatment modalities, but no singular approach has been defined as the gold standard. In this case, the authors employed different surgical techniques for treating pachyonychia congenita to evaluate the most effective approach. A 3-year-old boy presented with hypertrophic nail growth involving all digits of both hands and feet. Three surgical procedures were performed on the patient's fingers and toes using germinal matrix excision (GME) alone, GME plus partial sterile matrix excision (pSME), or GME plus complete sterile matrix excision (cSME). The digits treated with GME + cSME exhibited no recurrence of nail growth. Those treated with GME alone exhibited recurrence of hypertrophic nail growth, although their growth slowed. Excision of GME + cSME prevented recurrence of hypertrophic nails, while GME alone or with pSME led to slower-growing hypertrophic nails. Complete excision of the germinal and sterile matrices with skin graft closure may be a definitive treatment for pachyonychia congenita, but further studies are needed to validate these findings.

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Section: Discussionmentioning

confidence: 99%

“… 4 6 7 8 Control of the hypertrophic nail plates by amputation of the distal phalanx, while effective, is unacceptably extreme. 9 …”

Section: Discussionmentioning

confidence: 99%

Surgical Management of Pachyonychia Congenita in a 3-Year-Old

Sudduth,

Clinker,

Holdaway

et al. 2023

Arch Plast Surg

View full text Add to dashboard Cite

show abstract

“…Pachyonychia and leukokeratosis of the tongue are often noted in affected newborn babies. Later on, follicular kera tosis starts on the elbows and knees and spreads to other parts of the body (8,13). Histopathological examination reveals hyperkeratosis, acanthosis and horny plugs in follicular orifices (I, 13).…”

Section: Discussjonmentioning

confidence: 99%