Nail degloving, a polyetiologic condition with3 main patterns: A new syndrome

Robert Baran, MD,a and Christophe Perrin, MDb

Cannes and Nice, France

Nail degloving refers to partial or total avulsion of the nail and surrounding tissue (perionychium).Typically it appears as a thimble-shaped nail shedding or a partial or total loss of the nail organ with softtissue. Nail degloving represents the end result of a variety of insults to the nail apparatus, includingtrauma, dermatologic diseases, and drug reactions. ( J Am Acad Dermatol 2008;58:232-7.)

ANatomically, nail degloving encompasses 3presentations with some overlap. First, in thetypical thimble-shaped nail shedding, the

walls of the thimble are composed of the skin of thedistal digit including the nail plate (circumferentialskin shedding). Second, a partially sloughed-off nailplate with its surrounding tissue composes naildegloving. Third, shedding is restricted to the entirenail apparatus and its components (matrix, nail bed,hyponychium, and ventral aspect of the proximalnailfold) but spares the surrounding epidermis of thedistal digit.

In this polyetiologic condition, lichen planus is, toour knowledge, presented here for the first time asone cause of nail degloving. The clinical and histo-logic details of this case shed light on the patho-physiology of degloving, relating to drug-inducedand gangrenous causes.

TRAUMAThe traumatic causes may be found in the home,

in industry, or in the recreation field.1 Nail avulsionshave been reported from a loop of steel wire, by awet cord while sailing, or by a ring caught whenjumping off a trailer or a ladder. Crush injuries of thefingertip may be classified as tip-amputation, volar,dorsal, or circumferential injury (19 of 44 patients inthe series of Tajima2).

From the Nail Disease Center, Cannes,a and University of Nice

Laboratoire Central d’Anatomie Pathologieque, Hopital L

Pasteur, Nice.b

Funding sources: None.

Conflicts of interest: None declared.

Accepted for publication October 27, 2007.

Reprint requests: Robert Baran, MD, Nail Disease Center, 42, rue des

Serbes, 06400 Cannes, France. E-mail: baran.r@club-internet.fr.

Published online January 3, 2008.

0190-9622/$34.00

ª 2008 by the American Academy of Dermatology, Inc.

doi:10.1016/j.jaad.2007.10.643

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Sustained dorsal injury, sparing the nail matrixand bony phalanx, but concentrated on nail plateand nail bed, are herein exclusively discussed.

Both proximal and distal regions of the nail matrixare necessary to produce a normal nail plate, andinjury to either region may cause a scarred nail.However, the nail bed also plays an essential role inthe regrowth of the nail shape and size. Nail bedinjury is one subtype of fingertip injury that deter-mines whether or not a normal-looking nail regen-erates. Besides preservation of the matrix, a nearlyintact nail bed with a physiologically curved, smoothsurface and proper epithelial elements are necessaryprerequisites for normal nail regrowth, particularlyin regard to maintaining attachment of the nail plateto the underlying nail bed epithelium (Fig 1, A). Withthis in mind, it becomes important to restore thetraumatized nail bed with minimal debridement andwound closure when possible. When necessary, asplit-thickness nail bed graft from the adjoining areaon the nail bed or from a toe can be used to cover awider wound.3

Fig 1, A, shows one example of traumatic deglov-ing. After disinfection the avulsed nail plate wasreplaced on the torn nail bed, sutured on the lateralnailfold, and bandaged. This produced an excellentlong-term result (Fig 1, B). When the nail plate isunavailable, silicone sheeting may be used as asubstitute, sutured in place of the nail plate in similarfashion.4

IATROGENIC CAUSESIatrogenic diseases are a significant cause of nail

degloving. Toxic epidermal necrolysis provides themost typical cases5 (Fig 2 and Fig 3, A). Usually naildegloving as a result of this reaction is followed byregrowth of a normal nail (Fig 3, B).

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GANGRENOUS CONDITIONSGangrene refers to an end-stage severe necrotiz-

ing and sloughing process, and may be subdividedinto dry and wet types. Dry gangrene of the digits isgenerally allowed to demarcate itself: a line developsbetween the living tissue and the necrotic area. Theterminal necrotic tissue then sloughs at this line ofdemarcation.

Gangrene of the nail apparatus may result fromthe following. First, digital ischemia, which mayresult in frank (homogeneous) gangrene, may occurin diabetes, embolic diseases, or vasculitis. Digitalischemia has also been associated with underlying

Fig 1. A, Traumatic injury of distal digit sparing nail matrixand bone. B, Same digit, healed.

malignant neoplasms and disseminated intravascularcoagulation6 (Fig 4). The latter is most commonlyassociated with acute myeloid or lymphoblasticleukemia, but also with bacterial and viral infectionsof the newborn.7 Vascular malformations may pro-duce a similar picture. Second, streptococcal gan-grene appears most often in patients with anunderlying immunodeficiency. Bullae surroundingthe distal toes in patients with diabetes may produceprogressive necrosis around the nail, leading topartially sloughed off tissue. Third, as a secondaryevent, gangrene may result from trauma or infec-tion.8 Fourth is acute digital gangrene in the new-born. The occurrence of acute peripheral gangrenein newborns is a rare emergency event. A few hoursafter delivery, the newborn develops blisters on thedigits. Gangrene appears the following day.9-11 Thedifferential diagnosis includes infection; metabolic,genetic, and drug-induced conditions; a vasculitissyndrome; or conditions related to vascular malfor-mations. In the case of Wollina and Verma,12 thenewborn had acute finger gangrene caused by ma-ternal antiphospholipid syndrome.

DERMATOLOGIC DISEASE: CASE REPORTA 50-year-old man from Senegal presented with

all 10 dystrophic fingernails. Some demonstratedremnants of nail keratin, whereas others showedonychomadesis with a normal regrowing nail plate.However, two signs were common to all 10 nails: awhitish material at the base of the nail plates and aswollen proximal nailfold (Fig 5, A). The latter was

Fig 2. Partial nail degloving in Lyell’s syndrome. (Cour-tesy of Goettmann-Bonvallot, France.)

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painless but sensitive with pressure. When progress-ive pressure was applied on the proximal nailfold,the whitish material became more prominent (Fig 5,B) and appeared totally flat. Simultaneously, weobserved an advancing outward extrusion of theentire nail apparatus (Fig 5, C ), rigid enough tomimic a balloon fish completely opened at the rear(Fig 5, D).

The patient refused further examination. He wasotherwise in good health with normal blood workfindings (complete blood cell count; C-reactive pro-tein level; prothrombin time; antinuclear anti-bodies). His history was unremarkable.

METHODSThree specimens were examined histologically.

The main piece of tissue showed an epithelial ring atits proximal portion. This was processed in trans-verse serial sections. The superior and inferior por-tions had been separated to visualize the specimenmore carefully. The left sample corresponded to thekeratotic block surmounted laterally by the nailplate. Multiple longitudinal sections were takenstarting from this area toward the zone without thenail plate (Fig 6). The two other fragments involved,

Fig 3. A, Thimble-shaped nail shedding, total nail deglov-ing in Lyell’s syndrome caused by carbamazepine. B, Samepatient with regrowth of normal nails. (Courtsey of P.Souteyrand, France.)

respectively, sections of the proximal nailfold andthe nail matrix.

RESULTSFragments of the proximal nailfold and the matrix

showed a totally naked dermis fringed with a lym-phocytic infiltrate presenting as a lichenoid band.

The proximal ring of the round piece of tissuecorresponded to an epithelial layer with keratohyalingranules, almost totally lacking in dermis. The epi-thelium of the upper transverse sections was thin,and above it, basket-weave orthokeratotic layeredstratum corneum was visible. The basal layer wasalmost straight. In contrast, epithelium of the lowertransverse section was thick and remnants of kera-togenous zones were noted in a few rare areas(Fig 7). The horny layer of the lower transversesections was compact and associated with zonescomposed of a homogenous texture and an absenceof affinity for eosin stain, demonstrating a continuingsynthesis of the nail plate and resulting in theappearance of a pseudonail. The pseudonail wassurmounted by a compact cornified layer. The upper

Fig 4. A, Digital gangrene as result of disseminatedintravascular coagulation. B, Partially sloughed-off nailwith surrounding tissue, showing healed digit.

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Fig 5. A, Lichen planus involvement of whole nail apparatus. B, Partial extrusion of nailapparatus, with progressive pressure on proximal nailfold. C, Complete extrusion of entire nailunit. D, Appearance of balloon fish completely opened at rear.

portion of this pseudonail was undulated withdepressions filled by the compact horny layer(Fig 7). These histologic characteristics suggestedthat the upper transverse layers were the middle anddistal ventral portion of the proximal nailfold andthat the inferior sections were the nail matrix with amodified nail plate and the horny layer of theproximal ventral portion of the proximal nailfold.

Both samples showed evidence of lichenoiddermatitis: the basal membrane infiltrated by lym-phocytes showed vacuolar interface change andkeratinocytic necrosis. In addition, the lower trans-verse sections showed an irregular epidermal hyper-plasia with a triangular, sawtooth configuration.

The longitudinal samples (Figs 8 and 9) corre-sponded to the nail bed and hyponychium and mostlacked dermis except for rare fringes of dermalpapillae, adherent to the epithelium. These papillaewere filled with a lymphocytic infiltrate. Vacuolarinterface change was diffuse and basal necrosis and adense lymphocytic infiltrate obscured the dermoe-pidermal interface (Fig 9). The nail bed demon-strated hypergranulosis.

These histologic changes suggested the diagnosisof hyperkeratotic and pseudobullous nail lichen

planus, with dramatic evidence of avulsed epithelialstructures (ventral portion of the proximal nailfold,matrix, nail bed, and hyponychium), seeminglyejected from their dermal base.

DISCUSSIONClinically, nail degloving encompasses 3 main

varieties with some overlap. First is typical thimble-shaped nail shedding. The walls of the thimble arecomposed of the skin of the distal digit including thenail plate (circumferential skin shedding). Second ispartially sloughed nail plate with its surroundingtissue. Third is shedding restricted to the entire nailapparatus, sparing the surrounding epidermis of thedistal digit. This type was elegantly illustrated by theprevious case histology and presentation of naillichen planus. The different structures involvedinclude the ventral aspect of the proximal nailfold,nail matrix, nail bed, and hyponychium. The histo-pathologic features observed in this case demon-strate the process of degloving and reaction of thenail apparatus in lichenoid, iatrogenic, and gangre-nous diseases.

Two main causes explain this process (Figs 6 and10). First, is the fragility of the dermoepidermal

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junction. In lichen planus and in toxic epidermalnecrolysis this fragility is linked to the vacuolaralteration and/or cell death along the dermoepider-mal interface with an additional papillary edema iniatrogenic conditions. In cases of gangrene, thecleavage is produced by papillary edema and globalischemic necrosis of the epithelium secondary to thevascular thrombosis. In addition, the most proximalportions of the epithelium of the ventral aspect of theproximal nailfold and the proximal matrix keep theirattachments to the neighboring dermis (Fig 6 and10). Consequently they do not participate in the

Fig 6. Keratotic and pseudobullous lichen planus. Ventralpart of proximal nailfold in its middle and distal portion.AC, Cavity caused by artefact; CCLE, compact cornifiedlayer of proximal eponychium; CLM, cornified layer ofmatrix; HB, histologic borders between transverse andlongitudinal sections; IT, inferior transverse section; LCLE,laminated cornified layer of eponychium; NP, nail plate;ST, superior transverse section; single continuous blacklines, portion of nail apparatus that has been extruded bydigital pressure on proximal nailfold; double interruptedgreen lines, epithelial zones of nail apparatus thatremained adherent to dermis; single interrupted red/blackline, exposed dermis resulting from cleavage phenome-non involving dermoepidermal junction.

Fig 7. Inferior transverse section of nail matrix, sur-mounted by neonail plate and compact horny layer ofproximal portion of ventral aspect of proximal nailfold.CCLE, Compact cornified layer of eponychium; CLM,cornified layer of matrix; arrow, remnants of small kera-togenous zone (KZ ). (Hematoxylin-eosin stain; originalmagnification: 34.)

process of extrusion of the nail apparatus. Thisseparate plane of cleavage explains the detachmentof almost the complete nail apparatus, the formationof a partial or total cavity, and the subsequentregeneration of the nail unit.

The histology provides the key to understand thisnew syndrome and the different aspects of the initialcavity. The initial clinical cavitary appearance de-pends on two factors: (1) extension of the zone ofdermoepidermal cleavage on the proximal nailfoldand the region of the pulp; and (2) intensity of thereactive epithelial hyperplasia of the detached zone.

In lichen planus (Fig 6) the detachment involvesall the middle and distal portion of the ventral aspectof the proximal nailfold and spares the back portionof proximal nailfold and pulp epidermis. The lichen-oid process progresses slowly, explaining the epi-dermal hyperplasia. The cavity observed after nailapparatus extrusion corresponds to the inherentweakness of the two keratotic layers of the ventralaspect of the proximal nailfold, which is physiolog-ically made of two zones13: (1) a compact layer or

Fig 8. Longitudinal section of nail bed with granularmetaplasia. (Hematoxylin-eosin stain; original magnifica-tion: 34.)

Fig 9. Longitudinal section of nail bed. Note colloidbodies. (Hematoxylin-eosin stain; original magnification:320.)

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true cuticle; and (2) a laminated stratum corneum orfalse cuticle normally limited to the distal portion ofthe ventral aspect of the proximal nailfold. Theinferior border of the cavity is formed by the compactlayer of the proximal portion of the ventral aspect ofproximal nailfold (eponychium) and the nascent nailplate. The adherence between the two inferior hornylayers is the result of the interpenetration of thestratum corneum of the eponychium to the undula-tion of the pseudonail plate, composed of hyper-granulosis. The superior border of the cavity isformed by the en bloc detachment of the middleand distal portion of the epithelium of the ventralaspect of the proximal nailfold and of its layeredstratum corneum loosely connected to the compactlayer of the eponychium.

Conversely, in the setting of iatrogenic and gan-grenous diseases (Fig 10) the cleavage extends to the

Fig 10. Anatomy in cases of nail degloving caused bytoxic epidermal necrolysis and some cases of digitalgangrene. CB, Clinical and histologic border betweenzone where nail epithelium remains adherent to dermisand zone of dermoepithelial cleavage; NP, nail plate; singlecontinuous black lines, portion of nail apparatus extruded;double interrupted green lines, epithelial zones of nailapparatus left adherent to dermis; single interrupted red/black line, exposed dermis resulting from cleavage phe-nomenon involving dermoepidermal junction.

epidermis of the back of the proximal nailfold andinvolves the whole pulpar epidermis. In this situa-tion, the process starts suddenly. The epidermis ofthe ventral aspect of the pulp is degloved simulta-neously with the nail apparatus remaining rathernormal and opens a thimble-shaped cavity.

Despite the impressive and dramatic appearanceof nail degloving, it may be compatible with a fairlygood recovery and normal nail plate regrowth.

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