Periodic Shedding of the Nails

Abstract:  2 year old girl with seven month history of nail shedding

HPI:  This otherwise healthy 2 year-old girl has been losing nails since about 18 months of age.  By history, the nails turn black and then are shed.  The patient's father says his small toe nail sheds periodicaly and his father may have a nail dystrophy, also.  This grandfather has a muscular disorder.

OE:  There are six or seven nails with which shoe subungual hemorrhage, onycholysis, nail dystrophy or absent nails.  Fingers are more affected than toenails, however, many of the toenails are dystrophic.

Clinical Photos:

12/10/13

1/14/14

Here is a two week f/u of nail direcctly about this photo.  It's amazing how quickly the nails of young children grow.

Diagnosis:

The clinical picture is consistent with what has been called Periodic Shedding of the Nails (PPS).  There are only two articles on this entity and both are difficult to obtain.  It is possible that what was called PPS is really a localized variant of Epidermolysis Simplex. Since this is such a young child, the true nature of the disorder may become evident with the passage of time.

Discussion: We will try to obtain some opinions from experts who may have some experience here.  It is likely that this is autosomal dominant with varying degrees of penetrence.  It may improve with age.  Trauma probably plays a role in damaging the nail bed, so one wonders if there is some defect of the nail matrix or bed.  The finger pad erosion pictured above may indicate that the problem is more wide-spread than the nail matrix.

References

1. Cutis. 1980 Jun;25(6):622-3.

Familial dystrophic periodic shedding of the nails.

Martin S, Rudolph AH.

Abstract  A patient with an autosomal dominant nail dystrophy characterized by periodic, asymptomatic shedding of the nails followed by regrowth is described herein. This highly penetrant disorder is similar to two earlier cases found in the dermatologic literature.

2.  Br J Dermatol. 1973 May;88(5):497-8.

Periodic shedding of the nails.

Main RA.

3. Localized epidermolysis bullosa simplex (Weber-Cockayne type).

Villaseñor-Park J, English JC. J Pediatr Adolesc Gynecol. 2011 Dec;24(6):410-2.

 

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Nail Dystrophy x 3 Years

Abstract:  52 yo woman with 3 year history of a nail dystrophy

HPI:  The patient has had this problem for three years.  She works out a lot and recalls trauma to the toe,

O/E:  The left great toe nail is dystrophic,  The nail is quite short and there is brownish green discoloration under the abnormal nail.  There is some hemorrhage under the proximal nail fold.  

Clinical Photos:

Dermatoscopiic Image

Lab:  KOH was positive for hyphae, but fungal culture is negative at 14 days.

Impression:  Nail dystrophy in a 52 year-old woman.  While this is probably traumatic, the long history is of concern and I feel that biopsy should be considered to rule out malignancy.

Questions:  What is your diagnosis?  Would you obtain a biopsy to rule out malignancy given the long history.

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Transverse Leukonychia

Dr. Richard Ratzan, an Emergency Room physician in Connecticut, recently saw an institutionalized bipolar woman in her 50s for another problem and noted a distinctive nail dystrophy.  It looks like transverse leukonychia of which there are only a few references in the literature.  The woman has a history of biting but no significant medical history that he is aware of.  We don't know if she's had electroshock therapy or intermittent chemotherapy that may have been contributory.  Could some type of self-induced trauma have caused this?

Dr. Ratzan writes us: "Although i am not a dermatologist, i have long been interested in physical diagnosis and especially nails. i usually take a good look at my patients' nails. What struck me as interesting in this man was the following: i had never seen this pattern before; i could not and still can not imagine a pathophysiological process leading to such an unusual symmetrical pattern of feathered chevrons that were non-continuous across the longitudinal midline of the nail; and lastly, from a strictly aesthetic point of view, i find them almost weirdly beautiful!"

Discussion:  Transverse Leukonychia have been reported a number of times in the medical literature but most cases have been associated with combined chemotherapy or some other pharmacologic agents.  Reference # 3 is interesting but there is no abstract and the source is difficult to find.  Does anyone have access to this?  Muehrcke Lines and Mee's lines would appear to be different entities often confused with this type of transverse leukonychia.  Or, perhaps we are missing something inthis woman's history.

References:

1. Arch Dermatol. 1983 Apr;119(4):334-5.  Chemotherapy-induced transverse white lines in the fingernails.  James WD, Odom RB.  (A good review, but no abstract – only useful for those with access to Archive of Derm)

2.  CMAJ. 2012 Aug 27.  Muehrcke lines  Sharma V, Kumar V. (Shows why our patient does not have this.)

3.  Traumatic transverse leukonychia.  Maino KL, Stashower ME.  Skinmed. 2004 Jan-Feb;3(1):53-5. No abstract available.

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Alopecia Areata and Twenty Nail Distrophy

Abstract: 18 year old man with two year history of alopecia areata and six month history of dry lusterless nails.

HPI:  The patient is an 18 year-old college student who has had alopecia areata for the past 2 years.  He is well otherwise and has had no other problems until a number of months ago when he developed a nail dystrophy.   He takes no medications by mouth.  There is no family history of alopecia or autoimmune problems.  Treatment to date for alopecia has been intralesional triamcinlone with regrowth, however, new areas continue to evolve.

O/E:  There are 6 - 7 alopecic areas measuring from 2 to 6 cm in diameter widely scattered over the scalp.  He has some areas of alopepcia on his abdomen.
19 of his nails are dystrophic.  They are lusterless and many are greyish white in color, rough and friable.  One of his toenails appears normal

Clinical Photos:

Lab: All studies have been normal.  Records of these have been requested.

Diagnosis:  Alopecia areata and trachyonychia (aka 20 Nail Dystrophy)

Discussion and Questions: The patient has read about immunotherapy with DNCB and related chemicals.  Has anyone treated a patient with this modality who experienced long-lasting remission?  Do you know of good treatments for his nail dystrophy?

References: 
Alopecia universalis with twenty-nail dystrophy (trachyonychia).

Chien P Jr, Kovich OI.

Dermatol Online J. 2008 May 15;14(5):24.

Department of Dermatology, New York University, USA.

Abstract

A 43-year-old man presented with long-standing trachyonychia of all 20 nails, which worsened after the onset of alopecia universalis 18 months ago. Trachyonychia can be associated with alopecia universalis although the treatment strategies of both conditions differ. The principle of treating trachyonychia may involve regulating the differentiation of keratinocytes and/or reducing inflammation in the nail fold or nail matrix while treatment of alopecia universalis involves immunomodulation.

Free Full Text

Indian J Dermatol Venereol Leprol. 2011 Nov-Dec;77(6):640-5.

Trachyonychia: a comprehensive review.

Gordon KA, Vega JM, Tosti A.

Source

Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida 33136, USA. kagordon@med.miami.edu

Abstract

Trachyonychia or rough nails, may present as an idiopathic disorder of the nails or it can be associated with other dermatological conditions. The dystrophic nail findings seen in trachyonychia are characterized by brittle, thin nails, with excessive longitudinal ridging. The most common histopathologic features associated with trachyonychia are spongiosis and exocytosis of inflammatory cells into the nail epithelia; typical features of lichen planus or psoriasis can also be detected. Determining the cause of trachyonychia is challenging. Treatment is often unsatisfactory, although in general it should be aimed at the underlying cause, if found. In most cases, the nail abnormalities improve spontaneously.  Available full text

Vañó-Galván S, et. al. Sudden hair loss associated with trachyonychia. Cleve Clin J Med. 2008 Aug;75(8):567-8
Department of Dermatology, Ramón y Cajal Hospital, University of Alcalá, Madrid, Spain. sergiovano@yahoo.es  Free Full Text

UTILITY OF DERMOSCOPY IN ALOPECIA AREATA

Mandar Mane, Amiya Kumar Nath, and Devinder Mohan Thappa

Indian J Dermatol. 2011 Jul-Aug; 56(4): 407–411  F  Free Full Text

Twenty-nail dystrophy of alopecia areata.

Horn RT Jr, Odom RB.

Arch Dermatol. 1980 May;116(5):573-4

Abstract

We describe here a patient with dystrophy of all 20 nails, which has persisted for five years after the resolution of alopecia areata. We feel that the term "20-nail dystrophy" is best used to describe a clinical entity that can have several causes.

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Nail Dystrophy in a Nurse

A colleague requested our opinion regarding a woman who contacted him, but has not seen.  Here are her words and photos.

" I am a 61 yo palliative care nurse who has had a nail dystrophy for around 14 months. There is no pain or discomfort. I take no meds by mouth and am in good general health. Every 2 weeks I get a professional manicure and have never had acrylic, gel or silk.  I tried "formaldehyde free" products for a while, as well as Biotin 5000mcg daily and noted no improvement.  As part of my job, I visit patients in their homes and wash my hands before & after each visit. Rarely do I have the need to wear gloves.  Your suggestions will be welcome."

Questions at this time:
1) Are all nails involved (including toe-nails)?
2) Has a dermatologist been consulted and have KOH prep and/or fungal culture been done?

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Two Patients with Longitudinal Nail Dystrophy

This past month, I saw two patients with median nail dystrophies. This is an area that has only rarely been written about. These patients are presented for your interest and thoughts. If you experience difficult with the comment function, you can email DJ Elpern with your thoughts.

Case 1.
55 yo man with a 1-2 year history of a linear striation of the left thumbnail. This is painful with pressure and occasionally spontaneously painful. There is a three mm in diameter pink striation in the left thumbnail beginning at the proximal nail fold. The distal portion of the nail is somewhat deformed and there is the suggestion of an erythematous subungual papule.

Diagnosis: Possible Subungual tumor. I am considering glomangioma. (See below for follow-up)
Questions: Would the best approach be to avulse the entire nail and then do a small elipse? What else would you do here?
Follow-up: The patient first saw a hand surgeon who recommended amputation of the distal portion of the digit. Scared, he saw a second hand surgeon who said he thought this was a glomus tumor and excised it. Pathology confirmed the diagnosis of Glomus Tumor. This photo was taken approximately two months post-surgery.

18 mo post surgery

24 mo post surgery

Case 2.
60 yo woman with 3 month history of an asymptomatic longitudinal split on the left thumbnail. No history of trauma.

Diagnosis: I favor median nail canal (dystrophia unguis mediana canaliformis) here, although at first was concerned about a subungual tumor.
Question: Would you observe or explore and biopsy? Has anyone had success treating this entity?
Follow-up: This lesion was excised by an orthopedic surgeon in November of 2011. It was a Glomus tumor.

Comment: Until I prepared these cases for presentation the diagnoses were less clear to me (perhaps I am wrong anyways). Getting them ready for VGRD-Blog was a good educational exercise. Joubert wrote: "To teach is to learn twice."

Nail References:
Verma SB. Glomus tumor-induced longitudinal splitting of nail mimicking median canaliform dystrophy. Indian J Dermatol Venereol Leprol. 2008 May-Jun;74(3):257-9. (Free Full Text)
Abstract:
Median canaliform deformity of the nail is an uncommon entity, where there is longitudinal splitting of the nail. Longitudinal splitting of the nail is a rare phenomenon and can also occur following number of growths arising in the nail matrix. On examination there was a longitudinal split in the nail plate, beginning in the distal nail fold and extending proximally all the way to the proximal nail fold. There was a small, almost indiscernible, swelling in that area, which was exquisitely tender. The split part of the nail showed a little discoloration. There was no discharge, bleeding, or subungual mass visible. 'Love test' was positive in this case. After nail avulsion, a small 2 mm x 4 mm nodule was exposed and excised. Histopathological examination of the tumor showed a mantle of glomus cells surrounding the blood vessels.

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Melanonychia Totalis

Abstract: 70 yo African-American woman with black toe-nails for many years.

HPI: This otherwise healthy 70 yo woman was seen for lichen simplex chronicus of the dorsum of the feet. An incidental finding was that of black toe nails. Anamnesis reveals that this has been present for greater than ten years. She is was on no meds by mouth when this developed.

O/E: Most of her toe-nails are black. One or two have longitudinal melanocytic striae. Her finger nails are normal. The toe nails are thickened with subungual hyperkeratosis.

Clinical Photos:

Lab: The KOH was negative and a fungal culture was obtained on April 21, 2011

Diagnosis: Melanonychia. Is this a dermatophyte, a yeast or a saprophyte? We will wait to see what culture shows. What are your thoughts?

Reference:
A case of melanonychia due to Candida albicans
Lee SW, et. al. Clin Exp Dermatol. 2006 May;31(3):398-400.
Abstract: Melanonychia is characterized by tan, brown, or black pigmentation within the nail plate. Fungal melanonychia is rare and may simulate longitudinal melanonychia caused by melanocytic lesions. We report six cases of fungal melanonychia which were confirmed histopathologically or mycologically. On culture, Candida and/or Aspergillus species were isolated in four patients. The nail pigmentation improved after treatment with antifungal agents in all cases, but one patient experienced a new lesion on another nail after cessation of treatment. Fungal infection should be considered as a cause of melanonychia, and fungal melanonychia should be differentiated from the melanonychia caused by melanocytic lesions, particularly by subungual melanoma.

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Nail Dystrophy in an Eight Year-Old Girl

Introduction:  In the past, we published a case of localized acrodermatitis continua.  The father of a child with this diagnosis in the U.S. came across our post on VGRD and asked our advice for his daughter.  Your opinions may help with the diagnosis and management of this child.  One can only imagine how this disorder impacts on a young child. Perhaps, one of us has had a favorable outcome with a similar patient.


History:  Please help with an opinion on our eight year-old daughter who has had an acral dermatitis for the past 5 years.   The swelling started at the cuticle and slowly moved back towards the first knuckle over the years and was associated with itching. Initially it was diagnosed as insect bites.  About a year ago her fingers became more swollen and a doctor made a clinical diagnosis of fungus (no tests were done).  She was treated first with vinegar soaks, then triamcinalone cream then Grifulvin 125mg/tsp.  None was effective and we then saw a new dermatologist who referred us to a pediatric dermatologist who she made a diagnosis: Acrodermatitis Continua of Hallopeau.  She did a fungal culture which grew out a soil contaminant that was not felt to be significant.   Our daughter is presently on clobetasol ointment.  The nail looks a bit better but not the skin.  Treatment discussions so far have included Thalidomide, Psoralen plus UVA or UVB, Acitretin, Methotrexate and others.  We know that these medications can have serious side-effects and that this disease can be resistant to treatment.  Our daughter has a lot of finger pain and can't pick up thing with her fingers.  She is only a child and we'd appreciate your thoughts.

Clinical Photos:

Questions:
1) Are there alternative diagnoses?
2) What therapies have you had success within similar cases?
3)  Any further work-up?

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Onychomadesis

The patient is a 21 yo college student who emailed me around a month ago. He was away at school at the time:
Nov. 15, 2008 Dear Dr. Elpern, I was wondering if you had any idea what this skin rash / irritation is being caused by. On my hands and feet I've got these little red dots scattered all over. They don't itch, but offer a mild pain when applying pressure. Most of them are plush (sic) with the skin, but some of them are raised up slightly. Also my taste buds are inflamed and red... but I think this is an unrelated condition. Any help you could offer would be greatly appreciated.
He wrote back on December 20, 2008: Shortly after writing you the dots seemed to go away, so I didn't bother setting up an appointment; however, although the red dots went away, I did notice that the white half circle, that are supposed to be at the bottom of the nail, seemed to become weird and displaced on both middle fingers. About two weeks went by and nothing really changed. Yesterday things got worse. Both my middle finger nails seem to be falling off at their roots. I'm not sure what's causing this, and I was wondering if you thought I should set up an appointment, or if you think that I should seek help elsewhere.

O/E: The patient was seen on December 23, 2008: At this time, he had a separation of the proximal nail fold of both middle fingers. No other abnormal findings.

Clinical Photos:




Diagnosis: Post viral onychomadesis. The illness he had was most likely Hand, Foot and Mouth Disease or a related enterovirus infection. I have never seen nail dystrophy after this, but onychomadesis has been reported at least three times after similar episodes. One report is of an outbreak in Spain. I wonder if this is not another enterovirus infection.
Question: Has anyone else seen this?
References:
1. Salazar A, et al. Onychomadesis outbreak in Valencia, Spain, June 2008. Euro Surveill. 2008 Jul 3;13(27). pii: 18917. Available Full Text
2. Bernier V, Labrèze C, Bury F, Taïeb A. Nail matrix arrest in the course of hand, foot and mouth disease. Eur J Pediatr. 2001 Nov;160(11):649-51
Onychomadesis describes complete nail shedding from the proximal portion; it is consecutive to a nail matrix arrest and can affect both fingernails and toenails. It is a rare disorder in children. Except for serious generalised diseases or inherited forms, most cases are considered to be idiopathic. Few reports in literature concern common triggering phenomena. We present four patients in whom the same benign viral condition in childhood appeared as a stressful event preceding onychomadesis. In each case, spontaneous complete healing of the nails was achieved within a few weeks. CONCLUSION: Onychomadesis and/or onycholysis is a newly recognised complication in the course of viral infections presenting clinically as hand, foot and mouth disease, and because of mild forms, is probably underestimated.
Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: a report of five children. Pediatr Dermatol. 2000 Jan-Feb;17(1):7-11.
Hand-foot-mouth disease (HFMD) is a contagious enteroviral infection occurring primarily in children and characterized by a vesicular palmoplantar eruption and erosive stomatitis. Nail matrix arrest has been associated with a variety of drug exposures and systemic illnesses, including infections, and may result in a variety of changes, including transverse ridging (Beau's lines) and nail shedding (onychomadesis). The association of HFMD with Beau's lines and onychomadesis has not been reported previously. Five children, ages 22 months-4 years, presented with Beau's lines and/or onychomadesis following physician-diagnosed HFMD by 3-8 weeks. Three of the five patients experienced fever with HFMD, and none had a history of nail trauma, periungual dermatitis, periungual vesicular lesions, or a significant medication intake history. All patients experienced HFMD within 4 weeks of one another, and all resided in the suburbs of the Chicago metropolitan area. In all patients the nail changes were temporary with spontaneous normal regrowth. The mechanism of the nail matrix arrest is unclear, but the timing and geographic clustering of the patients suggests an epidemic caused by the same viral strain.

Comment: It is likely that this young man's nails will regrow. However, it may take longer than in a young child. All other previous cases have been in children. It is also possible that this is a related virus and not the usual putative agent of HFAM Disease.

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