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.

Congenital Hypopigmented Macules

Presented by Henry Foong, Ipoh, Malaysia
A healthy 16 year old girl complains of asymptomatic 1-2 mm in diameter hypopigmented macules on both shins since birth. There are similar, but to a lesser extent, macules on the arms. Her elder sister has similar lesions. In an older individual with later onset I would have thought of idiopathic guttate hypomelanosis. However, these lesions were congenital and her sister is similarly affected. It does not look like a form of dyschromia but plain hypopigmentation. so unlikely to be dyschromatosis symmetrical hereditaria? or dyschromia cutis amyloidosis? Does not look like pigmentary mosaicism either. Any suggestions? Click images to enlarge.










Impression: Congenital Hypopigmented Macules. Has anyone seen a similar case?

References:
1. Fukai K, et.al. Monozygotic twins with congenital guttate leukoderma. Osaka City Med J. 2005 Jun;51(1):33-6. fukai@msic.med.osaka-cu.ac.jp
Abstract: We report here two cases of congenital guttate hypomelanotic macules observed in monozygotic twins. They both have had discrete leukoderma regions in the axillae, inguinal region and lower abdomen since birth. The size and the shape did not change until at least the age of nine. Development of both patients was otherwise normal. The split-DOPA reaction revealed no DOPA-positive melanocytes in the hypomelanotic skin, but electron microscopy revealed melanocytes that were regular but decreased in number. Cytogenetic analysis of the peripheral leukocytes revealed normal female karyotype in both cases. Considering the unique pattern of the leukoderma lesions which occurred in both monozygotic twins, this might be a new clinical entity.

2. Grosshans E, Sengel D, Heid E. White lentiginosis [ in French] Ann Dermatol Venereol. 1994;121(1):7-10.
Abstract
INTRODUCTION: A congenital guttate hypomelanosis is an unusual feature not yet mentioned in the dermatologic literature.
CASE REPORT: We observed 1982 in a 28 y. female patient numerous guttate lesions, which were flat and pigmented on the light-exposed areas of her limbs, flat or papulokeratotic and depigmented on her trunk. These lesions disclosed a particular histological aspect characterized by a lentiginous hyperplasia of the epidermis, with elongated club-shaped rete ridges, and an unusual loss of pigmentation without disturbance of the keratinization. Further electronmicroscopical and immunohistochemical data were not available. The patient emphasized the congenital occurrence of these lesions, whose fixity could be assessed during a 4 year-follow up time.
COMMENTS: The unusual histological aspect allows the differentiation of these depigmented spots and other known similar conditions: macular leucoderma as sequellae of previous inflammatory diseases, hypomelanotic macules associated with genodermatoses, idiopathic guttate hypomelanoses.
CONCLUSION: This seems to be a not yet described entity which we propose to denominate "white lentiginosis".

KS in Renal Transplant Patient

Omid Zargari, a dermatologist from Rasht, Iran, is asking for your help regarding a 74 year old man with extensive Kaposi's sarcoma after renal transplantation. The disease began about two years ago, when he was on Cyclosporine (plus prednisolone). At that time, I asked the nephrologist to change CsA with Sirolimus. Now, he's on Pred+cellcept+sirolimus.
I've seen several cases of post-transplant KS. All of them regressed after discontinuing CsA and haven't seen a case with such extent. HHV8 screening is not available here. I referred him to an oncologist, but he refused to start any chemotherapy because he believed this is not a life-threatening condition....considering the amount of impact the disease has put on the QOL of this gentleman, he is seeking for any help...at least a palliation.
What do you suggest?

Smart Phone Fingers?

The patient is a 15 yo girl with a 2-3 year history of painful thumbs. The palmar surface of her thumbs were glazed with decreased fingerprint markings. She has mild hyperhidrosis palmaris. One great toe has mild plantar hyperkeratosis but is not glazed like the thumbs. I noticed a cell phone in her back pocket and asked her to show me how she uses it (see below). She's had this for three years. Her father said she's on the smart phone for hours a day.
Is this a new entity? Contact? Irritant? Repetitive Trauma? Comments?

BCC Tip Nose

The patient is a 70 yo woman who had a nasal bulb lesion biopsied in September 2010. This was an ill-defined area and two, 2-mm biopsies were taken. One showed a superficial and nodular BCC ang the other a melanocytic nevus. This was probably a collision lesion. The patient elected to wait and see what developed.

Today, April 29, 2010, the exam shows a residual lesion with arborizing blood vessels on dermoscopy. This lesion requires definitive treatment either with micrographic surgery or radiotherapy and the patient is leaning towards the former.

Question: With re: Moh's surgery, what kind of closure you you recommend?

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.

Amelanotic Acral Lentiginous Melanoma

Abstract: 61 yo man with 4 - 5 year hx of a tumor on foot.

HPI: The patient is a healthy 61 year old man with a 4 - 5 year history of a slowly growing lesion on the plantar aspect of his right foot. On a recent trip to Jamaica it bled, leading him to consult a podiatrist who astutely did a biopsy. The patient has sarcoidosis which has been treated with weekly i.m. methotrexate for the past two years. (I do not know the dose byt presume it is around 15 mg).

O/E: 2 x 1 cm flesh-colored nodule. Crust in photo is from punch biopsy. Remainder of cutaneous exam unremarkable. No palpable regional lymph nodes. Dermatoscopic exam was not rewarding.

Photo:

Dermoscopic Images:

Lab: Mild leucopenia 3700. Otherwise all chemistries and LDH normal

Pathology: ALM 3.68 (at least) mm thick, (at least) Level IV.
Tumor thickness may be deeper tumor is present at the base of the specimen.
Regression: Not Present
Vascular/lymphatic invasion: Not identified
Mitotic Activity: 7/10 HPF
Tumor Infiltrating Lymphocytes: Non-brisk
Vertical Growth Phase: Present

Discussion: Although this tumor is called "acral lentiginous melanoma" it clearly is a nodular lesion. Might it better be called "acral nodular melanoma?" The patient will need staging and the, depending on findings of staging studies, a wide-local excision with lymph node mapping . He is being referred to the melanoma clinic at Dartmouth Mary Hitchcock Medical Center.

This is an amelanotic acral melanoma that has been present 4 - 5 years by history. Amelanotic acral melanoma are scary lesions as clinically and dermoscopically they do not appear to be worrisome.
It is well-recognized that these can fool practitioners, as they are only rarely seen even by dermatologists and a high index of suspicion is needed. The podiatrist who saw the patient was astute to biopsy the lesion on his first visit.

References:
1. Acral lentiginous melanoma: a clinicoprognostic study of 126 cases.
Phan A, Touzet S, Dalle S, Ronger-Savlé S, Balme B, Thomas L.
Br J Dermatol. 2006 Sep;155(3):561-9.
Department of Dermatology, Hôtel Dieu, Claude Bernard University, 69288 Lyon cedex 02, France.
Abstract:
BACKGROUND: Although the histopathological subtype of melanoma has not been clearly proven to carry independent prognostic significance, acral lentiginous melanoma (ALM) seems to confer a poorer prognosis mainly because disease is often more advanced at the time of diagnosis.
OBJECTIVES: To investigate the distinctive epidemiological and clinical characteristics of ALM, a peculiar histological entity, and to identify prognostic factors.
METHODS: We performed a register-based review of cases from a single large referral centre, the University Hospital Department of Dermatology, Lyons, France. We reviewed patient demographics, the initial presentation of the lesion, and clinical outcome. ALM-specific and disease-free survival were estimated using the KaplanMeier method and compared using the log-rank test. A Cox model was used to identify prognostic factors.
RESULTS: One hundred and twenty-six patients were identified as having histopathology-proven ALM in our melanoma patient register from 1996 to 2004. There were 46 (37%) subungual ALM and 80 (63%) ALM on soles, palms and nonvolar sites. The mean age at diagnosis was 63 years. There were 44 (35%) men and 82 (65%) women, sex ratio M/F 1 : 1.86. The mean Breslow thickness was 2.51 mm (range: in situ to 20 mm). There was no evidence of overexposure to ultraviolet radiation, nor was there found a predisposing genetic trait. Only 16 (13%) patients recalled a history of trauma. Thirty-four ALM (28%) were unpigmented. The median ALM-specific and disease-free survival were 13.5 and 10.1 years, respectively. The 5-year survival rate was 76%. Multivariate analysis identified tumour thickness, male gender and amelanosis as independent clinical prognostic factors for both ALM-specific and disease-free survival.
CONCLUSIONS: Our study provides specific information on the clinical characteristics and outcome of this uncommon histological subtype of melanoma. However, the pathogenesis remains unknown. Breslow thickness, male gender and amelanosis were significantly associated with a poorer prognosis.

2. Acral lentiginous melanoma mimicking benign disease: the Emory experience.
Soon SL, Solomon AR Jr, Papadopoulos D, Murray DR, McAlpine B, Washington CV.
J Am Acad Dermatol. 2003 Feb;48(2):183-8.
Abstract
BACKGROUND: Plantar and subungual melanoma exhibits a higher misdiagnosis rate relative to other anatomic sites. Misdiagnosis and delay in diagnosis are statistically associated with poorer patient outcome. Awareness of atypical presentations of acral melanoma may, thus, be important to decrease misdiagnosis rates and improve patient outcome.
METHODS: We conducted a retrospective case review of plantar or lower-extremity subungual melanoma performed at Winship Cancer Center, a tertiary care, referral center affiliated with Emory University, between 1985 and 2001.
RESULTS: A total of 53 cases of plantar or lower-extremity subungual melanoma were identified. Of 53 cases with a final diagnosis of melanoma, 18 were initially misdiagnosed. Misdiagnoses included wart, callous, fungal disorder, foreign body, crusty lesion, sweat gland condition, blister, nonhealing wound, mole, keratoacanthoma, subungual hematoma, onychomycosis, ingrown toenail, and defective/infected toenail. Of the 18 misdiagnosed cases, 9 were clinically amelanotic.
CONCLUSION: Awareness that amelanotic variants of acral melanoma may assume the morphology of benign hyperkeratotic dermatoses may increase the rate of correct diagnosis and improve patient outcome.

A Complex Patient

The patient is a 61 year-old woman with long-standing insulin-dependent diabetes, rheumatoid arthritis and insulin-dependent diabetes. Her rheumatologist has treated her with methotrexate which she stopped b/c of side-effects. She has also had side-effects (mostly urticaria) with Humira and Remicaide. She was referred for her psoriasis by another dermatologist. Her meds include insulin and prednisone 10 mg per day.

O/E: The patient appears older than her stated age. She appears to have mild facial lipoatrophy. The stigmata of RA is seen in her hands. Her psoriasis is limited to plaques on her back.




Discussion: Given her infirmities and reaction to standard RA and psoriasis meds, I elected to start her on narrow band UVB and clobetasol ointment 0.05% applied after a bath (Soak and Smear protocol).

Questions: Is this real facial lipoatrophy? Is it related to the DM or RA. The patient has not risk factors for HIV or history of abnormal hemograms to suggest immunodeficiency.

Dermatomyositis?

Abstract: 84 yo man with three week history of erythema dorsa hands

HPI: The patient is an 84 yo man who presents with a three week history of a mostly painless eruption on the dorsal hands (left more than right). I have taken care of his skin for over a decade as he's had a thin melanoma and a number of non-melanoma skin cancers. In addition, he has Parkinson's disease and his only medication is carbodopa. Fourteen years ago, he had prostate Ca successfully treated with seeds. Although mentally alert, the patient has been somewhat frail for years and muscle weakness is difficult to evaluate. There are no other skin findings, no heliotrope, no poikioldermatous changes.

O/E: Dusky erythema on dorsum of left hand with a predilection for the MCP joints. Some crusting. Periungual erythema of proximal nail folds, two fingers, right hand.

Clinical Photos (April 4, 2011)




Lab: The patient saw a rheumatologist who did a thorough w/u for collagen vascular disease with a focus on dermatomyositis. All serologies, chemistries and the hemogram were completely normal. CPK was not done.

Impression: The dermatitis is suggestive of dermatomyositis (DM). It's early spring here and he may have been more exposed to light. At this point, I am considering an early and evolving DM. Amyopathic or hypomyopathic DM takes six months to confirm. Considering his age, a thorough evaluation for malignancy might be considered. This appears to be photo-located, so I considered PCT but will hold off on urinary porphyrins for the time being.

Questions: Your thoughts will be appreciated.

Addendum: Two colleagues (Amanda Oakley from NZ and Fran Storrs from Portland, OR, USA) suggested chilblains. I called the patient and asked him if he might have been out more recently without gloves. He told me his hands are usually cold and he has a 200 yard (~ 200 mtr) walk to his mailbox. He hasn't been wearing gloves recently. It's been a cold spring here -- I think chilblains is a more likely diagnosis and I asked him to wear gloves outside when it's < 50 F (10 C) and get back to me in a week. The extend of his involvement is more than we usually see with chilblains, but the dx makes good sense. I'll affix a f/u in a couple of weeks.

Alopecia Universalis

HPI: The patient is a 77-year-old woman who was seen for alopecia, which has been present for about eight months now. This followed chemotherapy for nonhodgkin’s lymphoma. She has a history of alopecia areata decades ago which resolved on its own.

O/E: The examination shows that this patient has alopecia universalis. She has a few eyelashes but no eyebrows, no body hair, no scalp hair.

DX: Alopecia universalis following chemotherapy. This is unusual. There is one report of alopecia universalis following treatment for hepatitis C with ribavirin and interferon.

PLAN: I am going to get a list from her of the medications she was treated with for NHL and see if there are any reports on this. I will also run this be some colleagues.

Question: Has anyone seen a similar patient?

References:

Unusual Nail Bed Tumor

Abstract: 67 yo man with 1 - 2 month hx of a nail bed tumor.

HPI: 67 yo man with 1 - 2 month hx of a nail bed tumor. The overlying nail has been destroyed. Lesion is asymptomatic.

O/E: 8 mm diameter flesh-colored tumor left thumb nail. No pigment. Lesion is solid, not friable.

Clinical Photos


Pathology: Hyperkeratosis, parakeratosis, epidermal hyperplasis. There are ectatic blood vessels in an edematous and fibrotic stroma. Iin the DDx is an unusual traumatized hemangioma or subungual fibroma.

Microscopic Photos courtesy of Deon Wolpowitz, Boston University SkinPath





Diagnosis: Benign nail bed tumor, not otherwise specified. Presented for ideas.

Questions: What is your diagnosis, and how would you approach this lesion?

Plan: The patient has been referred for defintive surgery to a Mohs surgeon.

Dermatitis and Failure to Thrive

Abstract: 3.5 month old male infant with Failure to Thrive and Dermatitis

HPI: This 3.5 mo old infant male is the product of a normal pregnancy and delivery. He has had a severe dermatitis since shortly after birth. The process is most prevalent on head and neck and torso. He has lost weight on 150 kcal/kg per day. On visits seems happy and content. Three older siblings are all normal.

O/E: Widespread scaly patches on trunk and scalp. Thick cradle cap, greasy scale scalp and face. Background erythema. Red excoriated napkin area.

Photos: Taken when child was 9 weeks old.















Labs: Hi K+ and NA on hospital admission for FTT (but since normalized). Mild eosiniophilia and increased plts. Normal: CBC, ZN, IgE, IgM, IgA, IgG, amino acids. Normal karyotype, negative FISH for Williams Syndrome. Complement levels will be done and hair will be looked at for trichorhexis invagninata.

Diagnosis: Unclear. Initially I thought this child had infantile seborrheic dermatitis, but that usually responds well to treatment. At this point, Leiner's and Netherton's syndromes need to be ruled out. While neglect was initially considered, the child's pediatrician feels at this time that the mother is competent and has raised three other normal children.

Discussion: I saw this child once six weeks ago and because of transportation problems they could not keep f/u appointments. The eruption was originally treated with HC valerate 0.2% cream. Scalp hygiene was discussed. At this point a systemic process is considered.

Questions: In addition to considering Netherton's and complement deficiency syndromes like Leiner's what else would you recommend?

Follow-Up: The patient saw a pediatric gastroenterologist who changed her formula to one that excluded all milk proteins -- the eruption cleared completely in a few days and weight gain ensued. This was a milk allergy that masqueraded as a serious underlying disorder.

Brachioradial Pruritus

We continue to get questions from readers -- but many do not leave an email address. If you want specific questions answered, I will try to do so but need an email to respond to. As of March 18, 2012, we've had 68 comments to this post -- and some people ask specific questions. If you want a response, you will have to include your email address. Otherwise, we have no mechanism to reply. We do not share email addresses with anyone. We do not bill anyone for anything.

June 15, 2012.  NEW LINK:  WE HAVE POSTED AN INFORMATION SHEET ON DermatologyCentral.  It's a work in process but should be helpful:  Brachioradial Pruritus Resource Page.

 

Novembver 31, 2024.  The NY Times has an article by Lisa Sanders on BRP: "She Was Scratching Her Arms Raw. Would Anything Stop This Itch?."  I madea pdf.  If you want a copy, email: djelpern@gmail.com .  I also posted a link. to yher NY Times article at this site.
 
I have had an interest in Brachioradial Pruritus (BRP) for over 25 years and published a paper on it in 1985 and co-wrote a chapter on BRP for eMedicine.com. (Actually, the lion's share of the writing was done by Julianne Mann, then a dermatology resident at Oregon Health Sciences University.) As a result of the eMedicine.com chapter, I receive a few requests for information each year. Two came in recently and, with their permission, are presented here. Perhaps, some of our readers have suggestions for management of this annoying and occasionally disabling problem. Note: It is amazing how many comments have been made by people who suffer with BRP. Around 55 at this time. If you wish to leave a comment, and would like us to acknowledge it, please send me your name and email address. It will not appear anywhere on this site; but if you want me to relpy to you I need your contact information. Thank you, DJ Elpern.

1. This is from a 32 y.o. equestrian
I am an otherwise healthy woman desperately searching for a doctor who may be able to discuss some treatment options for my very itchy arms! I am 32 year old, 110 lb, active, female resident of New England and have been suffering with itchy arms (without rash) for at least four years now. When I finally sought treatment- I was referred by my primary MD to a dermatologist who informed me that I had dry, sensitive skin, and prescribed a topical cream and an oral steroid - I carefully followed his advice for 2 years - with absolutely no relief from the itch. The only thing that relieved the symptoms (which flare more intensely in the evening hours) was ice packs. I was often woken from sleep by the itch.

The itch occurs in cyclical pattern- symptomatic for months, followed by a month or two of none or very mild symptoms, then followed by another flare. it is present in sunny months as well as winter.

Finally, frustrated by the unresolved symptoms, at my own expense, I had one visit with a dermatologist in a nearby large city who very quickly diagnosed brachioradial pruritus and prescribed topical Capsaicin. Capsaicin works - sort of. But, it is a total pain in the butt for minimal relief. I am seeking alternative treatment.....acupuncture, chiropractic, anything ... ? Do I need an X-ray? What do I do? I am on my own out here in a sea of medical professionals who seem unwilling to take this condition seriously or look for possible causes that may be an underlying cause for this condition of itchy arms....

It may also be of interest that I have a large amount of muscle/ligament type tension in my neck and shoulders- it's pretty severe, some of this tension is a manifestation of stress, some results from my very physical occupation as an Equestrian.... I am personally tempted to think that all the tension in my neck and shoulders may have something to do with the arm itch - but this conflicts with the cyclical nature of the itch. Has the tension is chronic?
To me, BRP it's a big deal!


2. This is from a 37 yo health care professional:
I have suffered with this for no less than 5 years. Until about a year ago it was sporadic, but has been substantially worse and constant for the last year or so. Nights are particularly difficult, as the itching becomes intolerable and uncontrollable. I have recently found some comfort with ice packs, but naturally having to traipse off to the kitchen several times a night is inconvenient and exhausting. The most recent prescriptions I have tried are 10mg cetirizide for daytime and 25mg doxepin (1-2 at bedtime), and also betamethosone cream. The cetirizide helps with other allergy issues I have, but doesn't offer any relief to the itchy arms. Also, the doxepin helps me get a few solid hours of sleep, if I take 2, but, as with hydroxizine, I feel "hungover" the next morning... AND insatiably hungry.
I do LOVE the sun, and have always found that I feel better through the cold months if I go tanning a couple times a week. In the winter months, I spend a bit of time tanning prior to spending any significant time outside in the summer. I have fair skin, and it helps prevent burning before spending a day at the beach. (which I do, ALL day, as often as possible.) I do not, however notice any increase or decrease in the itching based on exposure to sun/tanning. In the last year, however, I HAVE starting being a bit more careful and use a minimum of SPF 30 during peak hours at the beach. Here is a recent picture:
(Editor's note: This shows some typical findings. The skin looks a bit dry and lichenified and there is evidence of excoriation. Not all cases show these changes, some are more subtle.)


I have seen several doctors, including a dermatologist, regarding this debilitating itch and NOTHING has helped enough to warrant the side effects. I cannot seem to get anyone to understand that it isn't like a skin itch, it feels like its UNDER my skin. I scratch so much, especially at night, that I bleed. I am not a crazy, irrational person.... but the loss of sleep and inability to find anything that relieves this itching probably makes me seem as though I am.

Comment: Both of these patients appear to have chronic BRP. I see a similar patient once or twice a year. While most patients with BRP have episodic, relatively easy to treat disease, there are a few patients who have disabling symptoms. There is an aphorism, "It is often more important to treat the patient who has the disease than the disease the patient has." I think this applies to persons with chronic BRP. In a real way, these are orphan patients. Few dermatologists have the expertise, and fewer the time, to adequately evaluate and treat these patient. If you have suggestions, they would be most appreciated.

Actual Use

Most patients with BRP will get some relief from ice packs.  3M makes a reusable pack thatsells in the U.S. for $2.50.  This is an inexpensive way to start treating BRP if you have not used ice already.  You could use this 2 - 3 times a day for 15 - 20 minutes or whatever works best for you.

ULE?

Abstract: 4 yo girl with 3 week hx of papular eruption left axilla
HPI: This otherwise healthy four year old girl has had a mildly pruritic papular eruption which began in the left axilla with a dermatitic appearance and spread centrifugally where it appeared more papular. There was no antecedent illness. The eruption was first seen on 12/23/2010, cleared after a few days and they reoccurred. The child is not bothered by it. No animals at home and no other family members similarly affected.
O/E: Discrete erythematous papules in the left axilla and surrounding areas. The individual lesions are 3 - 5 mm in diameter. The right axilla and thoracic area are clear. There is left axcillary adenopathy
Clinical Photos: 12/31/2010
Left Axilla

Right Axilla
Lab: none

Diagnosis: Unilateral Laterothoracic Exanthem.

DDx: The lesions look like bites. Against that is the lack of symptoms, the unilateral location, and no likely cause of bites. Contact dermatitis seems unlikely.

Discussion: This is an unusual entity but the child seems well otherwise and I have recommended no treatment for present. I have not seen ULE before (to my knowledge); yet this seems the likely diagnosis. Unfortunately, the pictures in the textbooks are not very good. I suspect, that like Gianotti-Crosti, ULE may be caused by a number of viruses and that it will be difficult to come up with an etiology. The clinical appearance seems to be protean, but the distribution aids in making a diagnosis.

Reference:
1. Emedicine.com has a good chapter, however, they use the name "Asymmetric Periflexural Exanthem of Childhood."

2. McCuaig CC, et. al. Unilateral laterothoracic exanthem. A clinicopathologic study of forty-eight patients. J Am Acad Dermatol. 1996 Jun;34(6):979-84.
Department of Pediatrics, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
Abstract
BACKGROUND: Four years ago, we began seeing young children with an unusual, predominantly unilateral, morbilliform and eczematous, self-limited cutaneous eruption. It appeared to correspond to unilateral laterothoracic exanthem (ULE) reported from France and to an eruption described as "a new papular erythema of childhood" in the United States.
OBJECTIVE: We conducted a prospective study of ULE to define its clinical evolution, pathology, and therapy. In addition, we performed epidemiologic and microbiologic investigations in an attempt to determine the cause of ULE.
METHOD: We studied 48 children with ULE. In some patients, blood, urine, stool, as well as skin biopsy specimens were analyzed.
RESULTS: ULE is a morbilliform, eczematous eruption that often begins close to the axilla and spreads to become bilateral, although it usually retains a unilateral predominance. Patients' mean age at onset is 24.3 months, with a female predominance (2:1) and mean duration of 5 weeks, followed by spontaneous resolution that may or may not be improved with topical corticosteroids. It is characterized by a unique eccrine lymphocytic infiltration. Although signs of infection were reported by most patients, no one infectious agent was identified. No significant epidemiologic factor was found.
CONCLUSION: ULE, in young children, is a self-limited morbilliform and scarlatiniform eruption that may represent a specific skin reaction to one or more infectious agents.

Follow-up Note: The patient's symptoms continued to wax and wane and she was seen by a pediatric dermatologist. At that time, there was just one lesion in the left axilla ( see picture) and a diagnosis of psoriasis was made. There were no other stigmata for psoriasis. If this is the case, the initial picture did not suggest that. Since the sole lesion present now is a plaque in the left axilla, if this turns out difficult to control with topical steroids, consideration to using tacrolimus should be given.





References:
Tacrolimus ointment is effective for psoriasis on the face and intertriginous areas in pediatric patients.
Brune A, Miller DW, Lin P, Cotrim-Russi D, Paller AS. Pediatr Dermatol. 2007 Jan-Feb;24(1):76-80.
Abstract
Children with psoriasis often have involvement of the face and intertriginous areas. While corticosteroids have been the mainstay of treatment for plaque-type psoriasis, the face and intertriginous areas are more sensitive to local effects of topical steroid use such as cutaneous atrophy. Topical tacrolimus has shown promise in adult patients as an alternative antiinflammatory without the cutaneous side effects of steroids. Eleven patients between 6 and 15 years of age with facial or inverse psoriasis were evaluated in a 6-month, single-center, open-label trial. Clinical evaluations were made at baseline and days 30, 90, and 180. Severity was assessed using the physician's global assessment of improvement relative to baseline, a 6-point rating scale for signs of disease (erythema, infiltration, desquamation), and an overall severity score. Within the first 30 days of treatment, every patient had cleared or achieved excellent improvement with the use of tacrolimus ointment. Statistically significant improvement was achieved in each sign of disease and the overall severity score. The only adverse event reported in 6 months of observation was significant pruritus in one patient. We therefore conclude that tacrolimus ointment is an effective treatment for psoriasis on the face or intertriginous areas in children.

Dermatomal Eruption

Abstract: 39 yo man with two month history of dermatomal eruption.
HPI: This 39 yo man developed a dermatomal vesicular eruption 2 months ago. He was seen by his GP and treated with valcyclovir and it cleared somewhat but not completely. The eruption continues to evolve. He complains of pain and pruritus. Feels well otherwise. No underlying diseases known of.
O/E: There is a dermatomal process extending from T-10 to L 2 on the right side. The lesions are scaly patches. There are no vesicles. The lesions do not cross the mid-line. Area biopsied today is only a couple of days old, by history.
Photos:





























Diagnosis: Atypical Herpes Zoster. H.z. progressing over a two month period (especially after valcyclovir) is quite unusual in a healthy, immunocompetent person.

Plan: I did a biopsy and checked his chemistries and CBC. I have seen patients with HIV/AIDS and a patient with angioimmunoblastic lymphadenopathy with atypical HSV and HZ; but never a patient like this. Perhaps, this is something I am not thinking about. I will post a photomic when path is reported and lab results. For the time being, I prescribed acyclovir 800 mg 5 times a day.

Addendum: Fran Storrs felt this was an eczematous process, possibly a contact dermatitis. The pathology showed no multinucleated giant cells and had features of a "dermatitis." So, was this a dermatitis secondary to H.Z., an atypical contact dermatitis, or factitial (the patient did ask for pain meds when first seen, which were not given)? He is now being treated with a topical corticosteroid now and we'll see how he does. When seen for suture removal sight days after biopsy, the eruption looked a bit better, was less symptomatic and had not spread beyond the dermatomes first involved.

The Power of BLINCK

Presented by Yoon Cohen MS IV, University of New England, Biddeford, Maine and David Elpern MD, Williamstown, Massachusetts

Abstract: 68 yo woman with 4-6 months history of an atypical melanocytic lesion.

HPI: This healthy 68 yo woman with type II skin presented to the clinic with 4-6 months history of an atypical melanocytic lesion on the left knee. She had noticed an increase in size and change in color and was concerned about these changes in the lesion.

O/E: There was a 7 mm in a diameter asymmetrical brownish macule on the left knee. The lesion showed an irregular border with varied colors.

Clinical photographs:

Dermoscopic images:

Microscopic images:

Dermatopathology report:

The specimen exhibits a proliferation of moderate to severely atypical melanocytes distribubted in irregular nests, as well as singly at and above the dermal epidermal junction, with pagetoid spread to the granular layer and near confluence over at least three rete ridges. These findings support the histologic diagnosis of melanoma-in-situ.

Our appreciation to Dr. Deon Wolpowitz, MD from Boston Univeresity, Dermatopathology, for providing these photomicrographs for the case.

4X

10X

20X

20X

Diagnosis:

Malignant melanoma in situ

Discussion:

The BLINCK approach:

We followed the BLINCK checklist, introduced by Dr. Peter Bourne, a founder of the Skin Cancer College of Australia and New Zealand (SCCANZ). The score for the lesion was added up to 4 by criteria as following.

1. B. The lesion was not clearly benign at our first initial evaluation

2. L. The lesion appeared to be lonely without any other similar melanocytic lesion near by

3. I. The lesion appeared to be irregular outline and color on our dermoscopic exam

4. N & C. The patient was nervous about the change in color in past 4-6 months

5. K. The lesion exhibited known clues when viewed with a dermatoscope. See "Chaos and Clues" reference below.
BLINCK Score = 4

According to the BLINCK approach, a lesion should be biopsied if the BLINCK score is 2 or more out of a possible 4. Therefore, the we excised this lesion and sent for a pathologic evaluation.

According to Dr. Bourne, the BLINCK approach is presented as a simple method to assist the clinician with the decision of whether to biopsy a skin lesion or not. The use of this algorithm will improve the pickup rate of potentially serious skin cancers as well as reduce the number of unnecessary benign lesion excisions. BLINCK may be especially helpful to clinicians who have only basic or intermediate dermoscopy skills but who are regularly called upon to assess skin lesions in their practices.

Questions:

1. Would you consider using the BLINCK approach at your practice?

2. If you already have adapted the BLINCK approach, how have your experiences been?

References:

1. McColl I. BLINCK. http://idsblinck.blogspot.com/2009/11/blinck.html. Updated November 19, 2009. Accessed September 7, 2010

2. Rosendahl C, Kittler H, Cameron A, et al. CHAOS & CLUES - The Algorithm. http://www.chaosandclues.blogspot.com. Updated November 19, 2009. Accessed September 7, 2010

Palmoplantar Erythrodysesthesia Syndrome

Presented by Yoon Cohen, MS IV
University of New England College of Osteopathic Medicine

Abstract: 45 yo woman with a metastatic breast cancer and a painful hand/foot dermatitis.

HPI: This is a 45 yo woman who was diagnosed with a metastatic breast cancer a few years ago. She has developed "atopic dermatitis" like symptoms on her hands and feet since starting capecitabine. She has completed 5 cycles of Xeloda (capecitabine) and has had to reduce the dose because of this condition. The lesions started as dry edematous, erythematous areas on palms and soles with a tingling sensation.

O/E: The examination reveals general areas of desquamation and hyperlinearity with mild erythema on both palms. This is best shown on the first photograph. 

Clinical photographs:

Diagnosis: Palmoplantar erythrodysesthesia syndrome, hand-foot syndrome, chemotherapy-induced acral erythema

Discussion:

Chemotherapy-induced acral erythema or palmoplantar erythrodysesthesia syndrome is a well-defined reaction to some of the chemotherapeutic agents such as methotrexate, cytarabine, doxorubicin, fluorouracil, cytosine arabinoside, and bleomycin. This reaction is characterized by symmetric, well-demarcated, painful erythema of the palms and soles, which may progress to desquamation or blisters. It appears to be dose dependent, and is likely a direct toxic effect of the drug. Tingling on the palms and soles is followed in a few days by painful, symmetric, well-defined swelling and erythema [4].

Questions:

1. Please feel free to share your experiences with treatment options.

References:

1. Marini A, Hengge UR. Hand-foot syndrome with capecitabine therapy. Hautarzt. 2007 June; 58(6):532-6

Abstract: A 72-year-old patient with esophageal carcinoma developed a severe hand-foot syndrome during second-line therapy with the oral fluoropyrimidine capecitabine. We also summarize the current knowledge with regard to the hand-foot syndrome and distinguish it from palmoplantar erythrodysesthesia.

2. Degen A, Alter M, Satzger I, et al. The hand-foot-syndrome associated with medical tumor therapy- classification and management. J Dtsch Dermatol Ges. 2010 Sep; 8(9):652-61

Abstract: The hand-foot-syndrome (HFS, palmoplantar erythrodysesthesia, chemotherapy-associated acral erythema) is characterized by painful predominantly palmo-plantar lesions. The association with different chemotherapeutic agents has been known for over 20 years. More recently, HFS has been reported in association with regimens using targeted agents, in particular the multikinase inhibitors (MKI) sorafenib and sunitinib. The HFS associated with MKI has a different distribution and clinical appearance than the traditional disorder. In this review, similarities and differences between chemotherapy- and MKI-associated HFS are discussed and current recommendations for their prophylaxis and management are summarized.

3. Janusch M, Fischer M, Marsch WCh, et al. The hand-foot syndrome - a frequent secondary manifestation in antineoplastic chemotherapy. Eur J Dermatol. 2006 Sep-Oct; 16(5): 494-9

Abstract: The hand-foot syndrome (HFS) (palmoplantar erythrodysesthesia) designates acute, painful erythemas of the palms and soles of the feet caused by antineoplastic chemotherapies. The most frequent trigger substances are 5-fluoruracil and its derivates. At maximum severity, the HFS is bullous to erosive or ulcerous in character. The pathogenesis has not yet been clarified. Histologically, the HFS is characterized by a toxic keratinocyte reaction. Furthermore, there is sub-basal edema with a tendency to bullae, dilated blood and lymph capillaries and usually only mild perivascular lymphocytic infiltration. Early recognition and delineation from other differential diagnoses is prerequisite to targeted management of the disease. Depending on the severity, HFS requires dose reduction, interruption or switch in the antineoplastic chemotherapy. (You can access to the article at http://www.john-libbey-eurotext.fr/e-docs/00/04/26/D7/vers_alt/VersionPDF.pdf)

4. Habif TP. Clinical Dermatology. 5th edition. USA: Elsevier Science 2010

Keratolysis exfoliativa

HPI: The patient is a 27 yo medical assistant who was seen on August 27, 2010 with a six day history of slightly pruritic scaling of the palms.  She is well otherwise and has had no recent illnesses.

O/E: The examination reveals discrete areas of desquamation on both palms.  No vesicles. Soles are normal and remainder of cutaneous exam is unremarkable.

Photos:

Diagnosis: The clinical picture is consistent with Keratolysis exfoliativa.  Strangely, PubMed has only three references to this relatively common disorder, and only one is helpful (see below).  Most dermatologists are familiar with this entity.  There's a brief description on DermNet.

A throat culture taken to r/o post-streptococcal desquamation of the palms was negative.

Refererence:
Recurrent focal palmar peeling.
Lee YC, Rycroft RJ, White IR, McFadden JP.
Australas J Dermatol. 1996 Aug;37(3):143-4.
St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
Abstract
Recurrent focal palmar peeling, previously known as keratolysis exfoliativa, is an idiopathic condition characterized by chronic palmar and occasionally plantar peeling. It can be exacerbated by environmental factors, and may be misdiagnosed as chronic contact dermatitis. Accurate diagnosis is from the history and examination. It is supported by a negative patch test result. Three cases of recurrent focal palmar peeling are presented, of which two were misdiagnosed as chronic dermatitis. Although there are few references on recurrent focal palmar peeling, it is likely to be a common condition that rarely presents to dermatologists because it is largely asymptomatic. A correct diagnosis is essential due to the social, occupational and legal implications if misdiagnosed.