Keratosis Follicularis

Presented by DJ Elpern
Photomicrographs by Jag Bhawan

Abstract: 10 yo girl with 4 month history of a dermatosis on the neck

HPI: The patient is a pleasant 10 year old who presents for evaluation of a symmetrical papular eruption on the sides of the neck which has been present for about 4-5 months. She has been treated with a number of different topicals by her pediatrician without relief. The patient lives with a grandmother and there is no pertinent family history.

O/E: The examination show somewhat rough, 1 - 2 mm in diameter, keratotic micropapules on the lower folds of the neck. The remainder of the cutaneous examination is unremarkable.

Clinical Photos:

Biopsy: Focal acantholysis, multiple dyskeratotic cells, corps ronds and grains consistent with Darier's disease.

10 x and 20 x

40 x

Diagnosis: Keratosis follicularis (Darier's disease). It's unusual to see keratosis follicularis when it first appears. This is a sweet 10 year old and it's sad to contemplate what this may turn into. After "reviewing the literature" I decided to try pimecrolimus cream as there have been some reports of success.

Questions: How would you treat this child? Have you seen forme-frustes of keratosis follicularis?

References:

1. Good Overview: Darier's Disease eMedicine

2. Pérez-Carmona L, et. al. Successful treatment of Darier's disease with topical pimecrolimus. Eur J Dermatol. 2011 Mar-Apr;21(2):301-2.

3. (supplied by Yoon Cohen) Rubegni P, Poggiali S, Sbano P, Risulo M, Fimiani M. A case of Darier's disease successfully treated with topical tacrolimus. J Eur Acad Dermatol Venereol. 2006 Jan;20(1):84-7.
Abstract: Tacrolimus is a macrolide that inhibits T-cell activation. The most extensive experience with topical tacrolimus has been in treating atopic dermatitis but it has been used in various skin diseases, including Hailey-Hailey disease, with encouraging results. We report a case of extensive Darier's disease successfully treated with topical tacrolimus, after suspension of oral isotretrinoin due to major depression.

Facial flush in a pregnant woman

Presented by Henry Foong

Ipoh, Malaysia

A 37 year old restaurant waitress had these rashes on the face for several years, but worse recently since her pregnancy. She is G2P1 at the end of her first trimester. The rash was described as itching, burning. She had seen a dermatologist in Japan and was diagnosed as rosacea. There was no fever or polyarthralgia. Family history was insignificant. Drug history nil.

She feels very uncomfortable. Examination was unremarkable except facial flushing with for bilateral and symmetrical erythematous papules on both cheeks with a mild involvement of the bridge of nose. There was no comedones. Her scalp was normal.
What do you think of the diagnosis? Do you think this is rosacea? What other differentials would you consider - lupus erythematosus, seborrheic dermatitis? How would you manage her remembering that she 3 months pregnant? Would you use topical metrondazole?

Neurotic Excoriations

Abstract: 37 yo woman with few year history of excoriations

HPI: The patient is a disabled 37 yo woman who has suffered with painful sores on face, arms, buttocks, upper back for a few years. She was a trainer of race horses till a few years ago. She has a history of alcoholism. A few years back, she was diagnosed with alcoholic hepatitis and hemochromatosis (she gets regular phlebotomies). There is a history of sexual abuse starting at age 14 or 15 which continued for ten years. Thereafter she was in a physically abusive relationship. She is on a Fentanyl patch.

O/E: Fresh and healing excoriations on face, upper back, left earlobe, buttocks.


Diagnosis: Neurotic Excotiations (NE).

Discussion: In my experience, most women who excoriate their faces and bodies in this way have experienced sexual or physical abuse. This is similar to "cutting behavior." NE may be a minor varient of cutting. Adverse Childhood Experiences can manifest themselves in this kind of self-destructive behavior. Her alcoholism may be another expression.

Treatment: These patients are very diffuclt to reach. I started with clobetasol ointment and mupirocin ointment -- these are sometime helpful although one has to be careful not to use for too long on the face. Psychotrophic medications may be helpful. Cognitive behavioral therapy can help but is rarely available for poor patients. The ones who need it the most are those least likely to find a therapist who will help. Patients with NE like this woman are very needy. It can take months to reach them.

Painful Red Scrotum

Over the past twenty years, we have seen a few patients a year with scrotal burning and/or redness (erythema). Some of these individuals had used topical steroids for prolonged periods, some only for a few weeks. I don't recall if any had not used steroid creams. The condition is called scrotodynia, scrotopyrosis, and red scrotum syndrome. The medical literature gives few clues to its etiology, except that topical steroids can play a significant role in some (or many) of these patients. There is a condition called "vulvodynia" which is similar in some ways. This post tells one patient's story and is a call for information from physicians, other care givers and, importantly, from individuals who suffer with this disorder. It is anonymous. Hopefully as practitioners and patients collaborate we will reach some clarity and start to help those who suffer. If you are a patient making a comment please give your age, occupation and any other information you may consider pertinent.

Patient's History: (November 2011)
I am a health 46-year-old man in the technology field who has suffered with a burning scrotum for past two months. I had knee surgery in May of 2011 which sidelined me from physical activity until September of this year. Upon resuming a workout regiment (primarily of basketball and running) I developed what was diagnosed as a fungal in my groin (tinea cruris - commonly referred to as “jock itch”), specifically in the creases of my thighs. The red scrotum seemed to appear along with the fungal issue, but being unfamiliar with tinea cruris (it was my first time with the condition) I assumed that the red scrotum was part of the same problem. My first attempt at resolving the issue came with a visit to a dermatologist (who I happened to be seeing for a minor skin condition on my hands). It was a “by-the-way can you prescribe something for this rash I have” which first turned our/my attention to the red scrotum.

Initially the dermatologist prescribed Hydrocortisone ointment USP 2.5% for the redness/inflamation and Ketoconazole cream 2% for the fungal issue. The instructions were to first apply the Hydrocortisone to the inflamed area (the creases of my legs were rather red with a fungal rash) for one week to reduce the inflammation. Then apply the Ketoconazole for one week and return for evaluation. I applied the Hydrocortisone to the creases of my thighs and to my inflamed scrotum. The redness in the creases of my thighs subsided marginally but there was no change to the red scrotum. I then applied the Ketoconazole for one week and did see relief of the jock itch. Upon my return to the dermatologist I reported that the fungal treatment was working but there was no change in my scrotum. It was here where I first heard the term “Red Scrotum Syndrome” as a possible diagnosis. I was then prescribed Triamcinalone Acetonide ointment USP 0.1% (a topical steroid) and instructed to apply it to the scrotum for one more week, twice daily, (which I did) and report back. After one week of applying the Triamcinalone ointment to my scrotum there was no change in my condition. I was told by the dermatologist that she was out of ideas and to report to my primary care physician for further treatment.

The visit to my Primary Care Physician began with a careful review of the notes from my dermatologist coupled with a detailed description of what was happening by me. Upon examination my PCP admitted that he had never seen a case like this before. He stated that his medical references offered little help but he did find some info by doing an internet search. The research suggested a treatment of Doxycycline (an antibiotic) 100 mg, twice per day for 10 days. I promptly began taking the oral dose of Doxycycline but after 10 days again there was no change in my condition.

During my initial visit with my PCP I asked if I should stop using the Ketoconazole even though there was still remnants of the tinea cruris. The doctor said to stop all ointment treatment to the groin and instead take an oral anti-fungal medicine to kill the jock itch once and for all. Not knowing the dosage his office requested advice from another dermatologist who upon contacting prescribed Fluconazole (one pill one time). I have taken the Fluconazole and coupled with the Ketoconazole I seem to have the tinea cruris under control.

Next my PCP referred me to a urologist who, like my first Dermatologist and Primary Care Physician, admitted that he had never seen this condition before. He checked my prostate (normal) and gave me a urine test (which also came back normal). The urologist wished me luck and apologized for not being more helpful.

It is here where my luck changed as the second Dermatologist recognized the symptoms and suggested I pay him a visit. Upon examination he too diagnosed the condition as Red Scrotum Syndrome (RSS) or in some circles known as “Great Balls of Fire”. He knew of two doctors (one in Boston and one in Sweden) that have had experience with RSS. Pictures and a description were emailed to each and we await feedback. From prior cases and research the dermatologist advised me to take gabapentin (300 mg 3 times per day). Gabapentin was originally developed for the treatment of epilepsy, and currently is also used to relieve neuropathic pain. I am on day three of the medication and I do not feel any change in the condition.

Hopefully some relief is in sight as the pain is annoying. Some days are significantly worse than others. In fact on some days I continue my normal family and work routine and barely notice the RSS. On other days it’s more pronounced and sitting for any length of time at my desk is uncomfortable. Walking and sitting seem to aggravate the sensation. Having had the shingles (Herpes zoster) at the age of 44 I liken the pain to having scrotal shingles. Perhaps there is something neurological in the equation because I’ve been told that 44 years old is unusually young for shingles. A final note is that high levels of stress (mostly caused by work) occurred during my shingles and when the RSS manifested. A psychological component to the condition cannot be ruled out.

Unfortunately I’ve been told that I am what the medical field calls an “orphan” patient. That RSS exists in a medical space between Dermatology and Neurology and neither discipline is really focused on the condition. I know there are others out there who are suffering with the same pain and that have possibly found a solution to this annoying problem. Hopefully, this post is seen by others, offers helpful information and lets them know that they are not alone. I also hope that any sufferers out there who have had Red Scrotum Syndrome and discovered a remedy reply back and give us a helpful start.

References:
1. Gabapentin for Neuropathic Pain

2.  Wollina U.  Red scrotum syndrome.  J Dermatol Case Rep. 2011 Sep 21;5(3):38-41.  Red Scrotum Free Open Access

20 y.o. man with multisystem disease

Presented by Henry B.B. Foong
Foong Skin Clinic, Ipoh, Malaysia

Abstract: 20 yo man with mouth ulcers, arthralgias, skin nodules

HPI: The patient is a 20 yr old student who presented with a 3 year history of recurrent mouth ulcers, polyarthralgia (knee, ankles), fever and tender nodules over the shoulders, elbows and legs. The attacks occur about every 6 months and responded to oral prednisolone. Apparently the nodules run a predictable course - initial erythema, then tender nodule then ulcerate and then subside leaving behind post inflammatory hyperpigmentation – all over 3-4 weeks. There is no photosensitivity, alopecia or cough. There is a family history of similar illness.

O/E: Multiple erythematous tender nodules over the elbows, legs , upper shoulders and scrotum. Those on the scrotum – severe, multiple tender nodules, of which ulcerated with scab formation. Multiple tender ulcers were also noted on the inner mouth.

Clinical Photos: (taken with iPhone)


LAB: (Some pending)
TWBC 11, 700 (N 67% L 18% E 1% M 12% B1%) ESR 44 mm/hr
ANA
ANCA
Mycoplasma serology 1: 160 ( N<1:40)
LFT and renal normal
CXR

Pathology: Pending

Dignosis: Behcet’s? PAN? SLE?

Questions: What are your thoughts? Any further studies indicated?

Tumor in Vaccination Site

Abstract: 59 yo woman with six month history of tumor l. arm
HPI: The patient, a kindergarten teacher, was bitten on the hand by a child on March 20, 2011. School policy did not allow the child to be tested for hepatitis or HIV. Therefore, it was recommended that she receive hepatitis B vaccination. She had three shots ( March, June and December 2010) in the left deltoid area. In late January or early February 2011 she developed a tumor at the site of the vaccination.
O/E: There is a 1.2 cm. slightly friable tumor in the above-mentioned area. Dermoscopic exam shows some arborizing blood vessels.

Clinical Photograph:

Pathology: Basal Cell Carcinoma: Nodular and Infiltrating. No epidermal connection is apparent in submitted specimens.

Diagnosis: Basal Cell Carcinoma in Vaccination site.

Discussion: There have been sporadic reports of skin cancer developing at the sites of vaccination, but never one in a hepatitis B site. The latent period here is short. It's unclear what the initiating factor is. Our patient is a light-complected Caucasian, so has another risk factor, too. We plan to investigate this area further and present a case report with a review of the literature. Your thoughts will be helpful.

Temple Bracelet Dermatitis

Abstract: 20 yo woman with allergic contact dermatitis to a bracelet purchased at a temple in Beijing

HPI: This young woman purchased some prayer beads at a Beijing temple in early September, 2011. Within two - three weeks she developed a rash under the bracelet. She treated this with a number of topicals including a neomycin containing cream. She was seen in an ER a few days before she presented to my office and started on prednisone and Keflex.

O/E: A well-defined area of resolving dermatitis on left wrist. It appears to have been bullous.

Clinical Photos:
The clinical picture did not come out well. Second picture is of bracelet on unaffected wrist to show how she wore it.











Diagnosis and Discussion: This contact dermatitis is most likely secondary to wooden beads. There are a few pertinent references (see below). The patient also applied neosporin so we can't rule out that this may have played a role. She does not live near my office and is in college far away. My approach would be to treat with a topical corticosteroid and warn her about neomycin. If this recurs she can be patch tested. At this point, I do not know what kind of wood the bracelet is made of. The references I found were mostly about cocobolo wood. This may prove difficult to determine. Your comments will be welcome. Note: A number of our readers favor rosewood as the culprit (see reference # 3)

References:
1. Hausen BM. Allergic contact dermatitis from a wooden necklace. Am J Contact Dermat. 1997 Sep;8(3):185-7.
Abstract
A 36-year-old female kitchenworker twice developed eczematous lesions corresponding exactly to the area around her neck where she had worn a wooden necklace. Contact dermatitis lasted longer than 1 week. The necklace consisted of 42 brown wooden beads and 63 other wooden parts, 0.5 to 3 cm diameter. Most parts could be identified as Cocobolo wood, Brazilian and East Indian rosewood, and teak. Patch tests with the pure constituents gave +3-reactions to three dalbergions and obtusaquinone, which are known to be the sensitizers of Cocobolo and the above-mentioned rosewoods. Because of these test results, the identification of the species by eye examination could be corroborated. Further detailed questioning revealed that the patient had played a recorder, probably made from Cocobolo (Dalbergia retusa), when a child, to which she unknowingly became allergic.


2. Moratinos MM, Tevar E, Conde-Salazar L. Contact allergy to a cocobolo bracelet. Dermatitis. 2005 Sep;16(3):139-41.
Abstract
Tropical woods are highly valued because of their strength, hardness, and resistance to moisture. These characteristics make them easy to work with and extremely durable, and that is why they have been used in the manufacture of wooden jewelry, musical instruments, furniture, and handles of many different objects. We present a case of a 44-year-old man who developed pruritus, erythema, and blistering around his right wrist, corresponding exactly to the area where he had worn a wooden bracelet. Thin-layer chromatography performed with the extract of the shavings revealed (R)-4-methoxydalbergione and obtusaquinone (the main components of cocobolo wood) and (S)-4'-hydroxy-4-methoxydalbergione (in lower amounts). Patch-testing with sawdust from the bracelet resulted in a very strong reaction. Patch tests with the pure constituents yielded +++ reactions to the main sensitizers of cocobolo, including obtusaquinone, but also to sensitizers present in other rosewoods. This last fact can be explained by cross-reactivity between different dalbergiones. Contact dermatitis from tropical woods is more frequent than thought, owing to their high sensitizing properties. An exhaustive search can identify the allergen responsible in many cases.

3. Hausen BM. [Rosewood allergy due to an arm bracelet and a recorder]. Derm Beruf Umwelt. 1982;30(6):189-92. [Article in German]
Abstract
A 40-year-old woman developed dermatitis of the left forearm after wearing a bracelet manufactured from Brazilian rosewood (Dalbergia nigra All.). Swelling of the lips, itching and vesicles recurred when she played a recorder made from the same timber some years later. Epicutaneous tests were strongly positive after 120 h with 2 of the wood constituents: R-4-methoxydalbergione and S-4,4'-dimethoxydalbergione. The third quinone (S-4'-hydroxy-4-methoxydalbergione) only elicited a weak reaction. Shavings of the wooden bracelet extracted with benzene and ethanol and separation of the residues by thin layer chromatography yielded all 3 dalbergiones in remarkable amounts (congruent to 0,8%). Cross-reactions to the chemically near related R-3,4-dimethoxydalbergione, known as the strongest sensitiser of the dalbergione group, were not obtained, although guinea pig experiments had revealed cross-reactivities. Of the racemic

Lupus Erythematosus?

presented by Henry Foong:

A case in progress... 14 year-old Malaysian boy with photosensitive eruption


HPI:The patient is a 14 year old student with one year history of erythematous patches on the face, made worse with sun exposure. He is otherwise healthy with no systemic complaints. He has been on no medications byh mouth.

Examination showed few discrete 2-4 cm erythematous plaques on the cheeks, nose, upper and lower eyelids. No alopecia. No scarring.

I Suspect this is lupus erythematous. Acute LE or Subacute LE ? ANA serology and biopsy done. Results pending. Differentials - Jessner's lymphocytic infiltrates

Questions: What are your thoughts pending the biopsy findings? Would you do anything different from what I have done so far?

Painful Brusing in a 29 yo Woman

Presented by Hamish Dunwoodie, MBBS
Moncton, New Brunswick, Canada

Abstract: 29 yo woman with one week history of painful bruising on thighs

HPI: The patient is an otherwise healthy 29 yo woman with a one week history of painful bruises on her thighs. Five years ago she had leucocytoclastic vasculitis of her lower legs and very mild proteinuria. A renal consult felt she probably had mild IgA nephropathy. This has cleared. Her only medication is paroxetine, which she has been on for three months. She denies any trauma. The patient is a single mother of two children (11 and 3 years old) and lives alone with her kids. She was in school recently but is now on disability for "seizures" (although she is on no antiepileptic medications at present). She has been assaulted by a boyfriend in the past, but denies trauma this time.

O/E: There is purpura of the lateral thighs bilaterally. No evidence of LCV any longer. The remainder of the cutaneous examination is unremarkable.

Clinical Photo:


Lab: CBC, Chemistries, Urine Analysis all normal save for trace + rbcs. No proteinuria any longer.

Diagnosis: This is most likely traumatic purpura in a young woman who is reluctant todivulge an accurate history. Gardner Diamond Syndrome (autoerythrocyte sensitization syndrome, psychogenic purpura) was considered as well.

Questions: What are your thoughts?

References: (Full Text Online)
1. Gardner-Diamond syndrome: Difficulties in the management of patients with unexplained medical symptoms. Meeder R, Bannister S. Paediatr Child Health. 2006 Sep;11(7):416-9.

2. Gardner-Diamond Syndrome: bruising feeling. Bostwick JM, Imig MW. Mayo Clin Proc. 2008 May;83(5):572. (This is a short article)

Traumatic Ulcer

Abstract: 40 year-old man with non-healing wound

HPI: The patient is a 40 yo man who sustained traumatic abrasions of his leg and arm from a motorcycle accident on May 31, 2011. He has a history of chronic vesicular dermatitis of hands and feet complicated by recurrent staphyloccal cellulitis of legs. The wound on his right knee became infected and he was hospitalized over the summer on two occasions for parenteral antibiotics and debridement. As a result of this wound he has lot his job and his family is living marginally.

O/E: September 14, 2011. There is a nine cm relatively clean ulcer over the right knee. It has shown no tendancy to heal over the past month.

Clinical Photograph:

Diagnosis: Ulcer right knee.

Questions: How would you approach this lesion so that the patient can heal and get back to work? At present, he is getting dressing changes a few times a week and there are no plans for further surgical interventions. It looks like this will take months to heal by secondary intention.

Follow-Up: 10/19/2011 I have seen the patient on two occasions since this posting. The ulcer is ~ 75% better with just daily dressing changes with Vaseline impregnated gauze. He has not needed any further antibiotics. I expect it will be completely re-epitheliazed in two to three weeks.

Insect Bite Lymphangitis

Presented by Nai-Chien Yeat
Williams College, Williamstown, Massachusetts

Abstract: 20 year-old Malaysian college student with one day history of an itchy line on right arm.

Yeat's History:
I developed itchy welts all over my body shortly after moving into my new dorm room. A bite on my right wrist caused extensive swelling and intense itching within 24 hours of first discovery. Within 36 hours, a swollen, pruritic red streak extended from my wrist to my upper arm.
A bite on my left ring finger caused extensive swelling and intense itching within 24 hours. Within 36 hours, the swelling and itching had spread to the back of my hand.
After bumping into Dr. Elpern on the street, I started a course of antibiotics (Augmentin) and took antihistamines (Clarityne and Benadryl) to relieve the pruritus.

O/E: (DJE) I bumped into Mr. Yeat on Sunday morning, September 4th on the Williams College campus and he showed me his hands and arms. There were erythematous papules with some superficial crusts on the hands and a lymphangitic streak on the volar right arm extending towards the elbow. Other than pruritus, he felt well and had no fever.

Clinical Photos taken by Mr. Yeat





















Diagnosis: Although initially I was concerned about a bacterial lymphangitis, I now think this is most consistent with lymphangitis secondary to insect bite rather than a sign of a bacterial etiology. Yeat knows the initial lesions are bites and he feels well otherwise. I suppose a bite could have been superinfected with strep, so the Augmentin makes sense; but it could also be based on another mechanism. There are a few pertinent references including one from the BMJ which Mr. Yeat found (# 2). I am not convinced this is from bedbugs as many types of arthropod bites apparently can cause lymphangitis. It's curious that so few cases have been reported. This may be because the patients appear to have a bacterial process, are treated with antibiotics and get better as they would over a few days even without the medications. It would be important to know if bed bugs have been found in his dormitory.

(Note from Yeat one week after onset: "The swelling has completely subsided, and you can barely see the red streak that the lymphangitis left behind."

Questions: Mr. Yeat and I will appreciate your thoughts. Do you feel the Augmentin was necessary? Have any of you seen similar cases?

Reference: Superficial lymphangitis after arthropod bite: a distinctive but underrecognized entity?
1. Marque M, Girard C, Guillot B, Bessis D. myriammarque@yahoo.fr
Dermatology. 2008;217(3):262-7. Epub 2008 Aug 6.
Abstract
BACKGROUND: Acute bacterial lymphangitis is a common occurrence after skin damage. This diagnosis is often made in case of red linear streaks after arthropod bites, leading to the prescription of oral antibiotics. In this setting, noninfectious superficial lymphangitis after arthropod bites, an eruption rarely mentioned in the medical literature, appears as a diagnostic challenge.
OBJECTIVE: Our purpose was to study the clinical and histopathological features of this underrecognized condition.
METHODS: We collected the observations of six consecutive patients seen between the years 2003 and 2006, who developed an acute linear erythematous eruption along lymphatic vessels, mimicking common bacterial lymphangitis. Standard histological examinations were completed by immunopathological staining using the monoclonal antibody D2-40, a highly selective marker of lymphatic endothelium. Extensive review of the literature about acute noninfectious superficial lymphangitis was performed. Results: The clinical presentation and histological findings excluded an infectious etiology and suggested superficial lymphangitis after an arthropod bite in all the observations.
CONCLUSIONS: This article analyzes the clinical and histological features of noninfectious superficial lymphangitis after arthropod bite, a benign underrecognized condition mimicking common bacterial lymphangitis. Physicians should be aware of this benign reaction to avoid the useless prescription of antibiotics.

2. BMJ Case Reports 2010; doi:10.1136/bcr.09.2010.3310
Acute superficial lymphangitis following pigeon mite bite
Parvaiz A Koul, Syed Mudassir Qadri Full Text Online.

Erythema multiforme major

Presented by: Dr. Henry Foong
Ipoh, Malaysia

Abstract: Five Year-old boy with E. multiforme

The patient is a 5 year old boy presented with 3 day history of fever and generalised skin eruptions. Apparently it started with superficial lower lip erosion and the next day he had high fever and generalised skin eruptions on trunk, the upper and lower extremities. There was no family history of similar skin problems.

O/E he was afebrile. Generalised erythematous macules and plaques were noted on the face, trunk and extremities. The lesions were distributed acrally. Some of the macules had sharp margin round shape with concentric rings within it. A vesicle was noted on the centre of the macules. Few typical round macules were noted on the palms and soles. Clinically he has erythema multiforme major

TWBC 14, 900 (N11.4% L75.4% E2%) ESR 19. Mycoplasma antibody is negative. He is now empirically on oral acyclovir and oral clarithromycin.

The most likely cause of the EM is HSV infection in this patient. Wonder if you would use systemic corticosteroids in this patient?

Raccoon Purpura

I received this email from an otherwise healthy 23 yo woman who I saw a month ago for an unrelated problem: "I was wondering if you might have any insight to another skin problem I am having. After receiving some terrible news, I have popped a number of blood vessels around my eyes and face to the the point of having dark purple bruises around and on my eyes. I do not know what to do. I look like a victim of abuse and would like to heal my face as soon as possible."

Discussion: One can see eyelid purpura and petechiae with a number of pathologic processes (amyloidosis, coagulopathy) but also after valsalva maneuver, violent vomiting, coughing. I suspect the latter and need more information from the patient. Any thoughts?

Reference: Anesth Analg. 2007 Dec;105(6):1561-3, table of contents.
Periorbital ecchymoses during general anesthesia in a patient with primary amyloidosis: a harbinger for bleeding? Available Free Full Text Online
Weingarten TN, Hall BA, Richardson BF, Hofer RE, Sprung J.
Source
Department of Anesthesiology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Abstract: Primary amyloidosis is a result of proliferation of a population of plasma cells that leads to an increased secretion of monoclonal immunoglobulins (amyloid). Amyloid protein infiltrates increase capillary fragility. Such capillaries can burst, even after minor stress, resulting in periorbital hemorrhage. We describe a 64-yr-old man with primary amyloidosis who underwent general anesthesia. His eyes were gently closed with tape. Upon removal of the tape bilateral periorbital purpura was noted. All coagulation studies were normal. The periorbital hemorrhage was attributed to amyloidosis-induced capillary fragility.

Post-Operative Contact Dermatitis

Abstract: 63 yo woman with 5 day history of a dermatitis

HPI: A 63 yo woman developed a dermatitis 2 d post surgery. An arterial line had been placed in the L. radial artery pre-op. The area was first prepped with chlorhexidine, the line was placed, and the area covered with 6 x 7 cm Tegaderm Film. A venous line was placed in the R. external jugular vein and covered with Tegaderm w/o dermatitis.

O/E: An 8 x 8 cm erythematous vesicular and hemorrhagic plaque is seen in the area under the Tegaderm. Island of sparing in center of patch is where angiocath resided. This plaque is cool to touch. Neck completely clear.

Clinical Photos:

Lab and Pathology: Not deemed necessary at this time.

Diagnosis: Irritant vs. Allergic Contact Dermatitis. Not likely Tegaderm since area under patch on neck is clear. I am considering a toxic burn from chlorhexidine under wrist patch. (see Addendum)

Questions: What are your thoughts?

Addendum: The anesthesiologist reviewed his notes and found that he applied Tincture of Benzoin to the area around the arterial line to help keep the Tegaderm in place, but not on the neck for the venous line. Allergic Contact Dermatitis to Benzoin is well-reported. This seems to be the culprit here. Hopefully, wet compresses followed by clobetasol 0.05% ointment will be helpful. We are indebted to the anesthesiologist for reviewing the operative record and educating us! We will patch test her once her eruption has quieted down.

References: (Free Full Text)
1. Indian J Dermatol Venereol Leprol. 2006 Jan-Feb;72(1):62-3.
Contact dermatitis to compound tincture of benzoin applied under occlusion.
Lakshmi C, Srinivas CR.

2. BMC Dermatol. 2004 Mar 31;4:1.
Severe facial dermatitis as a late complication of aesthetic rhinoplasty; a case report.
Rajabian MH, Sodaify M, Aghaei S.
Department of Plastic Surgery Shiraz University of Medical Sciences, Shiraz

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.