Fascinoma

Every day one sees something one never encountered before.
Here's a diagnostic and perhaps therapeutic challenge.

This well-nourished, alert, well-oriented 82 yo woman presents with a six month history of an erosive dermatitis of the perirectal and vaginal area. Over the past year she has had pathological fractures of both femurs and a history of herpes zoster (not cofirmed). Remarkably, she has put up with these erosions which are apparently not that painful.

O/E: Sharply marginated clean erosions around rectum and vagina. Similar lesions are found in skin folds (abdominal and under breasts). She has some oral ulcerations under her dentures and crusted lesions on the scalp. No vesicles or bullae seen or noted by other physicians.

Lab: At this time CBC normal. Antibodies for pemphigus and pemphigoid are negative.

Biopsies performed on Feb. 8, 2007 for H&E and DIF.

Who has seen a similar picture?

I am thinking about:
Pemphigus variant
Necrolytic migratory erythema
Acrodermatitis enteropathica

Will order zinc and glucagon levels if pathology is not helpful. Someone may have seen this picture before.

Updated February 16, 2007
Here's a follow-up on this patient...
Path: Shows:
Subepidermal blister with dense superficial and mid perivascular and interstitial mixed inflammatory cell infiltrate including lymphocytes, plasma cells, eosinophils , dermal edema, and papillary dermal fibrosis .
NOTE : These changes are non-diagnostic but suggestive of cicatricial pemphigoid in view of the immunofluorescence findings (see below).

See immunofluorescence results and note. (Photo by Jag Bhawan)
DIRECT IMMUNOFLUORESCENCE RESULTS : Linear "Immunostaining" was observed with IgG, IgA and intermittent with C3 at dermal epidermal junction and with C3 and IgA around blood vessels and appendages.
NOTE : These changes are non-diagnostic but consistent with cicatricial pemphigoid.

Putting this together, I suspect this is indeed an unusual variant of cicatricial pemphigoid. I have started her on prednisone 20 mg tid and tacrolimus ointment 0.1% (but insurance plans here do not always cover the ointment). A G6PD was ordered since I will probably use Dapson as well. I expect the management will be difficult.

February 22
Patient seen in f/u. To my surprise, there is early reepithelialization of perineal erosions. Oral erosions still prominent. I've lowered her prednisone to 20 mg bid -- awaiting G6PD results. Perhaps, the tacrolimus is helping. The comments havc been helpful.

Winter Itch

The Cold has finally hit us here in the Northeast. People are streaming into dermatology practices with "Winter Itch." This is an article from today's NY Times which should help our computer savvy patients.

Winter Itch

New York Times: Skin Deep Section  February 8, 2007

Feeling Parched? Itching Like Crazy? It Must Be Winter
By LAUREL NAVERSEN GERAGHTY

It gets worse as the winter drags on, and weeks of air that is too cold and heat that is too dry take their toll. Arms, thighs, knees, elbows and calves start looking like the surface of the moon: cracked, chalky and stretched. And then comes the itch.

Alexandra Lynner Gilbert, 25, an interior design assistant from Greenwich, Conn., said it has become so bad that she once begged a co-worker to trade sweaters because she needed cotton against her skin.

Allison Schwartz, 33, a graduate student from Brooklyn and self-proclaimed “queen of irrational rashes,” said she now wears only cashmere because any other wool makes her squirm.

Lisa Ziegler, 38, of Fresh Meadows, Queens, said after a hot shower she feels the urge to claw her skin.

And Lisa Henke, 32, of Seattle, said that it took a move from Washington to the drenched air of Washington State to make her skin problems go away.

“Winters in D.C. were horrible for me,” she wrote in an e-mail message. “My skin would become scaly and itchy and would require multiple latherings of lotion to keep from falling off.”

The lower temperatures, humidity-free air and layers of wool and other fabrics can leave skin irritated and uncomfortable.

“A good 25 percent of the population has some degree of winter itch, if not more,” said Dr. Julie Schaffer, an assistant professor of dermatology at New York University School of Medicine. “It’s common to the point where it’s almost a normal thing to have in the winter.”

For most people, it is just an annoyance. But those with conditions that cause itch, like psoriasis or eczema, can really suffer.

“Torturous is a good word for what some people experience,” said Dr. Jeffrey D. Bernhard, who wrote “Itch” (McGraw-Hill, 1994), a textbook.

“People may gouge at their skin, or they may rub and scratch till they bleed,” said Dr. Bernhard, a professor at the University of Massachusetts Medical School in Worcester and editor of the Journal of the American Academy of Dermatology. “Many people would rather put up with pain than itch.”

Dr. Bernhard said it is a lack of moisture that creates the problems. “You have a combination of exceptionally dry, cold air — which indoor heating makes even drier — overbathing and use of harsh soaps,” which can strip the skin of its moisture, he said. “At some point, the dry air will suck water out of your skin like a sponge.”

Without moisture, the skin hardens and flakes and begins to irritate the nerves closest to the surface. “When the outermost skin cells dry out, they have more ragged and sharp edges, and they may be stimulating those fine itch nerve endings in the skin,” Dr. Bernhard said.

In young people there is no difference in skin dryness across ethnicities, said Dr. Victoria Holloway Barbosa, who is the director of L’Oréal Institute for Ethnic Hair and Skin Research. But those who described themselves as African-American or Caucasian in skin studies tend to have increasingly dry skin with age and may be particularly vulnerable to itch later in life, she said. Those who identify themselves as Asian or Latino did not have similar complaints. Doctors said there is no magic cure for itch, but there are simple steps to diminish the problem.

Dr. Michael Tharp, the chairman of the dermatology department at Rush University Medical Center in Chicago, said it should be standard operating procedure “to shower not in hot water, but in lukewarm water.” The hotter the shower water, he said, the more moisture will be extracted from the skin.

He also recommended washing with a mild cleanser, “preferably a moisturizing body wash, which has lots of lipids, which could theoretically put oil into the skin and help repair the barrier functions.”

After stepping out of the shower, it’s good to apply cream or Vaseline within three minutes before the dampness can evaporate, a trick dermatologists call the “soak and smear” technique.

“The only way to rehydrate the stratum corneum” — the skin’s outermost layer — “is to trap it against the skin,” using a thick emollient, said Dr. Tharp, who is a consultant to Unilever, Novartis and other skin care and drug companies.

Dr. Barbosa said a humidifier, bath oil or over-the-counter hydrocortisone cream are other ways to treat dryness and itch.

Certain ingredients can ease the urge to scratch by interfering with the itch signal that travels from the skin to the brain. Creams containing menthol, for example, “actually stimulate the same nerve ending receptors that cold and touch stimulate, so they create another sensation that substitutes for itch,” which would relieve it somewhat, Dr. Bernhard said.

For people who experience nighttime itching that disrupts their sleep, an antihistamine like Benadryl may offer relief, said Dr. Alan R. Shalita, the chairman of dermatology at SUNY Downstate Medical Center, who does consulting, lecturing and research supervision for dermatology and cosmetics companies, including Allergan, Estée Lauder, and Galderma.

“A lot of people notice the itching more at night, because whatever their activities are during the day, it distracts them from feeling the itch,” Dr. Shalita said.

In most cases, the itch should just fade away. But when it persists or becomes more than a minor nuisance, a trip to the doctor becomes worthwhile, Dr. Bernhard said.

“At some point, a diagnosis has to be made, because it could be a sign of an underlying skin disease like eczema or psoriasis or hives, or even a kidney or liver problem,” he said.

A Common Entity

How many times have you experienced this?
The waiting room is full and a patient comes in, settles down comfortably and says, "I won't take up much of your time today, I see how busy you are. So I wrote down a few questions." Thus spake a healthy 55 year old man as he sat down and pulled out this post-it from his pocket. "La Maladie du Petit Papier," I thought with resignation.

PubMed has only five references to this entity we all know. The first in Italian from 1967. * We each have our own way of handling this. Some docs grab the list from the patient and say, ""Let's have a look at this." Others say, we'll deal with only three today; you choose." I usually let them read all of them and then proceed.

How do you handle this event? A couple of years ago there was a piece in the BMJ comparing the malady of the petit papier with the newly emerging la maladie du grand printout. The latter may be more common in the major cities of North America than elsewhere.

* Iandolo C. [The "petit papier" sign] [Article in Italian]
Policlinico [Prat]. 1967 Mar 6;74(10):321-8.

Mystery from Micronesia

We received this note from a physician in Hawaii. Your thoughts will be appreciated.

"Two years ago, I was shown photos of a severe skin condition which afflicted more than 80 persons on the tiny atoll of Satowan in Chuuk. Now, more than 100 persons on Satowan are affected and about 10 on the neighboring atoll of Lekinioch.

Affected persons develop thick, pink plaques, usually on the arms and legs – this appearance resulted in the nickname “Spam.” While the condition is not life-threatening, the people with the condition are sometimes ostracized and made to feel shame and embarrassment. Anecdotally, the plaques are more common in those who work or play outdoors (particularly taro farmers) and may sometimes occur following skin trauma.

The only treatment used on the plaques has been surgical debridement. Dr. Bosco Buliche who practices in Weno, the capital of Chuuk, has seen and photographed cases on Satowan. He has posted 5 cases on the Pacific Island Healthcare Project website (through TAMC), including one with biopsy results, which showed only nonspecific inflammation – we could transport only a fixed specimen, unfortunately. Opinions from various practitioners on the cause of the condition have run the gamut from “island psoriasis” to mycobacterial infection.

So, two years later, there is still no answer. It would be wonderful to find someone with the interest and resources to investigate the problem. Suggestions will be most welcome."

Clinical Photos:

Stranger than Fiction

Abstract:
50 yo man with unusual facial eruption

Presented by:
DJ Elpern

History:
This 50 yo man develops an eruption on his left malar eminence annually. This occurs after his late December deer hunting trip. He is exposed to cold air. Does not take any new meds at that time. The eruption has a burning sensation.

Exam:
Annular plaques with central pallor and peripheral dusky red color left malar. No other similar lesions.

Clinical Photos:
"X"marks the biopsy sites














Lab and Path:
DIAGNOSIS: Tumid lupus erythematosus
NOTE : The specimen exhibits flattened epidermis , mild to moderate papillary dermal edema, dilated superficial blood vessels and a moderate superficial and deep perivascular and periappendageal lymphocytic infiltrate with numerous extravasated erythrocytes and marked superficial and deep interstitial mucin deposition. These findings support the histologic diagnosis . The differential diagnosis includes , in the appropriate clinical setting , lymphocytic infiltrate of Jessner. P.A.S. stain is negative for fungal hyphae and basement membrane zone thickening. Clinico-pathologic correlation is suggested.











Therapy and Course:
The patient was biopsied and seen a week later for suture removal. No treatment was rendered. Here is how he looked at day six post biopsy.















Questions and Teaching Points:
Histologically this is lupus. However, the patient is asymptomatic and the lesions cleared without therapy. The cold exposure is most likely key. He may have a form of L.E. There is an entity called chilblain lupus which could fit here. Most of those patients had SLE. Has anyone seen a similar case? I will suggest serologies to him. He does not need any therapy at this time.

References
This and other references can be found in PubMed if "lupus chilblain cold" is searched.

Franceschini F, et. al.
Chilblain lupus erythematosus is associated with antibodies to SSA/Ro.
Adv Exp Med Biol. 1999;455:167-71.

Clinical Immunology Unit, Spedali Civili, Brescia, Italy.

Chillblain Lupus Erythematosus (CL) of Hutchinson is a subtype of Lupus Erythematosus characterized by erythematous lesions symmetrically distributed on the face, nose, fingers and toes, knees and heels. The lesions are induced by cold, damp climates. A number of patients affected by CL eventually develop features of Systemic Lupus Erythematosus (SLE). We report here 7 patients, all but one affected by SLE, with chilblain cutaneous lesions on their hands, feet and face. The onset of CL preceded the diagnosis of SLE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the other 2 cases. Six out of the seven patients referred typical Raynaud's phenomenon and one had acrocyanosis. CL lesions developed and were aggravated by the cold during autumn and winter, they improved during summer. Skin biopsy performed in 5 patients from the lesions showed, on histology, a typical pattern of alterations with granular deposits at the dermo-epidermal junction on direct immunofluorescence. Laboratory findings showed: ANA and anti-SSA/Ro were detected in all the patients, anti-SSA/Ro were isolated in 4 patients and associated with anti-Sm in one case, anti-U1 RNP in one case and with anti-Sm and anti-RNP in a third case. Complement consumption was observed in 5 patients, anti-dsDNA in the six patients with SLE, hypergammaglobulinemia in 4 and rheumatoid factor in one. The fine specificity of anti-SSA/Ro as determined by immunoblotting using a human spleen extract as a substrate, showed: anti-60kD and anti-52 kD in two sera, anti-60kD isolated in 2 sera, anti-52kD isolated in one serum (from the patient without SLE) while 2 sera did not blotted. In conclusion, our study confirms the previous report of anti-SSA/Ro antibodies in association with CL. This clinical and serologic association widens the spectrum of cutaneous disease that is associated with antibodies to SSA/Ro to include conditions such as to SCLE, hypergammaglobulinemic purpura and neonatal lupus.

Alopecia

Imiquimod + 5FU

Abstract 62 yo man with SCIN of finger. Failed Imiquimod and responded to combination of 5% 5FU + Imiquimod

Presented by: D.J. Elpern, Williamstown, Massachusetts, USA

History This 62 yo man had a 15 year history of a plaque on his left index finger. He had been told it was a wart in the past.

Exam
2.0 cm in diameter verrucous plaque.

Clinical Photos






Lab and Path Because of long history a biopsy was done to r/o SCC. The path showed in situ squamous cell carcinoma.

Therapy: We discussed micrographic surgery vs. topical chemotherapy and he elected the latter. After two weeks of nightly imiquimod there was no reaction. I then added 5% 5FU cream as described in a recent article. Within two weeks the area was ulcerated and painful. The treatment was stopped and he will have a repeat biopsy to test for cure in 2 - 3 months.

Questions and Teaching Points The combination of 5FU and imiquimod is interesting and will be a therapeutic advance, but like imiquimod, it will take some time to master the "Art." I was surprised at the intensity of this reaction. I suspect he may have done better if I used the 5FU three times a week. Your comments are welcome

References
J Am Acad Dermatol. 2006 Dec;55(6):1092-4.

Topical combination therapy for cutaneous squamous cell carcinoma in situ with5-fluorouracil cream and imiquimod cream in patients who have failed topical monotherapy.

Ondo AL, Mings SM, Pestak RM, Shanler SD.

Topical therapeutic options for cutaneous squamous cell carcinoma in situ include 5-fluorouracil cream and imiquimod cream. Such treatment may be preferable to surgical or destructive modalities in certain anatomic locations and in instances where patients are unwilling or poor surgical candidates. We present 4 such patients with cutaneous squamous cell carcinoma in situ involving a digit. Each patient failed treatment with imiquimod cream as monotherapy. In addition, two patients failed treatment with 5-fluorouracil cream as monotherapy. All 4 responded completely to 5-fluorouracil and imiquimod cream as combination therapy. In patients who have failed monotherapy with a topical agent for cutaneous squamous cell carcinoma in situ, combination treatment using both topical 5-fluorouracil cream and imiquimod cream may be considered as an alternative therapeutic strategy.

Deadly Allopurinol Hypersensitivity Syndrome

The patient was a 59-year-old man who presented with 3-week history of generalised skin eruptions associated with high fever. He was well previously and was started on allopurinol for asymptomatic hyperuriacemia. About 3 weeks later, he started feeling unwell and giddy. Then he developed a generalised erythematous eruptions on the lower legs which then spread to the trunk and face. He was admitted at the general hospital but discharged five days later. He persisted feeling unwell despite discharged from the ward. The skin eruptions worsened.

On examination he was febrile. Temp: 38.5 degC He appeared jaundiced and sallow. There was marked pitting ankle edema. Generalised erythema all over the face, trunk and extremties were noted. Diffuse scaling was noted on te face, neck and upper limbs. There was no hepatosplenomegaly.

Clinical diagnosis: Allopurinol Hypersensitivity Syndrome

Blood urea was 27.6mmol/l and creatinine 9.3mmol/l

SGPT 242 mmol/l
SGOT 115 mmol/l
Alk Po4ase 567 mmol/l
Bilirubin 87.7 umol/l

Hb7.8gm%
TWBC 18 000

Immediately the allopurinol was stopped and he was dialysed and started on IV hydrocortisone 200mg 6hrly. IV ciprofloxacin 750mg bd was initiated was empirical treatment for possible septicemia. He responded well but when the IV hydrocortisone was tailed down, his condition deteriorated. His jaundice became deepened and he became more drowsy. We increased the dose of IV hydrocortisone and withhold other drugs as well - there may be some cross reaction. IV albumin was transfused. He improved and became more alert. CT Brain and ultrasound abdomen was unremarkable. We hope the supportive therapy can pull him through.

Drug hypersensitivity syndrome is a severe idiosyncratic reaction associated with taking drugs. Other names are "drug rash with eosinophilia and systemic symptoms" (DRESS) and "drug induced delayed multiorgan hypersensitivity syndrome" (DIDMOHS). The most common triggering agents are antiepileptic drugs (phenytoin, phenobarbital, and carbamazapine), sulphonamides, and allopurinol.

The exact mechanism for the development of allopurinol hypersensitivity syndrome is unknown; the pathological substrate is often a diffuse vasculitis induced by a type III hypersensitivity reaction, with formation of immune complexes that precipitate in vascular endothelium and promote an inflammatory reaction. Accumulation of oxypurinol (the principal metabolite of allopurinol) in renal insufficiency is considered a crucial factor for the development of allopurinol hypersensitivity syndrome and may lead to tissue damage by toxic or immunological mechanisms.

References:
Alfonso Gutiérrez-Macías, Eva Lizarralde-Palacios, Pedro Martínez-Odriozola, Felipe Miguel-De la Villa Clinical reviewLesson of the week. Fatal allopurinol hypersensitivity syndrome after treatment of asymptomatic hyperuricaemia BMJ 2005;331:623-624

Y C Chan, Y K Tay,S K Ng Allopurinol Hypersensitivity Syndrome and Acute Myocardial Infarction— Two Case Reports. Ann Acad Med Singapore 2002; 31:231-3

Unique Drug Eruption

This 83 yo old man has chronic renal failure with a creatinine of 18 (!!) but does not need dialysis yet. His hemoglobin has been maintained with Procrit for the past four years. Because of a low transferrin saturation (30 %), his nephrologist added a new form of iron infusion around 2 months ago. (This was in another state and I don't have the name of the product) Within two weeks he started developing a generalized pruritic eruption. He stopped the iron infusions, but the rash persists.

There is only one report of a similar process (see below); but it is likely that more cases are extant as this therapy becomes more popular.
For the time being, he will be treated with triamcinalone 0.1% ointment after soaking in a tub. This is William James et. al's "Soak and Smear" technique. (see ref)

It's likely that the iron will stay in his system for some time. There are no treatment guidelines here; and as the patient can sleep at night, I prefer not to give him antihistamines at this time.





Reference

1. The safety and efficacy of ferumoxytol therapy in anemic chronic kidney disease patients.
Spinowitz BS, Schwenk MH,Jacobs PM et al
Kidney Int. 2005 Oct;68(4):1801-7.

BACKGROUND: Administration of safe and effective iron therapy in patients with chronic kidney disease is a time consuming process. This phase II clinical trial studied ferumoxytol, a semi-synthetic carbohydrate-coated iron oxide administered by rapid intravenous injection to anemic chronic kidney disease patients (predialysis or undergoing peritoneal dialysis). METHODS: Inclusion criteria included hemoglobin delayed pruritic erythematous rash, and transient pain at the injection site. CONCLUSION: Although larger studies are required, this small study demonstrates that ferumoxytol can be safe and effective in increasing iron stores, is associated with an increased hemoglobin response, and is well tolerated at a rapid infusion rate.

2. Soak and smear: a standard technique revisited. Gutman AB, Kligman AM, Sciacca J, Arch Dermatol. 2005 Dec;141(12):1556-9

BACKGROUND: Atopic dermatitis, nummular eczema, chronic hand dermatitis, palmar plantar psoriasis, and xerotic eczema are common inflammatory skin conditions. They may be refractory to conventional topical and even systemic treatment. Little evidence is available that demonstrates the benefits of aggressive topical treatment of patients with these disorders. OBJECTIVE: To describe a simple, inexpensive, effective topical treatment with an accompanying patient educational sheet. DESIGN: A retrospective study of 28 patients referred to a tertiary care center for refractory chronic pruritic eruptions. Intervention with a plain water 20-minute soak followed by smearing of mid-strength to high-strength corticosteroid ointment led to clearing or dramatic improvement. RESULTS: Objective and symptomatic improvement was obtained from aggressive topical treatment. It was well accepted in this group of referral patients. CONCLUSIONS: Hydration for 20 minutes before bedtime followed by ointment application to wet skin and alteration of cleansing habits is an effective method for caring for several common skin conditions. Prospective studies are needed to further validate these findings.

VGRD 2.0

"And gladly wolde he lerne and gladly teche" Chaucer

Welcome to VGRD 2.0

This updated version of VGRD will allow more interactive postings by dermatologists. Virtual Grand Rounds in Dermatology (VGRD) was formed in 2000 to serve as a place for dermatologists the world over to meet one another and share interesting and challenging patients. One may want to ask a question about diagnosis or therapy, present an interesting clinical photo or post a photo or photomicrograph. We are a group of clinical and academic dermatologists who believe that this form of web-based teledermatology can be both personally and professionally enriching. VGRD has hundreds of dermatologists on our mailing list and they cover all of the subspecialties in dermatology.

We would particularly like to invite dermatologists from both developing and developed countries to join us and share your experiences with our members. Together we will be able to provide better care for our patients.

We think you will find the new case presentation format to be clear and easy to follow. If you have suggestions on how we can improve it, please let us know.

We hope this form of electronic communication and teledermatology through shared knowledge will help to enhance our patient care. Your participation will help to make VGRD successful.


Yours sincerely,

David Elpern MD
The Skin Clinic
Williamstown, MA, USA
djelpern@gmail.com

Henry Foong FRCP
Foong Skin Specialist Clinic
Ipoh, Malaysia
bbfoong@gmail.com

Depressing Reality

Dear Colleagues,

Please help me with a 4 year-old lad who was referred today by his GP for genital warts. A court-appointed foster parent brought him in for evaluation. His 21 year-old mother left him recently with relatives. She is an inattentive parent with an alcohol and possibly a drug problem. The child was in pre-school but was suspended a few days ago because he was using sexually explicit language, being physically abusive to his peers, discussing French kissing and saying he was going to shoot himself.

The exam showed a quiet, calm and health 4 year-old. The only findings were lobulated and verrucous flesh-coloured papules grouped on the shaft of the penis. There were no bruises or evidence of trauma.

Diagnosis: Condyloma acuminata.

Photos were removed at advice of a pediatrician. Apparently, FBI may moniter some sites. Gets my Canadian soul anxious!

Discussion:
Although genital warts in children can occur in the absence of child sexual abuse, this case is suspicious. The child's broken home, the fact that he has been left with numerous teenage girls as baby sitters, his sexually explicit language, his antisocial behavior and language all are red flags for a child at risk.

Here, in Nova Scotia, we are mandated to report such children to the authorities. I would like to ask wo questions:

1) Your opinion as to whether reporting this child is appropriate.
2) How should he be treated?
a) electrosurgery under general anesthesia
b) cryo therapy
c) imiquimod or podophyllin.

Your thoughts and comments are most welcome.

Hamish Dunwoodie, M.D. FRCPC - Paediatrics
Moncton, NS, Canada

Unusual Case

This 53 yo man presented with a three week history of a pustular eruption on the left small finger. It began as a papule and slowly enlarged.
History reveals that he has a farm with 500 sheep. He has also been exposed to cats and dressed a deer around a month ago.

Diagnostic tests were done.





Follow-up: The KOH prep was positive. The fungal culture is positive at ten days. Thus, this is inflammatory tinea manus. Await fungal culture for typing of dermatophyte. Resolved with dilute Clorox soaks and 2% ketoconazole cream in one week.

12 days after initial visit.

Unilateral Bullae

History
The patient is a 28 yo man referred on a walk-in basis with an eight month history of a bullous eruption on the dorsum of his left hand. He gets one or two painless lesions a month. No history was sent over with the patient and he is a poor historian. His medications include Welbutrin, Clozeril and Soma (carisprodol/aspirin)


Exam:
The only findings are on the dorsum of the left hand. Here, there is a hemorrhagic bulla, a vesicle and areas of mild erythema at sites of previous lesions.





























Biopsy:
I suspect fixed drug or pseudoporphyria cutanea tarda. I biopsied the small papule and the edge of the bullae and made a follow-up appointment.
Path Report:
A subepidermal separation, individually necrotic keratinocytes , and a sparse superficial perivascular lymphocytic infiltrate with occasional neutrophils .
NOTE : (A and B). Amphophilic globular material is seen deposited in the dermis and around blood vessels in both specimens. This material is P.A.S. stain positive, and stains negative for amyloid and elastic tissue. The differential diagnosis could include porphyria cutanea tarda , although this is usually less inflammatory. A subepidermal autoimmune bullous disorder could also be considered. An additional biopsy for direct immunofluorescence may be of help.


Laboratory Studiess:
CBC normal, LFTs normal, ferritin normal. Hep B and C negative, All urinary porphyrins well within normal levels. Uroporphyrin: 9.6 ug (nl < 30.0) Coproporphyrin 40 ug (normal < 65/24 hr)

Discussion: This is likely pseudoporphyria cutanea tarda. A similar case has been reported. Your comments are welcomed.

Mystery Rash in Runners

The Williams Cross Country running team went to a meet on the Connecticut shore on Saturday, October 28. There had been a big storm the previous day. The course took them through brackish water sometimes thigh deep. Within a 24 - 48 hours, around half of the runners had developed intensely pruritic lesions on arms and legs. The vast majority of the lesions were on exposed skin. The primary lesion appears to be a juicy papule or a clear vesicle. Some of these have crusted over in a few days. The lesions are discrete. They do not have the linear pattern characteristic of phytocontact dermatitis from a plant such as poison ivy. The pictures were taken of the legs of an 18 yo woman and a 21 yo man. The lesions were quite similar.




It's likely that this is from something dispersed in the brackish water they ran through. Runners from other schools were affected as well. About 50 runners from Williams College developed the eruption.

The possibilities include:
Atypical plant contact dermatitis (Some plant toxin such as urushiol churned up by the storm and floating in the water.)
Jellyfish nematocysts free floating after the storm and blown in
Bird schistosomes -- this is severe for that.
Bacterial infection (a culture was taken from one student and results were not specific)

Do you have other thoughts? What is your opinion?

Solution: The Cross Country course was redirected after the storm. A new path was cut though brush to circumvent a brackish area. It is likely that the new path went through poison ivy. The appearance of the rash 24 - 48 hours after exposure in the majority of runners and the quality of the itch suggests rhus contact dermatitis.

Teenager with a Red Nose

This 14 year-old girl has had a red nose for the past two years. It followed as severe sunburn. Her nose never looks normal. Occasionally, there are small cysts with bleeding and purulent drainage.
















I have seen this picture occasionally in teenagers. It looks like a rosacea variant. I'd also consider demodex.

Your suggestions are anticipated and will be welcome.

30 year old woman with changing mole

This 30 yo woman has noticed an enlarging pigmented lesion in her right axilla for almost a year. Her mother, a registered nurse, asked her to see a dermatologist. Her husband did not notice it.
The patient has Type II skin. The general cutaneous exam was unremarkable save for a 1.2 cm in diameter barely elevated plaque. There is a play of pigment and outline is irregular.

An excisional biopsy was performed.

Pathology: Malignant melanoma in situ , superficial spreading type. (Read by H. Byers of BU Skin Path who took photomicrographs) "The specimen exhibits a marked nested and lentiginous melanocytic proliferation with large severely atypical epithelioid cells. There is irregular nesting, focal confluent lentiginous melanocytic proliferation and cellular dyshesion."









































She is scheduled for a wider excision, 0.5 cm on either side. No further work-up other than regular follow-up visits.

Your thoughts are appreciated.

Positive Band Aid Sign

Most dermatologists know about the "positive band aid sign." To me, it means a skin cancer has been lurking there for a while.

This 82 yo man came in with a one year history of a "sore" on his back. The drainage stained his shirt. When the band aid was removed, there was a one cm in diameter clean friable tumor, most likely a basal cell.

The lesion was shave biopsied and desicated and curetted. I'll affix a copy of the biopsy report when I get it.

How many of you use the term "Positive band Aid Sign?"

BCC of Eyelid

This 43 yo man has a one year history of a lesion of the left lower lid. It measures 8 mm in diameter.
Biopsy from "X" confirms "nodular BCC."
Question: What is best therapy?
1) Mohs
2) Mohs + closure by ophthalmic plastic surgeon
3) Ophthalmic plastic surgeon handle all
4) Another approach

Cada vendador allabe sus agujas.
Every peddler praises his needles.

Therapeutic Question

The patient is an 83 year-old woman with a two year history of a lesion on the nose.
Clinically, this is a basal cell. My question is what you would recommend as therapy and why?

1) Micrographic surgery with forehead advancement flap.*
2) Micrographic surgery with graft or allowed to heal by secondary intention.
3) Radiotherapy
3) Other






















* Repair of defects on nasal sebaceous skin.
Dzubow LM.
Department of Dermatology, University of Pennsylvania Health System, Philadelphia, 19085, USA. leonarddzubow@comcast.net
BACKGROUND: Reconstructive procedures performed on sebaceous nasal skin are prone to partial flap necrosis, scar spread and inversion, and tissue mismatch. An ideal repair would optimize vascular integrity, minimize closure tension, and use adjacent tissue. OBJECTIVE: The purpose of this article is to describe a flap design and dynamics that permit satisfactory reconstruction of small- to medium-sized defects on nasal sebaceous tissue. METHODS: A modified advancement flap is described that may be used on central and off-midline defects of the nasal tip. RESULTS: Use of the modified advancement flap resulted in good cosmetic results with few adverse postsurgical events. CONCLUSIONS: The modified advancement flap satisfies the requirements of a hardy blood supply, minimization of closure tension, and use of adjacent tissue. The surgical results are predictable and rarely associated with complications.

An Incidental Finding

The patient is an 85 yo man who presented for generalized pruritus which has been present for two years. He has a history of prostate cancer and gastrointestinal bleeding. He's had a few transfusions over the past year or so and was concerned that they caused his itching.

He did not complain about his nose. I asked about it and he and his daughter gave this history: Three years ago he was hospitalized for pneumonia. He delvelped an inflammation of the ala nasi and was told it was "shingles." Biopsies were done and ruled out cancer. The left ala nasi was destroyed and this is how he healed.

Discussion. I wonder whether nasal oxygen was used and if the canula caused damage to the nose. It cold also have followed H. zoster. This is the picture that can be seen with Trigeminal Trophic Syndrome, a rare disorder that is thought to be traumatic in origin. Has anyone seen a similar picture and what are your thoughts.?

This is a good abstract:
Laryngoscope. 1988 Dec;98(12):1330-3.

Trigeminal trophic syndrome.

Arasi R, McKay M, Grist WJ.

Department of Dermatology, Emory University School of Medicine, Atlanta, GA.

Trigeminal trophic syndrome is an unusual condition also known as trigeminal
neurotrophic ulceration or trigeminal neuropathy with nasal ulceration. The
diagnosis is suggested when ulceration of the face, especially of the ala nasi,
occurs in a dermatome of the trigeminal nerve that has been rendered anesthetic
by a surgical or other process involving the trigeminal nerve or its central
sensory connections. A history of paresthesias and self-induced trauma to the
area further support the diagnosis. Neurological deficits causing trigeminal
trophic syndrome may result from surgical trigeminal ablation, vascular
disorders and infarction of the brainstem, acoustic neuroma, postencephalitic
parkinsonism, and syringobulbia. The following etiologies of nasal ulceration
should be excluded: postsurgical herpetic reactivation and ulceration, syphilis,
leishmaniasis, leprous trigeminal neuritis, yaws, blastomycosis,
paracoccidioidomycosis, lethal midline granuloma, pyoderma gangrenosum,
Wegener's granulomatosis, and basal cell carcinoma. In the case reported here,
the diagnosis of TTS was made primarily as a result of previous experience with
the syndrome, underscoring the importance of physician recognition of this
unusual disorder.

Tale of Two Lesions

I saw these two patients today. Your comments are most welcome. Path reports will be affixed when available.

1) 69 yo man with 1.5 month history of lesion right pinna. The lesion is slowly growing. The patient's son, a family doctor, asked him to make an appointment. This is a subtle lesion that could be a lentigo or a lentigo maligna. Two representative areas were biopsied.





















2) A 55 year-old priest whose parishioner noted a lesion on his left arm and asked him to see a doctor about it. He's been aware of this lesion for 3 months. It appears that the darkly pigmented papule began in a lentigo. I favor a diagnosis of melanoma here but this could be a seborrheic keratosis. An excisional biopsy of raised and macular portions was performed and based on pathology I will recommend further therapy.

Hypopigmentation in atopic dermatitis

This is a 7-year-old boy who developed multiple patches of hypopigmentation on the dorsum of the hands for 3 years. He had a history of atopic dermatitis and had severe eczema of the hands prior to development of the hypopigmentation on the hands. According to the mother, he had secondary infection with crusts formation on the hands. The lesions were patchy hypopigmented macules mainly on the dorsum of the hands and feet. There were no family history of similar pigmentation. Wood's lamp examination however showed accentuation of the hypopigmented lesions.

Based on history this is most likely post inflammatory. Other differential considered includes vitiligo. Vitiligo is probably unlikely in this patient as the hypopigmentation developed after he had eczema with secondary infection of the hands and feet.

Any suggestions for this hypopigmentation? Would UVB or tacrolimus ointment help?

Thanks for your comments.

A Case from New Brunswick

A friend from coastal New Brunswick sent this interesting case:

Dear Dr. Elpern,

The patient is a 91 year-old working lobsterman who was sent in by his GP for evaluation of a large fungating tumour on his left temple. By history, the lesion has been present for 15 years but has only really increased in size in the past 18 months. He's in vigourous good health otherwise.
On close examination, the tumour was found to be on a stalk. It's surface measured around 5 cm in diameter, whilst the pedicle was 9 mm in diameter.
After cleaning the area, I administered local anesthesia with lidocaine and epinephrine and snipped it off. I dissicated the base and applied a dressing.
I may follow-up with imiquimod (as recommended by a dermatologic friend) or may excise the defect secondarily.
We occasionally see dramatic tumours like this is watermen. They are a stoic lot.

I wonder, do your colleagues have any suggestions? I will post the path report when it is ready.

Cheers,

Dr. Hamish Dunwoodie, FRCSC
General Surgeon
Moncton, NB, Canada

Probably Lyme

The patient is a 56 year old man in good general health.
Six days ago, he developed an erythematous papule on the left abdomen.
Over the past week, this has grown into an oval 10 centimeter pruritic erythematous plaque.
This is a solitary lesion and the remainder of the cutaneous exam is unremarkable.
There is no history of tick bite, but this is an endemic area and the patient has an outdoor life-style.

I suspect this is Lyme Disease and that it is too early for serology to be helpful.
After discussion with the patient, he was started on doxycycline 100 mg bid.

(For interest, please see case of June 20th)