Zebibah

The zebibah, Arabic for raisin, is a dark circle of callused skin, or in some cases a protruding bump, between the hairline and the eyebrows. It emerges on the spot where worshipers press their foreheads into the ground during their daily prayers.

Here is a fine article in the NY Times on the ZEBIBAH.

One of our editors, Ben Barankin, published a letter on prayer marks in the International Journal of Dermatology. Barankin Letter

Dr. Barankin's letter was a comment on:

Prayer marks.
Abanmi AA, et. al.
Int J Dermatol. 2002 Jul;41(7):411-4.
Dermatology Department, Research Center,
Riyadh Al Kharj Armed Forces Hospital

Iatrogenic or Not?

Your opinion is sought.
The patient is a 42 y.o. woman with a long history of cystic acne. Typically she gets a few lesions a year. She has many ice pick scars from previous lesions. She was offered isotretinoin years ago but declined and indeed the lesions are less frequent now. She presents for intralesional triamcinalone when whe wishes to have the cysts treated. She was seen on December 5, 2007 with a tender cyst on the medial aspect of the left eyebrow. It looked like a typical acne cyst and was injected with Kenalog 3 mg per cc. Two days later she started to develop eyelid edema. She went to the Emergency Room and was given parenteral Rocephin and Cipro 500 mg bid. The swelling persists.



On examination, one can see where the initial cyst was. It's ~ 90% resolved. Below it there's a firm linear band that feels like a lymphangitic vessel.

I assume this must be related to the initial inflammatory lesion or the intralesional injection. I searched MEDLINE but could come up with no similar cases. If this is secondary to intralesional steroids, it is not reported. Could the original lesion have been a pyogenic infection that looked like her past cysts and could eyelid edema be secondary to lymphatic obstruction? The ER doc recommended another dose of Rocephin.
Addendum: The patient has never had fever, headache or other findings suggestive of a pyogenic infection.

Has anyone seen a similar case? Diagnostic and therapeutic suggestions are welcome.

Palmar Lichen Planus?

This case was presented by Dr Khalil Alhamdi, Basrah Medical College, Iraq.

S.G, 62 yo woman, presented with bilateral itchy palms 2 years duration with poor response to treatment. Physical examination is normal apart from bilateral diffuse hyperkeratotic violaceous palms. All laboratory investigations were normal

Histopathology: histopathological examination showed typical features of lichen plaus.

Diagnosis: palmer lichen planus.

What is the experience of our colleagues in seeing such a presentation of palmer lichen planus? comment please.

Epidermal nevus affecting foot

A 22 year old man presented with a history of linear hyperkeratotic lesion on the right foot since age 7 yrs. It hurts his foot when he wear his shoes.

Examination of the right foot showed a localised hyperkeratotic plaque extending linearly to the medial malleolus. It was not tender or inflamed. My impression is that of an epidemal nevus. Appreciate your thoughts on diagnosis and treatment options.

Annual Hot Spots Program

Conference Web Site

Winter's Toes

Abstract: 18 yo boy with pruritic, erythematous toes.

History: The patient was referred by his pediatrician with complaints of red, itchy lesions on his toes for two to three weeks. The pediatrician was concerned because some of the lesions looked vasculitic. The patient is in good general health. A non-smoker. He recollected as the interview progressed that he'd had similar but less severe problems in the past.

Physical Exam: Erythematous macules, some hemorrhagic in appearance on toes. One lesion slightly necrotic.

Photos:

Lab: CBC, ANA and other tests ordered at pediatric office
Pathology: none

Diagnosis: Chilblains (Lupus Pernio)
Reasons Presented and Questions: Similar patients are seen two or three times a year in my office. The typical case is a child or young adult with pruritic erythematous macules or papules on the toes or fingers. The lesions can look necrotic and sometimes ulcerate. The person is otherwise healthy. Pernio is most commonly seen in fall or winter, but I've seen it in spring, too. The lesions are worrisome in appearance to physician, parent, patient. The index case here was worried that "my toes will rot off." His mother was worried about "lupus." I reassured them that this is a common, self-limited problem. While nifedipine may help, it's not necessary. Wear warm socks. Give it time. There's a good review on emedicine.com. It says: "The direct cause of pernio is cold exposure. Chronic pernio may be secondary to various systemic diseases as follows:
Chronic myelomonocytic leukemia
Anorexia nervosa
Dysproteinemias
Macroglobulinemia
Cryoglobulinemia, cryofibrinogenemia, cold agglutinins
Antiphospholipid antibody syndrome
Raynaud disease"

But it's usually easy to differentiate which ones are worrisome and the vast majority will be simple chilblains. One may need to use some clinical judgment. Not rush to unnecessary testing. In my experience, and from what I've read, this occurs more when it is cold and damp in thin individuals. Not bone chilling cold, but the cold of early winter. When it is really cold, good socks and footwear may be more the norm and can protect a susceptible individual from chilblains.

11/29/07 -- Patient just seen:
These are the toes of a 70 yo man who has had chilblains for the past 30 winters. His toes are uncomfortable, itchy and occasionally painful. He's had no chronic illnesses and enjoys good health.


Here is an excellent full text reference: "Pernio in Children" from the journal, Pediatrics.

Notched Nose

This 55 year-old woman had micrographic surgery for a basal cell of the left ala three years ago. The graft did not take and she has been left with a notched ala. The defect, in the center of her face, is more disfiguring than it appears in this photo.


Question: What kind of repair could be done at this time to give her a better functional and cosmetic repair.

Uncommon Warts

SRLAMAT DATANG!
Welcome, New Members from the Penang Conference
Early morning, view before we convened for our scientific sessions. Penang is an amazing place to meet new friends, relax and sample a singular culture.

Please look at two interesting cases.

This is the tale of two young patients with warts who were seen back to back on November 17, 2007. Verruca vulgaris (the common wart) is sometimes quite "uncommon."

1. This is a seven year old boy who has had acute lymphocytic leukemia fr three years. He has had recalcitrant warts for the past nine months. At present he has failed liquid nitrogen, TCA and imiquimod. His present medications include methotrexate, 6 mercaptopurine, vincristine and dexamethasone. Each therapy for his warts has resulted in more florid reappearance. This child has suffered a lot over the past few years. Clearing his warts would be a small but pleasant victory for him. Your comments will be appreciated.








2. This is a 12 year old girl who initially had a small wart on her knee. Her physician treated with TCA and then liquid nitrogen over a few months. Each time the wart recurred larger. One can see concentric rings from previous treatments. I'd like to find a treatment that won't cause permanent scarring. In theory, the wart will disappear in a year or two, so the therapy should be benign. I prefen not the use liquid nitrogen or cautery here. Have you had success with warts like these?

Eczema herpeticum

A 2-year-old boy presented with a 3-day history of vesicular eruptions on the mouth followed by extensive pruritic skin eruptions on the upper and lower limbs. This was preceded by fever. He was otherwise well.

Examination of the skin showed extensive eczematous lesions on the lower and upper limbs. Excoriations, erosions and vesicles were also noted on the affected areas. Vesicular lesions and erosions were noted around the mouth. He was afebrile.

Clinically he has eczema herpeticum.

This is disseminated herpes infection at sites of epidermal disruptions. He was treated with oral acyclovir 50mg 5x daily and oral cloxacillin 125mg qid. Wet compresses with diluted KMNO4 and topical mometasone cream was used for the weepy eczematous eruptions. The lesions gradually subsided with the treatment.

Buttock eruptions

This is an 11-month-old girl with a one day history of skin eruptions on the shoulders and gluteal areas. She was otherwise well. no fever. Feeding well. She was referred to me by a pediatrician.

Exam: symmetrical patches of erythematous vesiculopapules on the gluteal areas. Similar lesions were noted on the shoulders and chest. No lesions were noted on the upper limbs. Hep B was not checked.

Is this viral? Gianotti Crosti Syndrome?

Thanks.

Nasal Lesion in a Child

The patient is a 30 month old girl with a 15 month history of a papule on the left ala nasi. Her father has Hereditary Leiomyoma Renal Cell Carcinoma syndrome (HLRCC) and the patient is positive for the gene. The question is whether this papule is a possible sign of the HLRCC syndrome or an unrelated entity.

The tumor is 3-4 mm in diameter and has been growing very slowly. The child is in good general health otherwise. The lesion is red in color, regular in outline. Clinical and dermoscopic pictures are presented. (I am new to dermascopic photography and it was difficult to focus on this area in a cooperative but somewhat squirmy child.)

Your thoughts will be appreciated.

Paronychia in a Child

The patient is an eight year-old girl with a three week history of paronychial inflammation. Mild pain at onset and now. No history of trauma or finger sucking. She was first seen by her pediatrician around ten days after onset and told it was improving and to give it time. No improvement after ten days; given a ten day course of cephalexin and urged to do warm soaks. No improvement noted. Seen in Dermatology Clinic October 26, 2007.



































Examination: Marked inflammation of proximal and lateral nail folds. No fluctuance. Perhaps, mild pain. No discharge. KOH from chalky material at proximal nail fold negative. The entire nail is dystrophic, perhaps suggesting longer involvement,

Questions:
Is this an acute bacterial or a chronic candidal paronychia?
Should the proximal nail fold be explored?
Other than bacterial and fungal cultures, any further studies?
What would you do at this point?

Hand Bullae in the Setting of Hemodialysis

Presented by Tucker Slingerland, M.D. Chief Resident in Family Practice at the University of Vermont.

HPI: A 76 year old woman with multiple medical problems including chronic hemodialysis, hypertension, hypertriglyceridemia, and type II diabetes mellitus presents for a second opinion on bullous lesions involving the dorsal hands, fingers, tips of ears and scalp. She had been told she had "porphyria," given sparse information about her condition, and started on a course of cloroquine with questionable effect.


O/E: There are healing erosions on the dorsae of both hands. At this time, no bullae.















Labs and Biopsies: These were not sent to us

Discussion:A literature review using key words porphyria cutanea tarda (PCT) and dialysis yielded a useful case report published in J Am Acad Dermatology 2000 by Shieh et al.(1) As reviewed in the case report, PCT is most commonly an acquired condition due to hepatic injury secondary to toxins (including drugs), hepatitis C, HIV, and hemochromatosis which leads to a defect in heme biosynthesis with resulting increase in iron stores (ferritin) and porphyrins. These porphyrins cause photosensitivity particularly on the dorsae of the hands. With increasing numbers of patients with chronic renal insufficiency on maintenance hemodialysis, recent case reports estimate a PCT prevalence of 1.2-18% .

A clinical diagnosis can be confirmed with a porphrin profile (serum, urine, fecal), skin biopsy findings detecting a subepidermal vesicle with little inflammation, and immunofluorescence studies. Urine tests may be difficult in the dialysis patient with little or no urine output. Serum iron studies, which are non specific but easier to obtain, will usually reflect high iron stores.

The diagnosis PCT requires excluding pseudoporphyria (PP), a condition also consisting of photocutaneous bullous lesions. PP can occur following sun exposure in patients who take known photosensitizing agents such as furosemide, 5-flurouracil, flutamide, isotretinoin, tetracycline, and naprosyn. The key to differentiating PP from PCT is that PP occurs without a significant increase in plasma, urinary, or fecal porphyrins. There have also been cases of hemodialysis associated pseudoporphyria; further emphasizing a need for laboratory confirmation in our case. (2)

In patients with PCT in the absence of renal failure, phlebotomy 250-500 ml 1-2 times per week) to decrease iron stores and antimalarials (chloroquine chelates porphyrins, makes them water soluble thereby allowing renal excretion) is the treatment of choice. Since chronic renal failure often coexists with chronic and sometimes severe anemia, high dose erythropoeitin (more than 150 U/kg 3 times a week) along with careful, very small volume phlebotomy (100 ml weekly) may be a promising option as reported in at least three case reports.

In contrast, case reports of PP in the setting of ESRD/dialysis suggest an easier treatment option having reported good results with the use of oral n-acetylcysteine (mucomyst).

The management of PCT in patients with renal failure is clearly a challenge that requires careful attention to other medications (avoidance of iron supplementation), frequent lab tests to help judge phlebotomy amounts and erythropoeitin dosage, and continued patient education. For these reasons, a team approach would be ideal.

Take Home Messages:
Bullae on the dorsum of the hands, especially those that heal slowly are pathognomonic for PCT or PP. They can also be seen in phytophotocontact dermatitis from the psoralen related compounds in certain plants (especially limes and lemons that are squeezed for drinks.) Recognizing this sign will lead one to the correct diagnosis.

References:
1. Shieh S, Cohen JL, Lim HW. Management of porphyria cutanea tarda in the setting of chronic renal failure: a case report and review. J Am Acad Dermatol. 2000 Apr;42(4):645-52.
The treatment of porphyria cutanea tarda (PCT) in patients with chronic renal failure poses a therapeutic challenge. In the absence of renal failure, phlebotomy and oral antimalarials have been the standard of care for PCT. However, in the presence of renal failure, associated chronic anemia often precludes the use of phlebotomy, and oral antimalarials are usually ineffective. We describe a patient with severe symptomatic PCT and chronic renal failure whose disease was successfully managed with a combination of high-dose erythropoietin and small volume phlebotomy. We also review several previously reported approaches to management of PCT in the setting of renal failure, which include small repeated phlebotomy, erythropoietin, deferoxamine, chloroquine, plasma exchange, high-efficiency/high-flux hemodialysis, cholestyramine, charcoal hemoperfusion, and kidney transplantation. An algorithm for the management of these patients is proposed.

2. N. S. Cooke, K. McKenna. A case of haemodialysis-associated pseudoporphyria successfully treated with oral N-acetylcysteine. Clinical and Experimental Dermatology 32, 64–66. (2007)
We report a 33-year-old woman with haemodialysis-associated pseudoporphyria successfully responding to treatment with oral N-acetylcysteine. We briefly review the current literature on bullous skin disorders in end-stage renal disease, and compare and contrast the pathogenesis of pseudoporphyria and porphyria cutanea tarda in this context. We also discuss the antioxidant properties and clinical applications of N-acetylcysteine, including the treatment of haemodialysis-associated pseudoporphyria.

3. A good review of PCT appears at: Emedicne/PCT but is not helpful for a patient with PCT and hemodialysis.

4. A good reference on pseudoporphyria

5. Valuable chapter on Bullous Disease of Diabetes

Perplexing Periodic Pruritus

This case was posted by David Elpern MD as a rapid publication for VGRD
We have been having technical problems with VGRD and have elected to publish this case here. Your comments will be appreciated -- please mail them directly DJ Elpern and we will post them to VGRD when it is up and running,

78 yo man with episodic incapacitating pruritus

Duration: 4 years

Distribution: Generalized, but sparing head and feet


The patient is a 78-year-old semi-retired music producer who presents with a four-year history of episodic intense pruritus that is generalized but has spared the face and the feet. It may have begun on the brachioradial areas of the arms but over a period of time spread to involve the sacrum, the chest, the back and the legs. He describes “a burning itch that you just cannot ignore.” At times he has “gone into a panic” over this. The itch can feel like “hot needles” applied to certain areas of the skin. He has also had scrotal pruritus for two years

The patient has seen a number of different physicians for this including four dermatologists. He has had biopsies done with nonspecific findings and direct and indirect immunofluorescence studies also have been noncontributory. There have been positive ANAs but no one has been able to correlate those with his pruritus.

His brother, has a similar problem and has been treated with CsA and azathioprine with good results.

Treatment:
1. multiple antihistamines -†no help.
2. multiple topical steroids -†no help.
3. Gabapentin - caused severe dizziness...unable to use.)
4. prednisone, three courses - resolved or alleviated symptoms.
(though less completely the second and third time.)
5. plaquenil, one month†- no help.
6. naltrexone, 2 weeks†- no help.
7. dapsone, 2 weeks†- no help.
8. pentoxifylline 400 qd x 2 weeks†- no help.
9. cellcept 2000 mg x 19 days†- no help.
10. Local heat hot compress seems to resolve it quickly though only temporarily).
11. Immuran

O/E: Excoriated papules – some crusted. Arms, legs, lower back, sacrum – sparing mid back, face. No vesicles or bullae.

Bx:
A superficial perivascular and interstitial lymphocytic infiltrate with scattered eosinophils and occasional neutrophils . Note : These changes are urticarial in nature. The histologic differential diagnosis includes urticaria or a dermal hypersensitivity reaction such as a drug eruption or insect bite reaction. Clinico-pathologic correlation is suggested.

Diagnosis: Uncertain. Possibly Subacute Prurigo

Reason Presented:
For diagnostic and therapeutic suggestions.. Have you seen similar cases? What has been the outcome?

DISCUSSION: All of his dermnatologists thought of D.H. but the biopsies have not confirmed this and dapsone and gluten free diet have not helped.

Please respond directly to DJ Elpern

Nail Abnormality in a young girl

This case was submitted by Dr Choon Siew Eng FRCP, a dermatologist from Johor Bahru, Malaysia

A 14-year-old Malay girl presented with a 7-month history of asymptomatic nail abnormality affecting all fingernails. Good general health. No previous of current skin problem or hair loss. No family history of skin problem. No personal or family history of atopy. No family history of autoimmune disease. No nail biting habit. Never varnish nails and no excessive wet work. No on any medication, traditional preparations or supplements.

All fingernails are abnormal ranging from mild horizontal lamellar dystropy, longitudinal, v-shaped, u-shaped and w-shaped sulci as shown in images with variable erythema at the edges of abnormalities. Majority of eponychium appeared normal. Lunula and periungual skin no capillary dilatation noted although some had streaky hyperpigmentation. No palmar erythema. Finger pulps, toe nails, scalp and oral mucosa normal. No other skin lesions

Laboratory: Fungal culture negative

Histopathology: Reject biopsy.

Diagnosis: Lichen Planus, Part of connective tissue disease,

Comments: Would appreciate help in diagnosis and suggestion on further investigations and management.

Questions: Am puzzled by how normal and healthy the unaffected parts of nails look. Had never seen such pattern of erythema. Have anyone seen similar abnormality? Is it justifiable to offer patient systemic steroid to prevent further nails damage and scarring without an histologic diagnosis?

Mistakes

Osler urged us to "Carry a small note-book, and never ask a new patient a question without note-book and pencil in hand......Begin early to make a three-fold category - clear cases, doubtful cases and mistakes. And learn to play the game fair, no self-deception, no shrinking from the truth; mercy and consideration for the other man, but none for yourself, upon whom you have to keep an incessant watch. It is only by getting your cases grouped in this way that you can make any real progress in your post-collegiate education; only in this way you gain wisdom with experience."

In this spirit, I present the case of G.K. an 82 yo woman who I have been following for 6 months for a pustulo-vesicular dermatitis of her feet. It had features of dyshidrotic eczema at only minimal control.

On August 31, 2007 the patient was reevaluated. Clinically and dermoscopically there was a suggestion of burrows and a scabies prep was taken. To my great surprise there were 4 - 5 mites and numerous eggs and feces in the mineral oil mount. She has no eruption of arms or torso and her only itching was on her feet.




I took this image from the web, but it shows what I saw -- a number of mites and eggs.

Diagnosis: Localized Norwegian Scabies. This patient is confined to a wheelchair, but is active and alert. Further history revealed that her grown children had scabies three years ago and all were treated (as was this patient) with 5% permethrin cream. Seemingly, they all got better, but then three years later this patient has a localized form of Norwegian scabies. There are only one or two case reports of localized Norwegian scabies, and none in patients like this.

Treatment: The patient, her husband and son who all live together will be treated with Elimite. She will have treatments every other day for two weeks for her feet and complete skin treatments twice, one week apart. She will be seen back in two weeks. A culture was taken for the question of secondary infection (this grew out coagulase sensitive Staph and she was placed on an appropriate antibiotic).

Over the years, I have been humbled by scabies time and again. Scabies localized to the plantar aspects of the feet is just the latest incarnation.

Addendum: Here is an article which addresses localized Nowegian scabies co-authored by the prolix Ted Rosen. Our patient is presumable immunocompetent.

Localised genital Norwegian scabies in an AIDS patient.
Perna AG, Bell K, Rosen T. Sex Transm Infect 2004;80:72-3.
OBJECTIVES: We present a case of an AIDS patient with Norwegian scabies manifest
by a single, crusted plaque localised to the glans penis. METHODS: A 45 year old
man with AIDS presented to our clinic complaining of a red papular pruritic rash
on his abdomen and anterior thighs and a single, thick, crusted, non-pruritic
lesion on the penis. He had been treated with lindane topically prior to the
development of the penile lesion without resolution of the pruritus or red
papular lesions. A mineral oil preparation was obtained from the hyperkeratotic
penile lesion and revealed numerous mite eggs and faeces. RESULTS: The diagnosis
of localised, genital Norwegian scabies was made. The patient was treated with
ivermectin 200 micro g/kg per dose taken as two doses, 14 days apart, with
complete resolution of both pruritus and skin lesions. CONCLUSIONS: This patient
is the first known report of Norwegian scabies localised as a single lesion on
the penis. He was successfully treated with oral ivermectin monotherapy.

Rosacea

This posting is from Liliana Rivis, a F.P. resident from Augusta, ME. It is intended for her colleagues as a learning experience, but others are welcome to read and comment if they wish.

The patient is a 43 yo CNA with a two year history of a facial eruption. She had tried metrolgel on a number of occasions and doxycycline for seven days at a time without significant improvement. She is a smoker( 1PPD x years).

O/E; She has an erythema on the cheeks, chin, bridge of the nose and up to the forehead. It spares the nasolabial folds and the hair line. On this erythematous rash are many red papules ( involving even the lower eye lid) covered with very dry skin. There are no obvious teleangiectasias.

She was started on doxycycline 50 mg PO QD for 1 month and I will see her in 2 weeks. I did not give her anything topical . I did not know if I can combine PO doxy and topical metrogel, and for how long. In Habif says to start doxy at 100-200 a day and taper off after rash is resolving.

Dave Elpern's Comments:
This is a woman with moderately severe rosacea. There's a good discussion on rosacea on eMedicine.com. Sometimes, one of the triggers is important (sunlight, hot liquids, alcohol, sunlight, etc.) but more often it's a combination of factors. If the patient is computer literate, I refer them to Dermnet.org.nz. A lot of this is moot because these patients usually respond to doxycycline or minocycline. Mostly start with 100 mg b.i.d. and taper after a couple of months. (Small patients of less than 50 kg may get dizzy on this dose of minocycline so start lower and increase over 1 - 2 weeks). Even tetracycline can work well with a starting dose of 500 mg bid or tid. During the summer, one needs to warn the patients against TCN and doxycycline because these can cause a phototoxic eruption, Minocycline only very rarely does this. If no response to a tetracyclihne in 2 - 3 months consider isotretinoin.

The seven days of doxycycline she had was not a long enough trial. Milder cases maay do well with topical metronidazole cream or gel (Metrogel, Metrocream, Noritate cream). Severe cases or those who do not respond to the tetracyclines should be considered for isotretinoin which usually works in doses lower than those employed for cystic acne. The biggest pitfall is not giving the drug enough time to work -- one must tell the patients to give it eight to twelve weeks. This patient needs to be encouraged to stick with the oral medication.
References:
1. eMedicine.com
2. Dermnet.org.nz

Evolving Lupus Variant?

Your thoughts are most welcome with regards to this patient. I don't have a firm diagnosis yet, but suspect this will turn out to be a form of lupus -- possibly subacute or acute LE..

HPI: A 47 year old woman was seen on June 11th, 2007 with with one month history of two 8 cm. plaques on her legs, KOH negative. Did not look like panniculitis. Initially thought to be Lyme disease, the lesions did not respond to doxycycline.

Because of progression of lesions and question of hypersensitivity disorder per biopsy the patient was treated with prednisone and the eruption subsided over a few weeks.

On 10 mg of pred a day, around 5 weeks after starting prednisone, the patient experienced a marked flare. This was during a long weekend at the beach. She said the weather was overcast and she wasn't out that much. Initial lesions recurred and there were some new papules and plaques on arms and legs. She has marked facial erythema and erythema of neck and upper chest. Other than pruritus, she feels well. No new meds.

Late August to Sept. 2007. Patient's eruption flared on legs and arms. On September 4, for the first time a definite butterfly rash on malar eminences. She feels well other than pruritus and has no arthralgias or constitutional symptoms. Sept. 5th, butterfly rash gone.

O/E: Erythematous plaques on legs. Facial erythema. Flushing of neck. Scattered papules and plaques legs and to a lesser extent arms. See photographs

6/30/07



8/28/07


9/4/07




Lab: 7/4/07 CBC and chemistries normal. ESR 24
8/28/07 Repeat CBC (normal) ESR 17, ANA + anticentromere 1:360
9/4/07 Repeat CBC, PLT normal, ESR still 17

Pathology: 6/30/07
Mild epidermal spongiosis with focal lymphocytic exocytosis and mild to moderate superficial and deep perivascular and interstitial lymphohistiocytic infiltrate with scattered neutrophils and rare eosinophils , extravasated erythrocytes and papillary dermal edema .

Pathologist's Note : These changes are consistent with an allergic dermal hypersensitivity reaction with mild vasculopathy, and may be seen in erythema chronicum migrans . These are not the changes of erythema nodosum . The differential diagnosis includes , in the appropriate clinical setting , a drug eruption or a pigmented purpuric eruption . P.A.S. stain is negative for fungal organisms. Clinico-pathologic correlation is suggested.

Pathology 8/27/07
Hyperkeratosis , focal parakeratosis , flattened epidermis , mild papillary dermal edema , ectatic blood vessels, a mild to moderately dense superficial and mid perivascular lymphocytic infiltrate with occasional plasma cells and extravasated erythrocytes .

Pathologist's Note : These changes are non-diagnostic . The differential diagnosis includes , in the appropriate clinical setting , a deep gyrate erythema, a collagen vascular disease , or possibly a drug eruption . The paucity of eosinophils does not favor the lymphocytic variant of urticarial vasculitis. P.A.S. stain is negative for fungal organisms. P.A.S. stain is negative for fungal organisms.

Repeat Biopsy: 9/4/07 Awaited.

Discussion: I think this patient most likely has subacute L.E. Other than her skin she feels well. There is a suggestion of photosensitivity from the facts that the process began in early June and exacerbated after visits to the beach and Labor Day weekend when it was warm and sunny. Her hemogram is normal, renal function and UA normal, no fever or serositis. I've watched this evolve over three months. It's been interesting and I could be wrong. I will start her on hydroxychloroquine and get an eye consult.

Your thoughts are important to me.

The Orphan Patient

Walter Shelley wrote this important poem about those patients who challenge all of us. We published it in Dermanities a while back and recently Bhushan Kumar from Chandigarh brought it to our attention again. Thank you, Bhushan! Some of the patients who find their way onto this Blog or VGRG

Here it is for those who may have missed it in Dermanities.

Prayer of the Orphan Patient to the Doctor

Walter B. Shelley
Dermanities May 5, 2007; 5(1)

Prayer of the Orphan Patient to the Doctor

Walter B. Shelley & E. Dorinda Shelley

Listen to me

Don’t be cynical, indifferent, or in a hurry.
Ask me what makes my problem better or worse.
Ask me what I think the cause is.
Ask me to look for clues and teach me what they are.

Think about me

Think of my problem when you read those books, journals, and atlases.
Think of my problem when you attend meetings.
Think of asking your colleagues about me.

Test me

Order specific tests to help you decide on my diagnosis and treatment.
Could I have AIDS, cancer, or lupus?
Do I need a biopsy? A challenge with a medication?
Do I need hospital help?
Do I need to see a consultant?

Don’t give up on me

There is always one more treatment you can try. Just imagine I have a different disease and treat me for that.
See me during an attack to get new ideas and new tests.
Ask me lots of questions during every visit.

I won’t give up on you, for I am an orphan

Mokihana Dermatitis

Presented by Caitlin Stiglmeier

I am a 23-year old woman with Type 1 skin who was gathering the fragrant mokihana berries in Kokee, Kauai’s mountain park. Twenty-four hours later, I developed a rash of red marks on my arms, half of which later developed into vesiculo-bullous eruptions. The bullae were left alone; the large blister on the forearm was accidentally burst; the remaining smaller eruptions eventually collapsed over a week’s period.

Pelea anisata, (common name: mokihana) is native only to Kauai, Hawaii, growing at elevations of 1,200 to 4,000 feet. The berries are used in lei-making. It is a multi-trunked tree that grows somewhat vine-like. The oils from its leaves and berries have an anise-like aroma and contain furocoumarin which is the cause of the phytophotodermatitis.

Mokihana is found in the mountain areas of Kauai


The berries are prised for their anise-like fragrance.


My Mokihana Dermatitis at day 5


One month later


Reference:
Elpern DJ, Mitchell JC. Phytophotodermatitis from mokihana fruits (Pelea anisata H. Mann, fam. Rutaceae) in Hawaiian lei. Contact Dermatitis. 1984 Apr;10(4):224-6.
Abstract: Bullous dermatitis, which resolved leaving hyperpigmentation and which was clinically consistent with phytophotodermatitis , affected the skin of the neck of 2 individuals who wore Hawaiian leis (as neck garlands ) made of the fruits of Pelea anisata . In a Daniels culture plate system portions of the fruits showed phototoxicity.