Friday, July 07, 2017

10 cm annular plaque in a 62 yo globe trotter

The patient is an otherwise healthy 62 yo man with an eight month history of an anympromatic plaque on his right arm.  His work takes him to places like the Atacama desert of Chile, Baghdad and Mosul, Japan, and Indonesia where he repairs specialized equipment in the field.  He was treated in a number of clinics with prednisone, various topical azoles and 40 days of grizeofulvin to no effect.

O/E:  The is a solitary 10 cm ring-shaped plaque on the right arm.  Sensaton to pinprick is normal.

Clinical Image:

Pathology:  A 4 mm punch biopsy was taken from the border of the ring. 
The specimen exhibits a superficial and deep interstitial proliferation of variably plump to spindle-shaped cells with mildly increased dermal mucin and a superficial and deep perivascular lymphocytic infiltrate with occasional plasma cells. The findings support the diagnosis of Interstitial Granulomatous Dermnatitis associated with a systemic disease.

Diagnosis:  Interstitial Granulomatous Dermnatitis (IGH) vs. Granuloma annulare.

The patient lives in an endemic region for Lyme disease.  His work takes him to Asia, South America, the Mid-East, and all parts of North America. Presently, he is in Iraq and will have a Lyme serology when he returns.  Certainly, the clinical picture could suggest erythema migrans, but an eight month history would be unusual.  There are a host of case reports of IGH in association with arthritis, collagen vascular disease, inflammatory bowel disease and even Lyme disease; but they are hard to make sense of.  (Guilt by association?) Although we see erythema chronicum migrans with regularity in New England, this lesion did not jump out as typical to me; but in retrospect, it could be the interface between IGH, GA and ECM. An old ECM?   Food for thought.  How long will the primary lesion of Lyme disease remain without treatment.  Eight months seems a long time.

References:
1. The expanding spectrum of cutaneous borreliosis.
Eisendle K1, Zelger B. G Ital Dermatol Venereol. 2009 Apr;144(2):157-71.
Abstract:  The known spectrum of skin manifestations in cutaneous Lyme disease is continuously expanding and can not be regarded as completed. Besides the classical manifestations of cutaneous borreliosis like erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans evidence is growing that at least in part also other skin manifestations, especially morphea, lichen sclerosus and cases of cutaneous B-cell lymphoma are causally related to infections with Borrelia. Also granuloma annulare and interstitial granulomatous dermatitis might be partly caused by Borrelia burgdorferi or similar strains. There are also single reports of other skin manifestations to be associated with borrelial infections like cutaneous sarcoidosis, necrobiosis lipoidica and necrobiotic xanthogranuloma. In addition, as the modern chameleon of dermatology, cutaneous borreliosis, especially borrelial lymphocytoma, mimics other skin conditions, as has been shown for erythema annulare centrifugum or lymphocytic infiltration (Jessner Kanof) of the skin.

2. Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern.  Peroni A et. al.  Br J Dermatol. 2012 Apr;166(4):775-83.
Abstract
BACKGROUND:
Interstitial granulomatous dermatitis (IGD) is a rare disease for which a clinical-pathological correlation is essential to establish diagnosis.
RESULTS:
All cases showed a predominant CD68-positive macrophage infiltrate distributed between collagen bundles of the mid- and deep dermis. Macrophages were also surrounding degenerated collagen fibres. A few neutrophils and/or eosinophils were also present. No vasculitis or significant mucin deposition was observed. Of the 62 cases of IGD reported since 1993, 53 fulfilled stringent diagnostic criteria. Erythematous papules and plaques on the trunk and proximal limbs were the dominant manifestation. Approximately 10% of patients had cord-like lesions. More than 50% of patients with IGD had arthralgia or arthritis, and less commonly other rheumatic disorders. Disease duration is months to years, but long-term prognosis seems favourable.
CONCLUSIONS:
IGD is a distinct entity with a typical histological and clinical pattern. The importance and the nature of the association with extracutaneous diseases remains to be clarified. Patients should be screened for rheumatic and autoimmune diseases.
 
3. Erythema migrans: a spectrum of histopathologic changes.
Wilson TC et. al. Am J Dermatopathol. 2012 Dec;34(8):834-7.
Abstract: Early cutaneous Lyme disease, erythema migrans, manifests as a gyrate erythema at the site of a tick bite. The standard histopathologic description is that of a superficial and deep perivascular lymphocytic infiltrate in which plasma cells are identified at the periphery of the lesion and eosinophils in the center. Deviation from these commonly accepted histopathologic findings may lead to an erroneous diagnosis. Herein, we describe 4 cases of erythema migrans, all biopsied at the periphery of the lesion and confirmed by serologic studies, demonstrating a variety of unconventional histopathologic patterns. These findings include eosinophils and neutrophils at the periphery of the expanding annular plaque of erythema migrans, focal interface change, spongiosis, involvement of the superficial vascular plexus alone, and an absence of plasma cells in all cases. These cases highlight the varied and nonspecific histopathologic changes that can be seen in erythema migrans, including the absence of plasma cells and the presence of focal interface change. Based on these findings, the dermatopathologist should always consider erythema migrans as a diagnostic possibility in a biopsy specimen from an expanding gyrate or annular erythema despite the presence of unusual features. In atypical clinical cases, serologic confirmation may be required for diagnosis in the presence of histopathologic findings considered unconventional for erythema migrans.


Saturday, July 01, 2017

Single Digit Leuconycia

The patient is a 64 yo school teacher who presents for a white nail.
Anamnesis: "The nail has been white for at least 6 months.  No other fingernails or toenails are white.  I had a ring on that finger that got super tight. I have finally removed the ring.  Sometimes the finger hurts, even now, like it may be arthritic."
The patient's questions are:
Is there something I can do to get it back to being a normal color?
Will it ever go back on its own to being normal - no longer white?

O/E:  The nail of the 4th finger of the left hand is uniformly white.  There is no flaking or friability.  Other than the color, the nail plate looks normal. All other finger- and toenails are normal

Photo:

Diagnosis:  Ring finger Leuconycia totalis.  This does not look like superficial white onycholysis.  At the time I saw the patient, I did not think to do a KOK prep since the nail plate looked so normal.

Thoughts: Single-digit leuconychia totalis has not been reported in the medical literature.  It is likely that prolonged ischemia may have played a role.
Dr. Eckart Haneke's comments were helpful: "Single digit leukonychia is just a description of the condition, not a diagnosis. I think this is due to the long-term poor blood supply of the finger. There is no similarity to superficial white onychomycosis. I cannot predict the future development. It may get slowly better, but this will take some more time. If you want to know something more about the situation you need a comparative demonstration of the blood circulation, e.g. by thermography or another similar method, of this and the contralateral finger. Diffuse leukonychia is sometimes seen in Raynaud's and other disturbances of blood supply."

Reference:
Leuconychia in reflex sympathetic dystrophy:
a chance association? Vanhooteghem O, et.al.
Br J Dermatol. 1998 Aug;139(2):355-6.
PDF of reference.


Leprosy, New York Times, August 31, 2108

We have come a long way since 1908; yet we still have a long ways to go.

Wednesday, June 28, 2017

Primary Lyme Disease

The patient is a 67 yo woman who presents for evaluation of a 14 cm diameter annular patch on her right lower abdomen,  No history of a tick bite, but there is an erythematous papule eccentrically placed in the patch.  She is a gardener who lives in an endemic area (Northeast U.S.A.)

This appears to be a good example of early localized Lyme disease


She was started on doxycycline, and if she tolerates it, will take it for two to three weeks.

Labs were ordered, mostly because she has a history of Lyme a few years ago, and I wanted to make sure that there was no likely co-infection.  

From Dermnet: Erythema migrans, a red expanding patch of skin, is the most typical sign of Lyme disease and is present in 70–80% of cases. It usually appears 7–14 days (range 3–33 days) after the infected tick bite. It starts at the site of the tick bite as a red papule or macule that gradually expands. The size of the rash can reach several dozens of centimetres in diameter. A central spot surrounded by clear skin that is in turn ringed by an expanding red rash (like a bull's-eye) is the most typical appearance. Erythema migrans may also present as a uniform erythematous patch or red patch with central hardening and blistering. The redness can vary from pink to very intensive purple.

Sunday, June 25, 2017

Black Dermographism

The patient, a 75 year-old man noted a blackish under his right sideburn for a couple of weeks.  He googled black marks on the skin and became anxious regarding the possibly of skin cancer, in particular melanoma.

O/E:  Dark greenish-black area with ill-defined borders.
When asked to see his eyeglasses:
an alcohol prep pad was rubbed over the area:
and the pigment disappeared:
The patient was embarrassed and left the office smiling and profoundly relieved.

Diagnosis:  Thisis an example of "black dermographism."
Cyberchondria exacerbated the patient's anxiety prompting the office visit.  Cyberchondria is a 21st Century illness syndrome that we see daily in our practices..


Comment:  From Visual Diagnosis on Black Dermographism
The term "black dermographism" designates the fact that under certain conditions a well defined black line appears where the skin is stroked with certain metals.This phenomenon was first reported by the Russians Emdin and Kusmenko1 in 1925. They contrasted the well known red and white dermographisms with black dermographism and stated that only the latter was to be regarded as a true one, i. e. "actual writing on the skin," since both the white and red dermographisms are due to stimulation of the nerve and muscle fibers of the blood vessels of the skin.According to these authors, the black "writing" which appears on the skin following application of certain blunt metallic implements with moderate pressure represents not a chemical but a physical process due to the particles of the metal rubbed off by friction and remaining on the skin. While they originally assumed that a hysterical condition.

References:
1. Black Dermographism
Erin Lowe, DO; Scott Lim, DO
JAMA Dermatol. 2017;153(3):352-353

Friday, June 16, 2017

The Fung Shui Nevus

Presented by: Micah Ashkenazi
Root Town, Ohio

The patient is a 19 year-old American-born, Chinese college student living in Ohio.  Her mother brings her in for an office visit regarding a nevus..  The 2 mm in diameter lesion has been present near the bulb of the nose for years with no worrisome changes.

Diagnosis:  Acquired melanocytic nevus.  Possibly a blue nevus.

Discussion:  I reassured the patient's mother that this is a benign lesion and can be safely observed.  The mother only speaks Cantonese.  She and her daughter have a longish conversation in Chinese.  The daughter tells me that her mother believes that moles on this part of the nose have bad fung shui.  In that case, I acquiesced and agreed to remove the mole which can be done with a 3 mm punch.  A shave may leave some residual pigment and that would be unacceptable to them.

The patient and her mother take fung shui seriously and want the lesion removed.  This will be scheduled at a propitious time as decided by their astrologer in Chinatown.

Clearly, this is a cultural issue, not a medical one.  Failure to consider that might have led to a disconnect between the patient, her family and the physician. How many other similar scenarios have I missed over the years?

Reference:
PubMed is strangely silent on Feng Shui; however there are many references on Google.

Face Reading Feng Shui in Chinese Five Arts.  It says, " If there is a mole on the nose, it will be bad luck between 40 to 50 years old."

                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                  

Wednesday, June 07, 2017

Between a Rock and a Hard Place

This is the saga of a man whose care poses therapeutic dilemmas.

HPI:  The patient is a 69 yo man with a greater than 40 year history of severe psoriasis.  He was a research subject at a prestigious university hospital for many years.  Over that time, he has received PUVA, methotrexate and an investigational drug, Rapamycin.  He has been treated with NB UVB and acitretin with some improvement.  As a result of his therapies, he has developed numerous squamous cell carcinomas.  These pose therapeutic challenges They are painful, foul smelling and difficult for the patient to care for.  An additional problem is occasionally disabling trigeminal neuralgia.  He has had many well-differentiated SCCs excised over the past decade.  He has atrial fibrillation, Factor 5 deficiency and is on warfarin and a host of cardiac medication.

O/E:  Wide-spread erythema and scale covering entire integument except head and neck.  There is a 3 cm exophytic tumor of the left pre-tibial area and smaller similar tumors on right leg, back and chest.

Clinical Photos (6.6.17)
L. Pretibial
Diagnosis:  Generalized psoriasis, Squamous cell carcinomas

Questions:  Should these lesions be excised? Should we consider  one of the new targeted therapies?  Do any of our members have expertise with them?  This man has a number of co-morbidities which may make therapy more difficult.

References:

Oral therapy for nonmelanoma skin cancer in patients with advanced disease and large tumor burden: a review of the literature with focus on a new generation of targeted therapies.
Rudnick EW, Thareja S, Cherpelis B. Int J Dermatol. 2016 Mar;55(3):249-58
Author information
Abstract: This review of the literature aims to describe previous and current treatment options for oral therapy in locally advanced and metastatic NMSC otherwise unamenable to standard treatment. Oral Smoothened (Smo) inhibitors Vismodegib, Sonidegib, and Taladegib have shown to be effective in several trials. Oral tyrosine kinase inhibitors Erlotinib and Gefitinib, which target epidermal growth factor receptor (EGFR), have early supporting data and are currently undergoing large multicenter trials. Oral therapy in NMSC is useful in high risk patients with recurrent and aggressive disease who may not tolerate other systemic therapies.

Sunday, May 28, 2017

A Woman with Fever, Malaise, Myalgias and Rash

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White Islands in a Sea of Red

Presented by Dr Henry B.B. Foong
from Foong Skin Specialist Clinic
Ipoh, Malaysia

A 45-year-old woman presented with a one-week history of fever and generalised rash. She initially had fever and sore throat and subsequently developed shoulder myalgia.  She saw her general practitioner who prescribed antihistamines and analgesics.  She then developed rashes on the legs.  Thinking this could be a drug eruption, the patient consulted her general practitioner again who did not think this was a cutaneous drug eruption.  Instead, he prescribed amoxicillin/clavulanate, but the rash spread further to her trunk and upper limbs.  The rash was only mildly pruritic.  She had no eye, oral or genital symptoms. There was no history of contact with patients with similar illness.

Physical examination showed stable vital signs with no dehydration. There was generalized confluent erythema distributed mainly on the legs but extending to trunk and upper extremities.  Prominent “islands of white” within the erythema over the lower legs were noted. It did not blanch completely, leaving behind petechiael lesions when pressed.

Clinical Photographs



Lab: Blood tests were positive for Dengue IgG and IgM antibody.
TWBC 5,100 (N38% L 36% M 22% E3% B1%) platelets 71,000 and Hct 38%

Pathology: 2 skin biopsies were taken from the right leg. One from the confluent generalised erythema and another from the white islands. Histopathology is pending

Diagnosis: Exanthem of Dengue Fever

Discussion
Dengue Fever ranks among one of the most important arthropod borne viral infections with high morbidity and mortality in the tropics and subtropics including urban populations. It is caused by Dengue virus, a flavivirus which is transmitted by mosquitos Aedes aegypti and Aedes albopictus.  There are 4 distinct serotypes DENV-1,2,3 and 4. Each episode of infection induces a life-long protective immunity to the homologous serotype but confers only  partial  and  transient  protection  against  other  serotypes.

The cutaneous manifestations of dengue fever are characteristic.  Skin eruptions appear in 80% of patients during the remission of the fever. Approximately one-half of patients develop a centrifugal macular, maculopapular, scarlatiniform, or petechial eruption. The cutaneous eruptions may become confluent, with small, round islands of sparing, the so-called white islands in a sea of red. The rash characteristically starts on the dorsum of the hands and feet and spreads to the arms, legs, and torso; the face is rarely involved. The eruption seen in dengue is usually short-lived, lasting from a few hours to several days.

Reference:

from eMedicine reference