Thursday, August 03, 2017

Labial Melanocytic Macule


The patient is a 62 yo woman who presents with an 18 month history of a pigmented macule on the lower lip.  She has noted no change over time.  No history of skin cancer.

O/E:  There is a 6 mm in diameter dark tan macule on the lower lip.  Dermatocopic exam shows pigmented dots.

Photos:
Diagnosis:  This appears to be a Labial Melanocytic Macule.

Discussion:  There are only very few references that describe the dermatoscopic appearance of these lesions.  Those I found were solitary case reports.  The brown dots may indicate the lesion is in the active growth phase.  I offered to biopsy the lesion, but  told the patient it was likely a LMM and is shepreferred, we could follow her up in 3 months.

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?

Post 3 mm punch bx


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

-->
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


Friday, May 26, 2017

Atypical Melanocytic Lesions

The patient is an 87 yo man with a history of dysplastic nevi and melanoma-in-situ.  He is seen every four to sic months.  The current exam found a 1.5 cm atypical melanocytic lesion on his left mid back.

Clinical and Dermatoscopic Images

What are your thoughts?

Sunday, May 21, 2017

PVLE Variant?

May 12, 2017
The patient is a 75 yo man with a 7 day history of a pruritic papular eruption on his legs.  This began on a recent trip to Florida after sun exposure.  He is on no photosensitizing medications.

O/E:  Erythematous non-blanching papules on both lower extremities.

Clinical Images:
Lab:  CBC, ANA normal

Pathology:  No evidence of vasculitis

Diagnosis:  Could this be an example of Papulo-vesicular Light Eruption?

References:
1.  Papulovesicular light eruption. A defined subset of polymorphous light eruption.
Elpern DJ, Morison WL, Hood AF.  Arch Dermatol. 1985 Oct;121(10):1286-8.
Abstract:  A distinctive photodermatitis is seen commonly in tourists visiting Hawaii. Analysis of 150 cases revealed that the eruption is acute in onset following exposure to sunlight, is confined to exposed areas, and mainly affects young to middle-aged white women. The clinical presentation is remarkably uniform, consisting of papules, papulovesicles, or vesicles. These findings are consistent with a diagnosis of polymorphous light eruption (PMLE) and the histologic picture supports this diagnosis. Other clinical variants of PMLE were not seen in our patients, however, which suggests that this condition is more monomorphous than polymorphous. We suggest papulovesicular light eruption as a suitable name for this common and distinctive subset of PMLE. 

2.  Papulovesicular light eruption. A defined subset of polymorphous light eruption.
Elpern DJ, Morison WL, Hood AF. Arch Dermatol. 1985 Oct;121(10):1286-8.
Abstract: A distinctive photodermatitis is seen commonly in tourists visiting Hawaii. Analysis of 150 cases revealed that the eruption is acute in onset following exposure to sunlight, is confined to exposed areas, and mainly affects young to middle-aged white women. The clinical presentation is remarkably uniform, consisting of papules, papulovesicles, or vesicles. These findings are consistent with a diagnosis of polymorphous light eruption (PMLE) and the histologic picture supports this diagnosis. Other clinical variants of PMLE were not seen in our patients, however, which suggests that this condition is more monomorphous than polymorphous. We suggest papulovesicular light eruption as a suitable name for this common and distinctive subset of PMLE.

A Case for Diagnosis

The patient is a 58 yo woman with asymptomatic lesions under breasts, in the axillae and groin.  She is on no medications,  She has breast cancer seven years ago treated with lumpectomy and chemotherapy (no radiation) and has been off therapy for 6 years.

O/E:  slightly hyperpigmented macules in affected areas.  KOH negative.

Clinical Photos:
Left axilla
Pathology:  2 representative punch biopsies were taken. 
Report: Focal basal layer vacuolization, a mild superficial perivascular and focally band-like lymphocytic infiltrate with rare eosinophils, papillary dermal fibrosis, numerous melanophages, and telangiectasia, consistent with a lichenoid interface dermatitis with post-inflammatory hyperpigmentation.



NOTE: The differential diagnosis could include lichen planus (possibly lichen planus pigmentosus) or a lichenoid drug eruption.

Diagnosis: Lichen Planus Variant.  Is this annular atrophic lichen planus?

Reference:
Annular atrophic lichen planus.

Morales-Callaghan A Jr1, et. al. J Am Acad Dermatol. 2005 May;52(5):906-8.
Abstract: We describe a new case of annular atrophic lichen planus. Annular atrophic lichen planus is a rare variant of lichen planus that is clinically characterized by violaceous plaques of annular morphology with central atrophy and histopathologic findings of annular and atrophic lichen planus, respectively. There have been 4 cases of this entity reported previously.

Saturday, May 20, 2017

Recessive X-Linked Ichthyosis

The patient is a 65-year-old engineer who presents for evaluation of skin lesions.  He grew up in a Southern state and during high school and college worked summers doing roadwork.  He wants a screening for skin cancer. The patient has had a B-cell lymphoma 3-4 years ago. 

He has had a form of ichthyosis since about age 2.  Other family members are affected.  They are all on his mother’s side.  His mother’s brother and some male maternal cousins are similarly affected. He always felt like an outsider in his school, and was bullied in grade and high school because of this scaly skin.  This has informed his self-image as an adult.

EXAMINATION:  A complete skin examination was done. He has scaly polygonal, somewhat “dirty-appearing” plaques on the legs, torso, and arms.  He has normal palms.  Head and neck are normal.  He has no lesions that are worrisome for melanoma, nonmelanoma skin cancer, or actinic keratoses. 

Clinical Images:  

Polygonal Scale
Normal Palm in X-Linked Ichthyosis
IMPRESSION:  Recessiive X-linked ichthyosis.

PLAN:  We discussed his ichthyosis.  One can get free Aquaphor from the drug company and we will order some for him. 

I will run his case by some pediatric dermatology colleagues.  I do not know whether any further testing is needed for a 65-year-old man who is not going to have any children.  

Corneal opacities are relatively common in these patients and may be evident with slit-lamp examination both of adult males who are affected and of women who are carriers. Cryptorchidism has been reported in 20% of patients. A few cases of testicular cancer have developed in patients with X-linked ichthyosis and cryptorchidism.

Reference:
FIRST: The Foundation for Ichthyosis and Related Skin Types is a great resource for patients and providers.  See FIRST’s page on Recessive X-Linked Ichthyosis.







Saturday, May 06, 2017

60 yo man with scaly rash upper chest

This 60 yo disabled man has had a sic moth history of a mildly pruritic eruption of his upper chest.  I expected the KOH prem to be postive, but it was not.  He has lots of psychosocial problems (wife an alcoholic, daughter a heroin addict) -- not enough time to properly evaluate; so a biopsy was done.  If that is negative for fungal elements I would think about Casal's necklace whigh I have never seen.

Pathology:  The biopsy showed a mild superficial spongiotic dermatitis with no evidence offungal elements.

Follow-up:  When seen back for suture removal, another KOH prep was done.  It was positive for numerous septate hyaline hyphae.  The original KOH prep was taken from the advancing scaly border as was the biopsy.  Sampling error caused the misdiagnosis.  The patient was started on terbinafine 250 mg daily for six weeks.  Follow-up photos will be posted when he returns.

Thursday, April 27, 2017

63 y.o. Man with P.R.-like Eruption

The patient is a 63 yo man with a one month history of an asymptomatic eruption on the torso.  He has a history of non-Hodgkin's lymphoma treated successfully four years ago.

O/E:  Healthy-appearing man with Type III skin.  He has .5 to 1 cm in diameter plaques on his torso and proximal extremities.  On his left forehead, he has three follicular papules.

Clinical Images


Comment:  This looks banal; but considering the patient's history biopsy is necessary.  Two representative lesions were sampled with 4-mm trephines.

Pathology: Biopsy showed no atypical features and this most likely  represents Pityriasis rosea in a somewhat immunocompromised man.  Since his lymphoma treatment, his white count is ~ 1800 - 2000 but he feels well otherwise.

Monday, April 10, 2017

Pruritic Nodules

The patient is a 32 year old Hispanic woman with a 2 year history of pruritic nodules on her knees, right ankle and dorsum of right hand.Her health is good otherwise and she takes no medications by mouth.  She denies kneeling, rubbing or manipulating the skin.  Her primary physician has give her various topical steroids which were not effective.

O/E:  There are a few hyperpigmented nodules in these areas.  She has Type IV skin.

Clinical:
Lab:  None

Diagnosis: Similar lesions have been seen after  trauma from habitual pressure from kneeling, surfing, praying: but thepatient denies these.

I am not hopeful about impacting here.  What thoughts do you have?  Are any lab tests or biopsy indicated?

Thursday, April 06, 2017

78 yo man with scalp lesions

Abstract:  78 yo man with 1 year history of scalp lesions.

HPI:
May 9,2016:  Cryotherapy 4 keratoses on vertex of scalp
August 11, 2016: Crusted lesions at site of cryotherapy.  Clinical diagnosos of erosive pustular dermatosis of scalp made.  Treated with mupirocin oint and clobeyasol ointment  Initially improved.
Sept 9 2016:  Resolved
10/24/2016: Continued to do well
April 4, 2017: Recurrent lesions on vertex of scalp.  Thick crusted lesions (see photo)  The crusts were brownish and dirty looking, but unfortunately I removed them before taking the photo of April, 2017.

O/E:  What was initially hypertrophic keratosit papules were transformed into ~ 1 cm crusted erosive lesions.

Clinical  Photos:
Note pustule
4.4.17 (after crusts removed)
s/p 1 week Chlorhexidine, 4 days tacrolimus 0.1%

Lab:  August 11, 2016: + Staph aureus from lesion Vx scalp - usual sensitivities

Diagnosis:
This is either erosive pustular dermatosis of the scalp or squamous cell ca.  The rapid worsening since cryotherapy suggests the former.  It appears that biopsy may be necessary.  Not all cases of EPD respond to clobetasol ointment.

Questions:  Is this EPD or are these lesions squamous cell carcinomas.  Patient is reluctant to have a biopsy done. This process appears to have been gtriggered by the trauma of liquid nitrogen and did respond initially to clobetasol.  10 - 20% of EPD cases appear to be non-responders ro clobetasol.

Follow-up.  Marked improvement following chlorhexidine wash daily and topical tacrolimus 0.1% ointment at the suggestion of a colleague.  He recurred after clobetasol ointment.

References:
1. Erosive pustular dermatosis of the scalp: Clinical, trichoscopic, and histopathologic features of 20 cases.  [Current and thorough review]
Starace M, et. al.: J Am Acad Dermatol. 2017 Feb 14.
BACKGROUND: Erosive pustular dermatosis of the scalp is a chronic eruption that leads to scarring alopecia.
OBJECTIVE: The clinical, dermoscopic, and histopathological features and the course of the disease in 20 patients were reviewed and compared with the reports in the literature.
RESULTS:The mean age was 59.4 years. Androgenetic alopecia was present in 12 patients, 6 of whom showed actinic damage. Trauma was reported in 9 patients. Four patients were affected by autoimmune disorders. The vertex was the most common location. In all 20 patients trichoscopy showed an absence of follicular ostia with skin atrophy. Histopathology revealed 3 different features, depending on the disease duration. A reduction of inflammatory signs was observed in 14 patients treated with topical steroids and in all 3 patients treated with topical tacrolimus 0.1%.
CONCLUSIONS: The relatively high number of patients allowed us to identify a better diagnostic approach, using trichoscopy, and a more effective therapeutic strategy, with high-potency steroids or tacrolimus, which should be considered as first-line treatment.

2.  Disseminated Erosive Pustular Dermatosis also Involving the Mucosa: Successful Treatment with Oral Dapsone (Free Full Text)
Jamison D. Feramisco.  Acta Derm Venereol. 2012 Jan; 92(1): 91–92.

3.  Erosive pustular dermatosis of the scalp: a review with a focus on dapsone therapy.
Broussard KC. J Am Acad Dermatol. 2012 Apr;66(4):680-6
Abstract
BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory disorder of unknown origin characterized by pustules, erosions, and crusting in areas of alopecia that tend to be atrophic, actinically damaged, or both. The most common treatments reported include antibiotics and topical anti-inflammatories, which can be ineffective. In the search for effective treatment for EPDS, we share our experience with topical dapsone 5% gel.
OBSERVATIONS:We present 4 patients with EPDS, all with classic clinical presentations and histologic findings of EPDS, who had failed a variety of treatments including oral, intralesional, or topical steroids, tacrolimus, and antibiotics. All patients demonstrated rapid improvement or resolution with topical dapsone 5% gel.
CONCLUSION: Our observations demonstrate topical dapsone 5% gel to be a novel, safe, and efficacious therapeutic alternative for mild to moderate EPDS.

Monday, April 03, 2017

Herpes Zoster in a 10 year-old



The patient is an otherwise healthy ten year-old boy with a two day history of grouped zosteriform vesicles on the left arm, anterior shoulder and upper back.  He has mild discomfort.  No known illnesses and on no medication.  He had two immunizations for varicella at the approopriate ages.

Observation:  All of the grouped vesicles appeared to be of uniform size.

Lab:  A Tzanck smear was positive for multinucleated giant cells.

Diagnosis:  Herpes zoster roughly C3 - 6.

Herpes zoster in children is unusual but not all that rare.  There are a few cases of HZ after vaccination for varicella.  As he felt well, and as the effect of specific antiviral therapy is not striking; after discussion with his mother it was elected to simply follow.  In out opinion, in healthy children and young adults the course of HZ is usually relatively mild and almost never followed by post-herpetic neuralgia.  Immunity seems to wear off over time and it appears that this attenuated vaccine is capable of causing H.Z.  The other possibility is that this is zosteriform simplex.  We did not culture for that. 
While researching this case, we looked up "zosteriform simplex."  An observation (ref 4) indicates that in these patients the vesicles are of uniform size (as we see here).  This would tilt us towards a diagnosis of Zosteriform Herpes Simplex here, and not of vaccine failure.  Should this child get a recurrence, that would clinch the diagnosis.


Reference:
1. Herpes zoster in children.
Peterson N, Goodman S, Peterson M. Cutis. 2016 Aug;98(2):94-5.

Abstract:

Herpes zoster (HZ) in immunocompetent children is quite uncommon. Initial exposure to the varicella-zoster virus (VZV) may be from a wild-type or vaccine-related strain. Either strain may cause a latent infection and subsequent eruption of HZ. We present a case of HZ in a 15-month-old boy after receiving the varicella vaccination at 12 months of age. A review of the literature regarding the incidence, clinical characteristics, and diagnosis of HZ in children also is provided. 
 
2. Herpes zoster and zosteriform herpes simplex virus infections in immunocompetent adults.

Kalman CM, Laskin OL. Am J Med. 1986 Nov;81(5):775-8.

Abstract: Among 111 immunocompetent patients referred to a general hospital setting with the clinical diagnosis of herpes zoster, viral cultures were obtained from 47 patients. Six of these patients (13 percent) had herpes simplex virus isolated, with four of the six infections involving the facial distribution, and the other two involving the T4 (breast) distribution. Excluding those in whom herpes simplex virus was isolated, the mean age (+/- SD) of the remaining 105 patients was 50 +/- 19 years. Thirty-two percent of the patients were at least 65 years old; however, 39 percent were younger than 40 years of age. Thus, herpes zoster frequently occurs in young, immunocompetent adults. Also, since zosteriform rashes may be caused by herpes simplex virus, viral cultures of lesions are useful to differentiate infections caused by herpes simplex virus from those due to varicella-zoster virus. The need to distinguish between these two viruses may be important with the advent of antiviral drugs and for use of the proper epidemiologic isolation procedures.
3. Varicella Vaccine (Wiki)
Vaccines are less effective among high-risk patients, as well as being more dangerous because they contain attenuated live virus. In a study performed on children with an impaired immune system, 30% had lost the antibody after five years, and 8% had already caught wild chickenpox in that five-year period.


4. Zosteriform herpes simplex and herpes zoster: A clinical clue

Sanath Aithal, Sheela Kuruvila, and Satyaki Ganguly. Indian Dermatol Online J. 2013 Oct-Dec; 4(4): 369.  Free Full Text.

Excerpt: An important clinical observation by many authors that the vesicles of herpes simplex are uniform in size in contrast to the vesicles seen in herpes zoster, which vary in size. In other words, vesicles of herpes simplex are uniform within a cluster.