Friday, November 02, 2018

Parameatal Cyst


Presented by:
Caitlin Hogue, MBChB
The Launceston Skin Centere
Launceston, Tasmania

A 10 year old boy presented to our clinic with a two month history of an asymptomatic lesion adjacent to the urethral meatus.

O/E:  There is a 4 mm in diameter cystic lesion in the above-described area.

Clinical Photo:

 Diagnosis:  Parameatal Cyst

Plan: This is an unusual lesion.  Although the definitive treatment is surgical excisison, a small lesion may be observed.  Little is known about the behaviour of these uncommonly observed lesions.  We will try to identify a paediatric urologist who has some expertise with these lesions.

Reference: Parameatal Cyst: A Presentation of Rare Case and Review of Literature. Lal S and Agarwal Ankur. J Clin Diagn Res. 2013 Aug; 7(8): 1757–1758.  Free Full Text.
Abstract: A parameatal urethral cyst is a very rare congenital anomaly. It was first reported in two males in 1956 by Thompson and Lantin. About 50 cases have been published since then. Most of the cases which have been reported were from Japanese population and on extensive literature search, few cases were found to have been reported from India. We are reporting a case of a parameatal urethral cyst in a 7-year-old boy. Complete excision of the cyst with total removal of the epithelium is required for treatment and for prevention of recurrence.



Monday, October 01, 2018

Erosive Pustular Dermatosis of the Scalp

The patient is an 81 yo man with a long history of chronic lymphocytic leukemia.  His white counts are ~ 130,000 and platelets ~ 60,000.  He has had scores of nonmelanoma skin cancers (mostly squamous cell carcinomas) on the head and neck, torso and  all four extremities.  

He presents with yellow-brown crusts over his bald pate.  These have a mousy, earthy odor.  They were gently debrided and the subjacent areas were erosions.

Bacterial Culture:
3+ Pseudomonas
3+ Staph aureus

Clinical Images:

After initial debridement and chlorhexidine scrubs and mupirocin ointment for 33 days

Diagnosis:  Erosive Pustular Dermatosis of the Scalp

Plan: 
References:
  1. Thuraisingam T1, Mirmirani P. Erosive Pustular Dermatosis: A Manifestation of Immunosenescence A Report of 8 Cases. Skin Appendage Disord. 2018 Aug;4(3):180-186\
Abstract: Erosive pustular dermatosis (EPD) is a rare condition of the scalp and legs that is marked by crusted erosions or superficial ulcerations that may result in scarring alopecia and chronic wounds. The condition predominantly affects elderly female as compared to male patients. Its pathogenesis remains poorly understood. The majority of the cases in the literature are from the United Kingdom and continental Europe. In this series, we present 8 North American patients with EPD of the scalp, one of whom also had involvement of the legs and another with the involvement of the face. All our patients were advanced in age and had a predisposition to chronic actinic damage, which are common characteristics of EPD previously reported in the literature. We hypothesize that immunosenescence leads to an aberrant immune response to wound healing and, along with other factors such as a loss of the normal epidermal barrier, ultraviolet damage, and hormonal factors, may contribute to the development of this condition.

2 Wilk M1 et. al. Erosive pustular dermatosis of the scalp: reappraisal of an underrecognized entity. J Dtsch Dermatol Ges. 2018 Jan;16(1):15-19.
Abstract: Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory dermatosis of unknown etiology. Herein, we present a review of the disease and report our own clinical and histopathological experience in eleven patients. EPDS tends to spontaneously affect bald areas of the scalp in elderly individuals. A history of previous surgery at the same site - as observed in four of our patients - is common. Coronary artery disease, cerebrovascular insult, arterial hypertension, diabetes mellitus, and severe cases of cancer were frequent comorbidities. Most patients show an undulating clinical course despite topical anti-inflammatory treatment; in some individuals, the lesions heal with scarring. Histopathology reveals scaly crusts or erosions and granulation tissue-like changes in the dermis, evolving into a scar in more advanced stages. Apart from actinic/local damage, impaired immunity and microcirculation may be predisposing factors of the disease. Similar to pyoderma gangrenosum, EPDS must be considered in the context of nonhealing wounds in the elderly after the differential diagnoses mimicking EPDS have been ruled out. Given that previous or concomitant adjacent basal cell or squamous cell carcinoma is a common finding and that infiltrative variants extending beyond the clinically visible tumor may occur, histological mapping of the surrounding skin may be advisable in doubtful cases.

Saturday, September 22, 2018

Facial Eruption in a 2 year-old

Presented by Dr. Hamish Thain,  Dundee, Scotland
22 September, 2018

The patient is a 2 yr and 10 month old girl with a few month history of a facial eruption. It began as hive-like plaques but has evolved over the past few weeks.  Her paternal grandmother has Alpha-1 Antitrypsin disease and the child is a carrier.  No family history of collagen vascular disease.  She takes no medications save vitamins.

EXAMINATION:  The examination shows an erythematous papular and nodular eruption on both malar eminences and the left lower lid.   The remainder of the cutaneous exam is unremarkable.

Clinical Photos:
Lab:
CBC, BUN, ANA: all normal or negative.  ESR 7
Urine analysis normal

Pathology:  The slides were read by Deon Wolpowitz and the photmicrographs were taken by Erin Tabata, both of Boston University Department of Skin Pathology.
Intermittent compact hyperkeratosis with parakeratosis, intermittent basal layer vacuolization with squamatization of the basal cell layer and occasional individually necrotic keratinocytes and mild lymphocytic exocytosis, papillary dermal edema that is focally prominent in a few papillae, ectatic blood vessels, and a moderate superficial and deep perivascular and interstitial, and focally periappendageal, lymphohistiocytic infiltrate with few extravasated erythrocytes. 


Diagnosis:  Polymorphous Light variant.  This is not the common papulovesicular variant of PMLE.

Plan:  Initially, she will use broad-spectrum sun-blocks containing zinc oxide or titanium dioxide and a large floppy hat whilst outdoors.  As the season changes, and the light wanes, she should improve.  Topical corticosteroids, calcineurin inhibitors, and hydroxychloroquine will be considered if her parents want further therapy.  Phototesting at a centre is disruptive for this child, at this time.

What are your thoughts.

References
1.Alexis L. Dougherty, Cloyce L. Stetson, MD, Dr. Khachemoune. What are These Facial Plaques in a 4 year-old Child.  The Dermatologist. 8.20.2013  Link

Saturday, September 15, 2018

85 yo man with sinus tracts for diagnosis

Presented by: Dr. Neha Sagar,
Resident physician, Chhattisgarh, India

The patient, an 85 yo man with no known past medical history, presented to our OPD with large lesion with sinuses tracts on back since past 4 years. 

The lesion appeared as small sinus tracts with swelling over the right sided back.  These were pruritic in nature with  slowly & progressively increasing in size over 1 year & the lesion has not increased in size since past 3 years.   There was no history fever or cough or any other lesions noted.  He is not diabetic nor hypertensive.

No h/o specific medicinal intake or food allergy noted by the patient preceding the back lesion.

On examination:
A large lesion of approx 25 x 15 cm in size papular in nature with multiple discharging pustules noted over the same; scratch marks are present..
Swelling firm in consistency; & skin over the lesion is non-adherent to the lesion; Lesion is freely mobile over the base of scapula & muscles.

Clinical Photos:


 There are no specific systemic finding.

We approached this skin lesion as mycetoma or fungal infection; Scrofuloderma was also one of the D/D. But the skin biopsy revealed “Lobular Capillary Hemangioma.”

Path Report:
What other D/Ds can this lesion be?? 

How should we treat such lesion? How long can we treat this patient medically??
What specific counselling in terms of resolution of skin lesion should be done for the patient?
When can we consider surgical option (? wide excision); if any? What are the specific indications of the surgical removal of the lesion? 


Wednesday, July 11, 2018

Granulomatous Rosacea

A 34-year-old sociologist presented for evaluation of forehead lesions, which have been present for about 2 years.  These began about a year after her daughter's birth.  Before that, she was on oral contraceptives and was fine, but she has not been on any hormonal birth control since then.  She saw another dermatologist and was treated with topicals, a SilkPeel, Tretinoin.  She also took doxycycline for 2 – 4 weeks. Nothing helped.  She is anxious about her appearance. 

O/E:   The examination shows a pleasant, outgoing woman.  She has a somewhat pebbly appearance to the forehead with many, mostly not inflammatory discrete and confluent submillimeter papules.  There were a few erythematous papules.

Clinical Photos:


Initial Diagnosis:  I considered an acneiform eruption.  A 4 mm punch biopsy was performed.

Pathology:  Thanks to Assistant Professor Hye Jin Chung, MD from Boston University Skin Pathology for kindly providing these beautiful photomicrographs.
There is a moderate and superficial perivascular and perifollicular infiltrat.  Focal granulomas formation is noted.
                          


Presumptive Final Diagnosis: Granulomaous Rosacea
  
Discussion:  Is this really a subset of rosacea, or is it an acneiform disease sui generis? Clinically, it does not look like rosacea and it appears to be defined by dermatopathologists who only see small plugs of skin.  Similarly, perioral dermatitis is an acneiform disorder of uncertain etiology, but the diagnosis is strictly clinical.

References:
1. Lee GL, Zirwas MJ. Granulomatous Rosacea and Periorificial Dermatitis: Controversies and Review of Management and Treatment. Dermatol Clin. 2015 Jul;33(3):447-55.
Abstract: Granulomatous rosacea and periorificial dermatitis are common skin conditions affecting the face. This article examines the historical origin, causes, clinical presentation, and management strategies for these entities.  Link to Full Text.

2.
Omar Khokhar and Amor Khachemoune. A Case of granulomatous rosacea: Sorting granulomatous rosacea from other granulomatous diseases that affects the face. Dermatology Online Journal 2004 10 (1): 6  Free Full Text.
Abstract: Granulomatous rosacea is a variant of rosacea that may present similar to other granulomatous diseases. We present the case of a 45-year-old woman with a 2-year history of facial erythema with multiple papules and pustules on the cheeks, chin, and glabella. The patient responded to minocycline, resulting in healing 6 months without residual scarring. This patient's clinical and histological presentation and treatment outcome are to our assessment consistent with granulomatous rosacea. However, other clinically and histologically related entities will be discussed. These entities include, but are not limited to, perioral dermatitis, granulomatous periorificial dermatitis, lupus miliaris disseminatus faciei, facial afro-caribbean eruption syndrome, and sarcoidosis.


Sunday, July 08, 2018

Solitary Tricholemmoma

The patient is a 39-year-old home visitor who presents for evaluation of a lesion on the bulb of
the nose that has been present for about six months.

O/E: The examination shows a healthy, outgoing woman with type I skin. There is a solitary 3 mm in diameter dome-shaped papule with a central keratin on the bulb of the nose. The remainder of the cutaneous examination is unremarkable.
Clinical and Dermatoscopic Images: 

Preoperative diagnosis: This has the appearance of keratoacanthoma, squamous cell carcinoma, or hypertrophic actinic keratosis.

PLAN: The lesion was shave excised. The base was lightly electrodesiccated and curetted and a
specimen was submitted for pathology.

Pathology: Assistant Professor Hye Jin Chung, MD from Boston University Skin Pathology kindly provided the photomicrographs.

At x4:  parakeratosis, papillomatosis and a lobular proliferation of pale cells
At x10: peripheral palisading with focal eosinophilic hyaline basement membrane
Diagnosis:  Solitary Tricholemmoma

Daignosis:  Solitary tricholemmoma.  Tricholemmomas can be a marker for Cowden's disease.  However, I don't feel that a solitary tricholemmoma is a red flag in a 39 yo woman.  I asked the patient about a personal or family history of cancer and there was no history of thyroid, lung or colon.

This case is presented because there are few to no accessible cases of solitary tricholemmoma online and no dermatoscopic images that I could fine..

Reference:

Spiegel JH, Khodai N. Tricholemmoma of the nose. Am J Otolaryngol. 2006 Nov-Dec;27(6):430-2.

Abstract

OBJECTIVES: The objectives of this case report are to (1) identify clinical presentations of tricholemmoma, (2) discuss the characteristics of Cowden syndrome and the relationship between this syndrome and tricholemmoma, and (3) differentiate tricholemmoma from other superficial cutaneous tumors such as basal cell carcinoma and granulomas.

RESULTS:

Tricholemmoma was first described as a cutaneous neoplasm in 1962. It is associated with the Cowden syndrome and can be misdiagnosed as a more aggressive cutaneous malignancy. We report an unusual case of tricholemmoma presenting as a nasal mass.

CONCLUSIONS: Tricholemmoma is described as having a predilection for the head and neck, yet little information has been published about this disorder within the Otolaryngology literature. Otolaryngologist-head and neck surgeons must be familiar with this neoplasm because it can be frequently misdiagnosed as an aggressive cutaneous malignancy and hence can be incorrectly treated. We describe the presentation and diagnosis of tricholemmoma, describe the frequent association of this neoplasm with Cowden syndrome, and report an unusual presentation of tricholemmoma as an intranasal mass.

Tuesday, July 03, 2018

Renal Transplant Patient with a Black Toe

The patient is a 70 yo man who had a renal transplant around 10 years ago.  His immunosuppression consists of oral tacrolimus and prednisone, and he is seen annually by a dermatologist.  He had a 3 cm superficial squamous cell carcinoma ofhis scalp 5 years ago that was treated successfully with topical 5FU.

O/E: The current exam revealed a black area under the nail of the second toe on the left foot.  He said it's been like his for around a year and has not changed much.

Clinical and Dermatoscopic Images:
Diagnosis and Discussion:
While I am pretty certain that this is a subungual hematoma; if his history is accurate that may be worrisome.  There is little downside to removing the nail and biopsying the nail base if it is pigmented and this was scheduled in a week's time.  The fact that this is Morton's toe also favors subungual hematoma.

Thursday, June 21, 2018

44 yo woman with facial erythema


HPI: The patient is a 44 yo health care professional with an 18 month history of erythema of the chin and perioral area.  She has been seen by four dermatologists who have treated her for rosacea and perioral dermatitis with doxycycline and various topicals.  Nothing has helped.  The process began after her  mother died.  She lives at home with her boyfriend of 16 years and their two preteen children.  
She admits to being anxious and depressed as there are significant social problems at home.
She used a topical steroid for a few days when her lips were prurituc, not for weeks to months.
She takes no medications p.o. other than Xanax 0.25 mg h.s. There is no history of using a mask or any local contactant to this area that might explain this pattern.

O/E:  Shows a light-complected Caucasian with sharply demarcated erythema and mild scaling of the chin, and submental region.

Clinical Images:

 Patch Testing: True Test negative at 96 hours

 Discussion:  This is a perioral rash that does not look like perioral dermatitis.  The pattern suggests a contact dermatitis, but the history and patch testing do not corroborate that.  Perhaps, one of our readers will have had a similar patient.  At present, this is medically unexplained, but I suspect that I am missing something.

Addendum:  See comments of Dr. Howard Maibach.

Wednesday, June 06, 2018

DPP4-Inhibitor Drug-Induced Bullous Pemphigoid

The patient is a 68 yo woman with Type II diabetes and Stage IV renal failure who presented with a four month history of intense generalized pruritus.  Her PCP had treated her for scabies without effect.  Her medications  include Lantus, lisinopril, atorvastin risperadone  and Januvia (sitaglipin).  The Januvia was the  most recent new medication and it was started a month or two before she started to itch.

O/E:  there was a wide-spread dermatitis on torso and extremities.  No frank bullae but there was a suggestion of vesicles.  No burrows were seen.  Vesicles were noted on the palms and soles.

An eliptical biopsy was done as well as a 3 mm punch biopsy from perilesional skin for DIF.

Histopath showed: Eosinophilic spongiosis and spongiotic vesiculation.
DIF was positive for IgG in a linear pattern at the DEJ
Histological Photographs courtesy of Dr. Jag Bhawan, Boston University SkinPath Laboratory



Diagnosis:  Bullous Pemphigoid secondary to DPP-4 inhibitor.  

Discussion: DPP-4 inhibitors  are a class of drugs that are used for Type 2 diabetes.  There have been eight references to BP as a cutaneous drug effect DPP-4 inhibitors in PubMed (the first was in 2016).  It is clear that we will be seeing more of these patients what with the wide-spread usage of DPP-4 inhibitors.

Acknowledgement:  Special thanks to Rick Sontheimer, M.D. who alerted me to this phenomenon and Dr. Jag Bhawan for the pathology interpretation and the beautiful photomicrographs.


References:
 1. Bullous Pemphigoid Associated with the Dipeptidyl Peptidase-4 Inhibitor Sitagliptin in a Patient with Liver Cirrhosis Complicated with Rapidly Progressive Hepatocellular Carcinoma. Harada M et. al.  Intern Med. 2017 Sep 15;56(18):2471-2474  Free Ful Text

2. Dipeptidyl peptidase IV inhibitors, a risk factor for bullous pemphigoid: Retrospective multicenter case-control study from France and Switzerland.  Benzaquen M, det. Al.. J Am Acad Dermatol. 2018 Jun;78(6):1090-1096
CONCLUSIONS: DPP4is, especially vildagliptin, are associated with an increased risk for development of BP. Their use needs to be carefully evaluated, particularly in high-risk patients, such as males and those age 80 years or older.  Full Abstract.

3. Vildagliptin significantly increases the risk of bullous pemphigoid: A Finnish nationwide registry study. O. Varpuluoma et. al. J. Invest Dermatol:
Volume 138, Issue 5, Supplement, Page S46, 2018.  Full Abstract (Supplied by Rick Sontheimer) 


Tuesday, June 05, 2018

The Cobblestone Nevus: A Thing of Beauty

The patient is a 30 year-old woman in the 14th week of her first pregnancy.  Her obstetrician asked her to see a dermatologist regarding a congenital nevus.  She has noticed only slight change in the past decade.

O/E: There is a 3.0 cm bosselated nevus over the right costal margin.  Some of the facets vary in color.

Clinical + Dermatoscopic Images:

Diagnosis: Congenital Nevus with a bosselated (pebbly, cobblestone) appearance.  Does anyone have a comment?  Presented for comments and to share the beautiful dermatoscopic appearance.  Am I missing something?

Plan:  I will reevaluate after she delivers.

Thursday, May 17, 2018

Painful Finger Tips Secondary to Opdivo

The patient is a 65-year-old woman with metastatic lung cancer who presents for evaluation of painful fingertips.  She has also had marked axillary pruritus which she handles with Lotrisone spray. 

Her medications include metformin, two types of insulin, metoprolol, Lipitor, Lexapro, and irbesartan.  Recently, she has been started on Opdivo (nivolumab) every two weeks.  Within a few months of starting Opdivo, she developed intense axillary pruritus, and a month or so later, intensively painful fissuring of the fingertips.  Each treatment of Opdivo re-exacerbates the fingertip problem, which is unbearable. Her cancer has gone into remission and she feels well otherwise.  She especially enjoys her grandkids.

Her oncologist put her on prednisone 20 mg q.i.d. for the fingertip eczema.  Because of the patient's diabetes, her fasting blood sugars while on prednisone have gone up over 500 mg%. 

EXAMINATION:  The examination shows erosions on a few of the fingertips without erythema.  Her feet and axillae are normal.  

Clinical Photos:


We did a literature search and there are a few early reports of dermatitis with Opdivo but they do noot not affect the hands.  It may be too early to say; but given the literature on painful hands and feet related to cancer therapies this is probably a significant reaction.  

PLAN:  I would try to keep this simple and avoid the prednisone.  Start with wet soak for 20 minutes in warm water, followed by clobetasol ointment, followed by gloves (a modified Soak and Smear protocol).  We will see how she does with this.  I have the patient's permission to post her case on this site where we may get ideas and/or be of help to others with a similar problem.   There is a reference to preventing Hand Foot reactions with chemotherapy.  It seems that Celebrex (celecoxib) may be the best option. Note: Celecoxib has been reported to be associated with an increased incidence of heart attack and stroke.  It's use should not be cavalier.

References:.

  1. Survey of cutaneous adverse reactions to targeted cancer therapies: value of dermatological advice. Damiani G OncoSkin working group. G Ital Dermatol Venereol. 2018 May 11. Abstract: From June to October 2012, 25 patients with cutaneous adverse reactions linked to targeted therapies were included in the study. The main prescribed drugs were cetuximab (52%) and erlotinib (20%) and the most common reactions were folliculitis/pustules (40%) and rash/erythema (40%). Hand-foot reaction syndrome was present in 8% of patients. A total of 30% of patients treated for a cutaneous reaction underwent a consultation by a dermatologist. In these patients the rate of oncologic therapy continuation without regimen modifications was higher (100%), while it was progressively lower in patients treated by oncologists (71%) or without any specific treatment (60%). Adverse reaction should be recognized by both dermatologists and oncologists and a multidisciplinary approach is mandatory.


2. Prevention strategies for chemotherapy-induced hand-foot syndrome: a systematic review and meta-analysis of prospective randomised trials.
Macedo LT, et.al. Support Care Cancer. 2014 Jun;22(6):1585-93.
Abstract
Amongst 295 studies identified, only ten met the inclusion criteria. Celecoxib prevented both moderate to severe (odds ratio [OR] 0.39, 95 % confidence interval [CI] 0.20-0.73, P=0.003) and all-grade HFS (OR 0.47, 95 % CI 0.29-0.78, P=0.003), whereas pyridoxine and topical urea/lactic acid formulations failed to prove efficacy. There were no proven benefits in mild HFS. The use of topical antiperspirant has not been shown to improve results, according to a single trial.




Friday, May 11, 2018

Generalized Pustular Eruption in a 27 yo woman


The patient is a 27 yo woman with a 2 week history of an evolving, wide-spread eruption.  The initial lesions were on the popliteal fossae.  These were described as erythematous areas studded with pustules.  Over a week or two these generalized.  She has a history of mild psoriasis (scalp and elbows) for over a decade.  About three months ago she was started on bupropion 75 mg a day for anxiety and restlessness.  This was increased to 150 mg per day ~ 2 week before the onset of the dermatitis.  The patient has moderate cognitive impairment and Type 2 diabetes.  Here other medications include thyroid supplementation, metformin and insulin.  In the days since her initial office visit, the eruption has become more extensive and is taking on an erythrodermic appearance.  She was admitted to hospital two days after her office appointment.               

O/E:  When seen on May 7, 2018, she had a widespread eruption on arms, legs and torso,  The lesions were large arcuate patches with pustules at the periphery.  She was experiencing considerable pain.

Clinical Images:


 Pathology: (courtesy of DR. Erin Tababa, Fellow in Dermatology, Boston University)  

The biopsy shows prominent, relatively large, subcorneal pustules that are filled with a dense exudate of acute inflammatory. The neutrophils extended into the underlying epidermis, which has evidence of mild spongiosis. The papillary dermis is slightly oedematous, and there is a moderately dense perivascular inflammatory infiltrate with a predominance of neutrophils, although no eosinophils are noted.


Lab:
WBC: 29,000
Differential:  Shift to left
Eosinophils: normal
G6PD Normal

Chemistries normal.

Wound Culture: Pending

Dx:  We are initially considering subcorneal pustulr dermatosis, but with more history, especially considering the recent prescription of bupropion a drug-induced annular pustular psoriasis evolving into pustular and exanthematous psoriasis seemed more accurate.  Histopathology supported that.  Similar reactions have been reported to bupropion.  Our patient had been on the bupropion for over a month when this began which is longer than the patients in the case report below.  Reference 2 is a similar patient with a long latent period between initiation of drug and development of GPP.

Follow-up in hospital.  The eruption continued to evolve. It became more exanthematous and desquamative. These pictures were sent us by her mother.


Plan: 
Cyclosporin 3 – 4 mg per kg per day in divided doses
Wet dressings followed by triamcinolone 0.1% ointment bid – tid
Adjunctive secukinumab has been reported to be effective.

Follow-up:
Patient is doing very well.  These photos were taken 5 days after starting cyclosporine 100 gm q.i.d.  Her dose was dropped to 100 mg t.i.d.  She also was treated with wet dressings and triamcinalone ointment 0.1% (although the hospital only gave her 15 mg tubes, so she could not cover most ot the lesions. 
References:
1. Generalized pustular and erythrodermic psoriasis associated with bupropion treatment. Cox NH, Gordon PM, Dodd H. Br J Dermatol. 2002 Jun;146(6):1061-3.
Abstract: Severe drug eruptions may cause diagnostic and therapeutic difficulty when they mimic or provoke endogenous patterns of dermatosis. We report three patients with known psoriasis in whom use of bupropion (Zyban), prescribed to assist with cessation of smoking, led to severe pustular or erythrodermic exacerbation of psoriasis within 3-5 weeks. All patients were systemically unwell and required hospitalization to control the disease flare.

2. A diagnostic challenge: acute generalized exanthematous
pustulosis or pustular psoriasis due to terbinafine
L. Duckworth et.al. Clin Exp Dermatol. 2012 Jan;37(1):24-7
Abstract:  A 72-year-old man developed a generalized erythematous pustular eruption 11 weeks after commencing terbinafine. Clinically and histologically, the appearance was that of acute generalized exanthematous pustulosis (AGEP), and the disease was managed with topical preparations. Initial improvement was marred by relapse of acute pustulosis, now more in keeping with terbinafine-induced pustular psoriasis (PP),which was successfully treated with acitretin. This case highlights the difficulty of differentiating between AGEP and PP.

3. Acute generalized exanthematous pustulosis mimicking toxic epidermal necrolysis in patients with psoriasis: a coincidence?
Worsnop F, et. a. Clin Exp Dermatol. 2015 Aug;40(6):688-9

.


Tuesday, May 08, 2018

An Infant Girl from Rwanda with a Congenital Dermatosis

presented by Caitlun Stiglmeier, M.D.

I was hoping to have some input on this case.The patient is an 11 month old female infant born via spontaneous vaginal delivery, to a G1P1 mom (young, but not sure of mothers age). No reported maternal history of perinatal infections. TORCH screening performed on the child during this admission is negative. The child has bilateral retinal detachment, but her hearing is retained. She has gross developmental delay, and the rash you see in the photos started after birth on the R forearm, and has migrated since to all areas of the body, including the chest, back, yo the upper and lower extremities, and scalp. Her mother states the rash begins as blisters (second photo), which then open (no drainage noted per mom) and leave areas of hypopigmentation (third photo) and finally hyper-pigmentation (first photo).

The child does not seem bothered by this rash, it is not pruritic, and she does not have any fevers. Despite negative TORCH screening, I'm still thinking along the lines of a congenital-type of disease. Your thoughts would be greatly appreciated!


Dr. Yoon Cohen wrote:
The clinical description and the images seems to go with incontinentia pigmenti in this her 11 month infant girl.
For management, the skin changes of IP usually do not requires any specific treatment other than wound care for blisters to prevent secondary skin infection. The baseline eye exam and close follow up by an ophthalmologist will be important, especially with her retinal detachment history. Neurological evaluation should be done for potential seizures, encephalopathy and ischemic stroke. Dental evaluation is recommended after teeth erupt for pegged or missing teeth. Other ectodermal abnormalities can be seen as alopecia and nail dystrophy.  In countries with more resources, this infant would benefit from being seen at a center where the pediatric dermatologists have more experienc with genodermatoses.

References:
1. I.P. NORD site.
2. I.P. GARG (NIH Genetic and Rare Diseases information site)
3. I.P. World Community  (some parts in French)

Friday, April 27, 2018

FADES

The patient is a 17 yo boy with a 10 year history of asymptomatic hyperkeratosis of both elbows.  His knees are normal.  He has no personal history of atopy and both he and his mother deny that he rubs or scratches the area.  He's embarrassed by this and avoids sports as a result.  He is moderately obese and does not exercise or do sports.

O/E:  Symmetrical hyperkeratosis of both elbows.  Knees perfectly normal as is the remainder of his cutaneous exam.  He is moderately overweight.

Clinical Images:

Diagnosis: Asymptomatic Frictional Hyperkeratosis of the Elbows

Comment:  I suppose we all see this entity on frictional sites, but rarely name it.  The patient denies rubbing or scratching the area but was observed to lean on his elbows.  A similar problem has been reported (see reference  1. below).  I recommended starting with 5% Keralyt gel twice daily and may add tretinoic acid.  I propose that this type of hyperkeratosis can follow pressure on bony prominences, such as is seen on the foreheads of devout Muslims who pray 5 times a day (2).  Prayer marks in Muslims appear to be more common in diabetics (3).  The patient described in this VGRD  post could well have the metabolic syndrome.  I wonder if this might be important. I'd appreciate your  thoughts. 

Reference:

1. Frictional asymptomatic darkening of the extensor surfaces.
Krishnamurthy S, Sigdel S, Brodell RT. Cutis. 2005 Jun;75(6):349-55.
Abstract: Frictional asymptomatic darkening of the extensor surfaces (FADES), also known as hyperkeratosis of the elbows and knees, is commonly seen by dermatologists but has never been well characterized. Patients present with uniform, asymptomatic, brown darkening over the extensor surfaces of the elbows and knees with minimal scaling. Both frictional stress and family history may play a role in the pathogenesis of this condition. The results of cutaneous biopsy specimens typically reveal hyperkeratosis, acanthosis, and mild papillomatosis with minimal inflammation. Keratolytic agents such as lactic acid and urea cream along with avoiding frictional stress can be effective in the management of this condition. We describe a series of cases of FADES and its etiology and management options.
Comment in: Frictional asymptomatic darkening of the extensor surfaces. [Cutis. 2007]

2. Prayer marks. Abanmi AA et. al. Int J Dermatol. 2002 Jul;41(7):411-4.
Prayer marks (PMs) are asymptomatic, chronic skin changes that consist mainly of thickening, lichenification, and hyperpigmentation, and develop over a long period of time as a consequence of repeated, extended pressure on bony prominences during prayer. PubMed.


3. Prayer Marks in Immigrants from Bangladesh with Diabetes Who Live in Greece.  Papadakis G, et. al. J Immigr Minor Health. 2016 Feb;18(1):274-6. PubMed.