Thursday, December 19, 2019

ABOUT VGRD

Founded in 2000, Virtual Grand Rounds in Dermatology (VGRD) is a gathering place for dermatologists the world over to meet with one another and share interesting and/or challenging patients. In addition, we welcome all other health care practitioners with an interest in cutaneous disorders.  One may want to ask a question about diagnosis or therapy, present an interesting clinical photo or post a photomicrograph. We are a group of clinical and academic dermatologists who believe that web-based teledermatology can be both personally and professionally enriching.

Digital photography makes it possible to post clinical and microscopic images with ease. There are a dizzying number of cameras to choose from. The site creators will help you with advice here if you want.  In the past few years, smart phones have improved to the point where their images are more than acceptable.

Even if one lives in a city with a major medical center it is often difficult to get one's patients to Grand Rounds. And if one does, the turnout and discussion may be disappointing. VGRD is always available. You can post a message at 6:00 p.m. in Boston, Henry Foong may see it at 6:00 a.m. in Ipoh, Malaysia as he sits down at his home computer. Often, you will have received a few suggestions or comments when you log on the next morning.

VGRD has been a virtual consultative and collegial community for over 15 years. John Halle, the 16th Century English physician/poet, penned these perceptive words about the consultation in a long forgotten tract:

    When thou arte callde at anye time,
    A patient to see:
    And dost perceave the cure to grate,
    And ponderous for thee:

    See that thou laye disdeyne aside,
    And pryde of thyne own skyll:
    And think no shame counsell to take,
    But rather wyth good wyll.

    Get one or two of experte men,
    To helpe thee in that neede;
    To make them partakers wyth thee
    In that work to procede....

Halle's words guide us as we gather 500 years later in a consultative community the likes of which he probably could not have fathomed. So, let us "laye disdeyne aside,/ And pryde of [our] own skyll:/ And think no shame counsell to take,/ But rather wyth good wyll" join us in this global community of peers to help our patients and educate each other and ourselves.

Monday, December 10, 2018

17 Year-old Girl with Unexplained Bruising

The patient is a 17 year-old girl whose first episode of bruising occurred after trauma sustained while playing soccer in March of 2018.  The middle child of three, she comes from a stable family.  She is an active varsity athlete.

For purposes of VGRD, the present illness consists of episodes of unexplained ecchymoses occurring every few months since March 19th, 2018.  This initial ecchymosis was tender and persisted for a month.  She has had three more episodes of bruising since then, not all related to antecedent trauma.

The patient has had poorly explained pelvic pain for two years, menometrothagia, and significant urinary retention necessitating self-catherization.  Extensive pediatric urologic evaluation at a major medical center found only a “lazy bladder.”

Other constitutional symptoms include nausea, vomiting and weight loss.  Over the past two years she has consulted multiple primary pediatricians, a pediatric endocrinologist, a neurosurgeon, a pediatric nephrologist, two pediatric urologists, a pediatric gastroenterologist, a pediatric gynecologist, a neurologist, and a pediatric hematologist. 

Thorough hematologic/coagulation workup was normal except for a minor platelet defect on electron microscopy that was felt insufficient to be causing the ecchymoses.

Two weeks ago she had another spontaneous episode of ecchymoses on her abdomen and neck, that are illustrated in photos.  Although her past ecchymoses have been tender, this most recent extensive bruise on the neck was very painful, and exquisitely tender to light touch.  Over the past two weeks these are slowly resolving.

On questioning both patient and her parents deny any adverse childhood experiences and nothing suggests a factitial etiology.

Clinical Photos:


Lab: Extensive laboratory studies have been normal.
An intradermal autoerythrocyte sensitization test has not been done yet.  Among the many studies done, an MRI showed a small pituitary microadenoma that was considered to be an incidentaloma.

Diagnosis:  The history and clinical appearance suggests Gardner Diamond Syndrome (Autoerythrocyte Sensitization Syndrome).

Questions:  GDS is a controversial diagnosis. 
1. What are your thoughts regarding this entity, especially in reference to this young woman?  She will see a pediatric rheumatologist and a pediatric  dermatologist and a pediatrician with a special interest in adolescent medicine. 
2. How can you tie together her disparate pelvic and urologic symptoms, as well as her unexplained nausea and vomiting with her bruising?

One can imagine how unsettling and scary the past two years have been for this young person and her family.  Your thoughts and suggestions will be appreciated.

Tuesday, November 27, 2018

Primary Biliary Cholangitis and Pruritus


Primary Biliary Cholangitis and Pruritis

This 57 yo woman was first seen in February 2018 for generalized pruritus.  She suffered with insomnia secondary to the symptoms. Her long-term medications include lithium, Effexor (venlafaxine) and Abilify (aripiprazole).   Because of abnormal LFTs she was referred to a gastroenterologist.  A liver biopsy “was suspicious for primary biliary cholangitis vs. primary sclerosing cholangitis.”  She was started on ursadiol 500 mg b.i.d. and cholestyramine 4 gm b.i.d. 

O/E: This woman appeared tired, depressed and older than stated age.  There were excoriated papules on her arms and legs and accessible areas of torso.

Clinical Photo:


Lab: Because of initial symptoms, CBC and Chem profile were ordered in February 2018
CBC normal
Initial Chen Profile: Alk phos 718 IU   (nl 18 – 218)
                                 SGOT 309            (nl 15 – 37)
                                 SGPT 446            (nl 15 – 56)
Repeat 10.18)          AlK Phos 319
                                SGOT  40
                                SGPT  57
                                Bilirubin has always been wnl.

Pathology: Biopsy of a papules on her right leg showed irregular epidermal hyperplasia, markedly dilated and disrupted follicles containing necrotic debris and a few PAS + spores. Gram stain negative.  DX: Perifollicular fibrosis suggestive of perforating folliculitis.

Comment:  In spite of normalization of LFTs on ursadiol and cholestyramine her pruritus did not improve.  The etiology of pruritus in PBC is unclear.  She has been started on Rifampin 150 mg b.i.d. and Narrow Band UVB.  I assume her skin lesions are secondary to PBC pruritus and excoriations. Your comments will be helpful.

Reference:
Excellent reference suggested by Dr. Richard Sontheimer:

1. A systematic approach to the management of cholestatic pruritus in primary biliary cirrhosis.  Hegade VS, wt. al.  Frontline Gastroenterol. 2016 Jul;7(3):158-166.   Free Full Text

Dr. Amanda Oakley suggested the leg lesions look like porokeratosis.  The association has been reported once in the literature.


2.Porokeratosis in primary biliary cirrhosis during plasmapheresis.  Venencie PY, Verola O, Puissant A. J Am Acad Dermatol. 1986 Oct;15(4 Pt 1):709-10.  Free Full Text.






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?