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.

Subtle Facial Lesion in a Four Year-old Boy

The patient is a four year-old boy who was referred for evaluation of a slightly rough patch on his right cheek that has been present for over a year.  He is otherwise well.

O/E:  There is a subtle 1.5 cm in diameter erythematous patch on his right cheek.  Dermatoscopy revealed a group of sharply demarcated plugs that appear to be comedones.  Clinically, this was not as evident.

Clinical and Dermatological Images:

 Diagnosis:  Small subtle nevus comedonicus.

Discussion:  I feel this is probably a nevus comedonicus.  I’ve only seen a few of these and all were obvious: not so with this case.  Not much is known about the evolution of these lesions.  Topical retinoids are of some value; but since this doesn’t bother the patient I am reluctant to have his mother rub a topical agent on the area for two to three months.  Does anyone feel that tazarotene is preferable to tretinoin in similar cases?  Comedone extraction would be easy, but can be traumatizing in a young child.  Once I hear other ideas I will discuss options with his parents.

References:

1. Dermoscopy on nevus comedonicus: a case report and review of the literature.  Kamińska-Winciorek G1, Spiewak R.

Postepy Dermatol Alergol. 2013 Aug;30(4):252-4.  Free Full Text.

2. Nevus comedonicus: an updated review. Tchernev G, et. al. Dermatol Ther (Heidelb). 2013 May 25;3(1):33-40. Full Free Text.

Erysipelas in a 69 yo Woman

presented by Henry Foong
Ipoh, Malaysia

A 69-yr-old woman was seen with a 2-day history of rapidly enlarged swelling on the right cheek.  Initially she felt some discomfort on the right ear and then involved the right cheek.  She had no fever.

There was no history of trauma.  She had a history of hypertension. 

Examination showed unilateral swelling on the right cheek with increased temperature, tenderness and redness extending from right forehead to the right lower jaw.  It appeared oedematous, with swelling of the ipsilateral upper eyelid. It had a well defined raised border. The rash did not cross the bridge of the nose to the opposite cheek. No blisters were seen. Her regional nodes were not enlarged.

Clinically she has erysipelas.

TWBC 12,900 (N84%)

blood sugar  8.5 mol/l

BU and serum electrolytes normal

LFT normal

Erysipelas affects the superficial layer of the skin while cellulitis affects the deeper part of the dermis. There were no clusters of vesicles or erosions to suggest herpes zoster. She was not keen to be admitted and preferred oral medication as an outpatient  She was treated with oral cefuroxime 500mg bd for 10 days with wet compress dressing on the affected face. The erysipelas cleared with the treatment.

Pemphigus Vulgaris in a 43 yo Woman

The patient is a 43 yo Hispanic female. with several months of bleeding gums, sloughing tissue, and generalized oral pain.   She is otherwise healthy and takes no meds or supplements.  She has no skin or vaginal lesions.

Lab: Normal CBC and metabolic panel.

Her oral surgeon did a biopsy biopsy of normal mucosa adjacent to sloughing attached gingiva on anterior mandible and sent this for direct immunoflouresence (DIF).

Clinical Image:

Path:

H&E: Acantholysis

DIF:  Intercellular deposition of C3 and IgG.

Diagnosis: Pemphigus vulgaris

Questions:
This patient does not live close to an academic center and travel there would be difficult.
Does it make sense to initiate therapy with rituximab (if her insurance covers that) + prednisone. or should therapy be initiated with prednisone and conventional  adjunctive added later if nexessary?


References:

1. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial.

Joly P. et. al.   Lancet. 2017 May 20;389(10083):2031-2040.

This is a paper by the French study group on autoimmune bullous skin diseases.

CONCLUSIONS: 32.5% of our patients with moderate to severe pemphigus vulgaris failed prednisone and traditional CAT treatment and required rituximab therapy. Rituximab reduced the monthly prednisone intake in these patients by 73%. This suggests that a subset of patients with moderate to severe pemphigus may benefit from early institution of rituximab therapy. Rituximab significantly reduces the monthly prednisone requirement among CAT-resistant pemphigus vulgaris patients to levels on par with CAT-responsive patients.

2. Comparison of rituximab and conventional adjuvant therapy for pemphigus vulgaris: A retrospective analysis.

Agarwal A, Hall RP 3rd, Bañez LL, Cardones AR.

PLoS One. 2018 Sep 25;13(9):e0198074.  Free PMC Article

CONCLUSIONS: 32.5% of our patients with moderate to severe pemphigus vulgaris failed prednisone and traditional CAT treatment and required rituximab therapy. Rituximab reduced the monthly prednisone intake in these patients by 73%. This suggests that a subset of patients with moderate to severe pemphigus may benefit from early institution of rituximab therapy. Rituximab significantly reduces the monthly prednisone requirement among CAT-resistant pemphigus vulgaris patients to levels on par with CAT-responsive patients.

3. 

JAMA Dermatol. 2015 Aug;151(8):878-82.

Intralesional Rituximab in the Treatment of Refractory Oral Pemphigus Vulgaris.

Vinay K, Kanwar AJ, Mittal A, Dogra S, Minz RW, Hashimoto T.

JAMA Dermatol. 2015 Aug;151(8):878-82.

Abstract

IMPORTANCE:

Oral lesions of pemphigus vulgaris are usually recalcitrant and respond slowly to treatments. Corticosteroid injection is considered to be the most effective local treatment in oral pemphigus vulgaris. However, intralesional corticosteroids are not effective in all remnant lesions. In 3 such patients with pemphigus vulgaris, we evaluated the utility of 2 injections (on days 1 and 15) of intralesional rituximab, 5 mg/cm², in terms of accelerated healing, limitation of the use of systemic immunosuppressants, and reduction of their adverse effects.

OBSERVATIONS:

Three patients (1 man and 2 women) received 2 doses of intralesional rituximab in March and April 2013. All 3 patients responded to the treatment. In patients 1 and 2, the objective severity score was reduced to 0 at the final visit from a baseline score of 4 and 5, respectively (range, 0-11). The subject severity score in these patients was reduced to 1.0 and 0 from a baseline score of 22.0 and 22.5, respectively. After clinical remission was achieved, patient 3 developed a relapse of mucosal lesions. At the final visit, all of the patients were satisfied with the treatment, with a mean satisfaction score of 8 (maximum score, 10). We found a marked decline in the CD19 cell count from a pretreatment mean count of 287 cells/µL to 6 cells/µL on day 15 after a single intralesional rituximab injection. Adverse events were limited to local pain in 1 patient.

CONCLUSIONS AND RELEVANCE:

Intralesional rituximab administration lacks the adverse effects of intravenous administration. This method reduces the amount of drug administered and therefore is less expensive. Encouraging results from our study should prompt further evaluation of this novel route of rituximab administration in patients with refractory oral pemphigus vulgaris.

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.

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.

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.