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.

Demodeciasis: One off

The patient is an 86 yo man was tarted on imbruvica for a lymphoma in August of 2017.  Around a month later he developed a facial eruption that had the appearance of rosacea.  As it was mild, it was not treated.  The eruption has worsened over the past few months.

O/E  There are erythematous papules and pustule on the right malar eminence and erythema and mild swelling of the nose.  The left malar eminence and the remainder of the  head and neck are normal.

Clinical Photos:

Lab:  A scraping from two papules revealed numerous (apparently happy) demodex mites.

Diagnosis:  Demodeciasis, most likely as a side-effect of imbruvica.  This has not been reported in the literature at present, but I suspect it will be soon.

Treatment was initiated with Sklice (topical ivermectin).  If this is not effective, he will be offered oral ivermection.  The latter may have been a better strategy.

Follow-up 10 days after starting ivermectin Solution (Sklice);  ~ 50% better.

 

Reference:

1. Parmar S, Patel K, Pinilla-Ibarz J.

Ibrutinib (imbruvica): a novel targeted therapy for chronic lymphocytic leukemia. P T. 2014 Jul;39(7):483-519.  Free Full Text.

2. Patrizi A1, Bianchi F, Neri I.  Rosaceiform eruption induced by erlotinib. Dermatol Ther. 2008 Oct. Suppl 2:S43-5.

Abstract:

Adverse events with anti-epidermal growth factor receptor therapy mainly involve the skin. The most common cutaneous adverse event is an acneiform eruption, which occurs in more than 50% of cases. The aim of this paper is to report the case of rosaceiwform eruption induced by erlotinib in an 81-year-old-man and to discuss the pathogeneic role of Demodex folliculorum mites, found in the present patient, using skin scraping.

Granulomas in 62 year-old fisherman

Presented by Dr. Henry Foong
Ipoh, Malaysia

A 62 yr old man presented with 4 weeks history of pruritic papular lesions on the thighs bilaterally which then spread to the legs. The pruritus was intermittent and he felt feverish at times. No history of trauma. He is a retired fisherman and lives  in Teluk Intan about 60 km south of Ipoh, Malaysia.

O/E: showed multiple indurated erythematous papules, non-tender distributed symmetrically over the inner thighs and legs. Superficial erosions and ulcerations were noted on the affected areas. There was no crepitus.

The referring physician suspected elephantiasis. The patient is on oral antibiotics ( metronidazole and unasyn) and albendazole.  At one time he was on DEC (diethylcarbamazine)  but it was withheld because of side effects.

Lab: Blood counts showed normal TWBC with 7% eosinophils.  The physician did a colonoscopy last month and biopsy suggested underlying parasitic colon infection.

Culture of the skin lesions - Clostridium perfrigens.

A skin biopsy was performed on the skin papules on the thigh.

Pathology: Skin biopsy showed epidermis with marked spongiosis. the dermis show cluster and scattered granulomas with abundant surrounding neutrophils and plasma cells.  There is a huge collection neutrophils with necrotic material. There are multinucleate gaints granulomas. The inflammatory cells involved the subcutaneous fat.

Clinical and Pathological Images:

Impression:  Sporotrichosis?

Questions: What are your thoughts? What further studies would you consider.

Dupilumab Conjunctivitis

The patient is a 55 year-old woman who has been on dupilumab (Dupixent) for two months.  She had life-ling severe atopic dermatitis and states that her skin has never been this clear in her memory.  She has, however, developed a conjunctivitis of her lid and bulbar conjunctivae.  She is so happy that her skin is clear that she is not complaining, and her eyes are not uncomfortable.

There are no references to conjunctivitis on dupilumab, but the package insert indicates that 10% of patients have experienced this.  If you have any recommendations, I would appreciate hearing them.

Photos presented with patient permission

5/2/18.  Two months after starting Protopic ointment 0.03%, there has been no improvement of her conjunctivitis.  We have, therefore, prescribed fluoromethanlone 0.1% ophthalmic solution b.i.d.   The patient says that having the cojunctivitis is acceptable as long as her skin is doing well for the first time in > 50 years.  Photos taken before starting fluoromethalone ophthalmic.

Reference:

Conjunctivitis occurring in atopic dermatitis patients treated with dupilumab-clinical characteristics and treatment.

Wollenberg A J Allergy Clin Immunol Pract. 2018 Feb 9. pii: S2213-2198(18)30089-8.  Free Full Text.

Here, we report our experience with this clinically relevant complication of dupilumab treated AD seen in 25% and 50% of patients from our two centers from April 2016 to February 2017, and give treatment recommendations based on our personal experience with 13 moderate-to-severe dupilumab-treated AD patients developing conjunctivitis as adverse event. This conjunctivitis is reported in temporal association with dupilumab treatment, but a causal relation is not established.

However, in our limited experience, antihistamine eye drops and artificial tears did not confer any alleviation in this type of conjunctivitis. In 11 patients, dupilumab-related conjunctivitis was treated with topical tacrolimus or steroids, leading to clinically significant improvement or full recovery in all 11 patients treated.

Two treatment options were particularly effective.

In 5 patients conjunctivitis was treated with fluorometholone 0.1% eye drops, leading to significant improvement.

In four patients, conjunctivitis was treated with tacrolimus 0.03% eye ointment. Signs and clinical symptoms improved significantly in all patients, and in 2 patients, full recovery of conjunctivitis was achieved.

Mal Perforans Ulcer

The patient 74 year old divorcee who lives alone.  He is an insulin-dependent diabetic with peripheral neuropathy.  He has had a plantar ulcer for > 6 months that began after a callosity was pared down by a podiatrist.  He has been seen at a wound care clinic for six months where dressings are done.  He is afraid he may lose his foot.

O/E:  On the plantar aspect of the left foot, he has a clean. painless ulcer measuring about 1.4 cm in diameter.  There was a thick callosity the ulcer's periphery.  His pedal pulses are strong.


Clinical image:

Question:  How would you approach this ulcer?

6/15/2018
The patient had an orthopedic procedure two months ago.  This was intended to  redistribute some of  the pressure on the ulcerated area.  It was quite successful.  He has some mild foot edema presently, but the ulcer has healed completely.  His A1C is normalizing, too.

                         

Reference:

Lu SH, McLaren AM. Wound healing outcomes in a diabetic foot ulcer outpatient clinic at an acute care hospital: a retrospective study. J Wound Care. 2017 Oct 1;26(Sup10):S4-S11

Abstract

OBJECTIVE: Patients with diabetic foot ulcers (DFU) have an increased risk of lower extremity amputation. A retrospective chart review of patients with DFUs attending the Foot Treatment and Assessment chiropodist-led outpatient clinic at an inner-city academic hospital was conducted to determine wound healing outcomes and characteristics contributing to outcomes.

METHOD: We reviewed the complete clinical history of 279 patients with 332 DFUs spanning over a five-year period.

RESULTS: The mean age of patients was 61.5±12.5 years and most patients (83.5%) had one DFU. The majority of wounds (82.5%) were in the forefoot. Overall, 267/332 (80.5%) wounds healed. A greater proportion of wounds healed in the forefoot (82.5%) and midfoot (87.1%) than hindfoot (51.9%; p<0.001). Using a logistic regression model, palpable pedal pulse and use of a total contact cast were associated with better wound healing.

CONCLUSION: Our findings are the first to demonstrate the benefits of chiropodists leading an acute care outpatient clinic in the management of DFUs in Canada and delivers wound healing outcomes equivalent to or exceeding those previously published.

  

Nail Dystrophy in 59 yo Woman

The patient is a healthy librarian who noticed a reddish area under the left thumb nail ~ 3 months ago.  Shortly after that, the distal portion of the nail become yellowish.  She has not history of trauma and the area is not painful

O/E:  There is erythema noted medically in the nail bed and the nail plate is onycholytic.  No abnormality of the nail plate is noted other than the yellowish area and possibly a Beau's line.  The patient feels that the red area has migrated medially.

Clinical Image:

My thoughts:  I am concerned that there may be a tumor under the nail plate.  The fact that it is painless argues against glomus tumor.  I welcome suggestions.

Thank you,
DJE

Non-healing ulcer after surfing injury

Surfer's Sore

The patient is a 70 year-old surfer living in Hawaii.  Two months ago, he sustained a cut over his shin bone on a lava rock/sandstone shelf of a reef on Kauai.  It has not healed in spite of cleaning area daily with chlorhexidine scrub and applying Medihoney and triple anti bacteria ointment.  He says, "What is weird and kinda creepy is it feels like something is crawling around in there from time to time, especially while sleeping?"  The patient, a light-complected Caucasian, has a history of non-melanoma skin cancer.  He is scrupulous about sun-protection, but has spent more than a half a century with significant sun-exposure.

 

This is the sandstone slab on which the injury took place:

What are your thoughts?

References

1.  Sea Ulcers Andrew Nathanson, MD,  Surfing Medicine (Journal of Surfing Medical Association) Dec, 2014

Two year-old with enanthem and exanthem for two weeks

Presented by Will Shepard, M.D.

Gillette, WY

The patient is a two year-old girl with a two week history of oral and skin lesions.  She has been well and healthy otherwise and all of her milestones have been normal.  The present illness began with two ulcers on her tongue.   A few days later she started to develop skin lesions, first on the arms.  The new lesions start with erythematous macules and became crusted after 12 – 18 hrs.  She has continued to develop new lesions on the torso, face and extremities.  Throughout this period she has been healthy, no fevers, appetite normal and in no discomfort.

O/E:  The tongue lesions have disappeared.  The skin lesions are few in number and measure 0.5 to 1 cm in diameter.  They are scaly annular macules on an erythematous base.

Clinical Images:

Rough area from Bandaid

New Lesion present since patient seen yesterday:

Impression:  The onset of an acute problem with first oral and then skin lesions in an otherwise healthy toddler suggests a viral process.

References:

1  [Paraviral exanthems]. [Article in German]

Fölster-Holst R, Zawar V, Chuh A. Hautarzt. 2017 Mar;68(3):211-216.

Abstract: Paraviral exanthems are distinct skin diseases due to infections with different viruses. Although no virus has been identified so far in some exanthems, the main age of manifestation, the clinical course of the exanthem, and the extracutaneous symptoms are suggestive for a viral genesis. While many viral infections are a direct result of the infection, paraviral exanthems reflect the response of the immune system to the infectious pathogens. Viruses cannot be identified in the skin. Typical paraviral exanthems include Gianotti-Crosti syndrome, pityriasis rosea, pityriasis lichenoides, papular-purpuric gloves and sock syndrome, and asymmetrical periflexural exanthema. Unilateral mediothoracic exanthem, eruptive pseudoangiomatosis are rare and eruptive hypomelanosis has been described recently.

Atypical Pigmented lesion in an 81 yo Man

The patient is a light-complected Caucasian with Type I skin and a personal history of non-melanoma skin cancer.  His daughter has a history of melanoma.

He presented for a skin exam and was found to have an atypical pigmented lesion on his right shoulder.  He remembers that this lesion had been biopsied years ago at another facility and he was told it was fine.

O/E:  On the right shoulder there was a 1.2 mm in diameter irregularly pigmented macule with a play of color.  The dermatoscopic picture was worrisome and he was scheduled for an excisional biopsy.

Dermatoscopic image

Pathology:  The excisional biopsy showed a melanoma 0.3 mm thick, 0 mitoses per mm squared.  Free margins, but narrow.

(The old biopsy report from 2007 was reviewed.  This was a shave biopsy that showed a junctional nevus with mild to moderate atypia and margins were clear in the sections examined.)

Discussion:  The patient has a thin melanoma arising in the site of a previous biopsy.  This raises the question of whether shave biopsies of pigmented lesions are appropriate.  At any rate, a thin melanoma, 0.3 mm thick just requires a wide-local excisison with one cm margins.  Sentinel node biopsy is not indicated.  The patient will have regular skin exams from this point on.

Linear Scleroderma in a 40 year-old Woman

Presented by Hamish McDougall
Cape Breton, Nova Scotia

The patient is a 37 year old woman with a four year history of a slowly progressively asymptomatic area of induration on the posterior aspect of the left thigh (photo).  A biopsy showed thickened collagen bundles in the reticular dermis and a sparse superficial and deep lymphoplasmacytic infiltrate consistent with morphea.

Strangely, her father-in-law is seriously ill with systemic sclerosis.  He lives far from the patient and her husband.  At this time, we have discovered no common exposures the patient and her father-in-law have.  The patient lives in an endemic area for Lyme Disease and serological testing will be offered.

Questions:
Is there any value in obtaining serologies, other than Lyme studies, on this woman?
What treatment might be of value? 
Do you have alternate diagnoses?

References:
1. Localized Scleroderma Review Article (Like a chapter in a text book)

2. Morphea Sculpted in Silica: A Case Report of Limited Cutaneous Systemic Sclerosis in a Woman with Long-Time Exposure to Silica Dust.

Pedro Gomes J, Shoenfeld Y.  Free Full Text.

3.  "Borrelia-associated early-onset morphea": a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases.

Prinz JC, et. al. J Am Acad Dermatol. 2009 Feb;60(2):248-55. CONCLUSION:B burgdorferi infection may be relevant for the induction of a distinct autoimmune type of scleroderma; it may be called "Borrelia-associated early onset morphea" and is characterized by the combination of disease onset at younger age, infection with B burgdorferi, and evident autoimmune phenomena as reflected by high-titer antinuclear antibodies. As exemplified by the case reports, it may take a particularly severe course and require treatment of both infection and skin inflammation.

Metastatic melanoma in an elderly man

Presented by Henry Foong, M.D.
Ipoh, Malaysia

The patient is a 80-year-old man who presented with swelling of the left leg for 3 months. 

About 3 years ago he had a motor vehicle accident where he injured his left leg.  His attending doctor noticed a pigmented growth on the left foot associated with inguinal node swelling. A surgeon excised the pigmented growth on the left foot and removed some nodes from the left groin.  No histological reports were available at the moment.  The patient was well until recently when he noticed gradual swelling of the left leg with multiple pigmented nodules on the surface.  The leg was occasionally painful at night. He did not have any constitutional symptoms.  There was no family history of skin cancers. No significant other medical illness.

On examination his left leg was swollen and oedematous with many pigmented papules and nodules on the foot and lower 1/3 of the left leg.  A pigmented ulcerating tumour  5 x 5 cm was noted on the left foot which extended to the heel.  A firm matted lymph node swelling was noted on the left groin.  There was a surgical scar over the left groin.  No hepatosplenomegaly was present.

Biopsy of the pigmented papule on the foot was done and confirmed malignant melanoma.
Sheets and nests of malignant cells are seen invading the dermis. The tumour cells show marked pleomorphism, have increased nucleo-cytoplasmic ratio, vesicular nuclei with prominent nucleoli and eosinophilic cytoplasm. Many of the cells contain melanin pigment. Numerous mitotic figures are seen. The tumour is seen at the margins. Masson Fontana stain is focally positive.
Skin biopsy Report: Features are consistent with malignant melanoma.

His work up included an oncology referral. CT scan of abdomen and pelvis which showed pelvic and para-aortic  lymph 
node metastasis.  CXR normal.  TWBC was 16,400. BRAF gene mutation studies pending 

He was being treated for concomitant cellulitis with IV antibiotics.

Questions:  What are the treatment options (targeted therapy) for his metastatic melanoma? Would oral vemurafenib and Anti PD-1 antibodies e.g.  pembrolizumab be useful?  They are very expensive though for most patients in Malaysia.

It's almost certain this patient will probably opt for palliative treatment. What local treatment of the in-trasit metastasis would be useful for him?

Thank you for your thoughts!

17 year-old girl with 8 year history of scalp dermatitis

The patient is a 17 year-old girl with an 9 year history of thick scales on her scalp.  She has used multiple medications without relief.  The patient has been bullied at school where she has been called “lice girl.”  Socially, this has been traumatic.

O/E:  She is a well-developed and well-nourished 17 yo with thick chestnut colored hair or normal intelligence.  There are no areas of alopecia. Thick, silvery adherent scales are present on the occipital, parietal and temporal scalp.  When these are removed, hair roots come out, too.  The remainder of the cutaneous examination is normal.  No nail dystrophy.

Clinical Images (July 2017)

Lab: Fungal culture negative.  Bacterial culture 3+ Staph aureus.

Failed Treatments (per mother):

"Every single otc dandruff shampoo

Every prescription medicated dandruff shampoo

Scalpicin

Prescribed scalp drops with and without coal tar

Every Tea Tree product you can find otc

Hot oil treatments

P & S Oil

Nutrogena T-gel and T-sal

Olive oil"

Terbinafine 250 mg p.o. x 1 month
Keflex 500 mg b.i.d. x 2 weeks

Scalp Biopsy read by Lynne Goldberg (Boston University Skin Path): was felt to be most compatible with psoriasis.  Seborrhea was in the differential diagnosis but less likely.

Diagnosis:  Working Dx:  Tinea amiantacea secondary to psoriasis.

Discussion: This 17 yo girl has suffered with what appears to be tinea aminatacea for almost a decade.  It appears unlikely that this is psoriasis. Tinea capitis has been ruled out by culture.  Her bacterial culture showed 3+ S. aureus but I suspect this is a secondary invader as she did not improve with cewplanexin.  Since the fungal  culture was negative and these approaches were not helpful, I may recommend isotretinoin.  The use of this has been reported for T. aminatacea only and in a Korean case report.

Dr. Goldberg's rotocol for Scalp Psoriasis, Tinea amiantacea and Related disorders:

1. Wet hair at night

2. Apply Dermasmoothe scalp oil liberally to scalp. Leave on overnight

3. Sleep with this overnight in a shower cap (to protect pillow)

4. Shampoo in the morning with T-Sal or other dandruff shampoo

Do this nightly at first if possible, but after a week or so she will be better and will not need to do it every night.

References:

1. Abdel-Hamid I et al. Pityriasis amiantacea: a clinical and etiopathologic study of 85 patients. Int J Dermatol. 2003 Apr;42(4):260-4.

2. Kwon JI.  Isotretinoin for Tinea amiantacea (A Case Report). Korean J Dermatol 2012;50(11):1002-1005 (In Korean)

3.  Mannino G, McCaughey C, Vanness E. A case of pityriasis amiantacea with rapid response to treatment WMJ. 2014 Jun;113(3):119-20.  Full Free Text.

4. Scalp psoriasis: European consensus on grading and treatment algorithm.  Ortonne J. J Eur Acad Dermatol Venereol. 2009 Dec;23(12):1435-44.

Cheilitis Query

September 2017
The patient is a 70 yo Caucasian who has lived on Moorea, French Polynesia, for the past 50 years.  She contacted us recently about her painful lips because there is no dermatologist available to her at present.  Here is her anamnesis:

In early July when I went to Montreal, my lips started to bother me. I thought maybe it was 18 hours on a plane, or even maybe it was a sunburn from being in the pool with my grandchildren in sunny Vancouver a couple weeks before. It didn't subside and I bought several lip therapies - cocoa butter, Vaseline, Aquaphor. When I came home I used a mild steroid ointment for a couple weeks, but to no effect. I now carry Aquaphor with me all the time and apply it constantly. Chapstick with SPF (from a friend in the States) stings my lips, as does toothpaste. My lips are not chapped, as in flaky or peeling, but they feel and look burnt, even blistery sometimes, and they can feel severely tight, dry and very sore. Actually, my upper lip is not as involved as my lower lip, and the corners are not affected. 

Photo sent by patient to VGRD

Diagnosis: This appears to be actinic cheilitis or possibly allergic/irritant cheilitis.  Strangely,  the patient got more sun in Vancouver than she does in French Polynesia!

What are your thoughts?

Update (April 2018):

(from the patient) I want to share with you the results of a recent experiment I conducted unwittingly. Our daughter was visiting Tahiti for the last week, and we went to the beach several days in a row. Although I wore a hat and Vanicream lip sunscreen, I got too much sun and my lower lip has been on fire. I think actinic cheilitis was an early guess last year, and I have NO DOUBT that it was correct. I'm using Vaseline, of course, and the betamethasone dipropionate ointment after nothing else worked. I'm happy not to be puzzled, confused and freaked out this time around.  Note:  I think we may be dealing with a case of actinic prurigo of the lips (see references 3 and 4 below)

References:

1. Actinic cheilitis: a treatment review.

Shah AY, Doherty SD, Rosen T.

Abstract:  All other factors being equal, the presence of actinic cheilitis, a pre-invasive malignant lesion of the lips, doubles the risk of squamous cell carcinoma developing in this anatomic area. Various forms of local ablation, immunomodulation and surgical extirpation have been proposed as therapeutic interventions. This paper critically evaluates the available medical literature to highlight the evidence-based strength of each recommended therapy for actinic cheilitis. Vermilionectomy remains the gold standard for efficacy; trichloroacetic acid application is easy and convenient, but the least efficacious overall.

2. Contact allergy in cheilitis.

O'Gorman SM, Torgerson RR. Int J Dermatol. 2016 Jul;55(7):e386-91.

BACKGROUND: Recalcitrant non-actinic cheilitis may indicate contact allergy.

CONCLUSIONS: Contact allergy is an important consideration in recalcitrant cheilitis. Fragrances, antioxidants, and preservatives dominated the list of relevant allergens in our patients. Nickel and gold were among the top 10 allergens. Almost half (45%) of these patients had a final diagnosis of ACC. Patch testing beyond the oral complete series should be undertaken in any investigation of non-actinic cheilitis.

3. Actinic Prurigo Cheilitis: A Clinicopathologic Review of 75 Cases.

Plaza JA, et al. Am J Dermatopathol. 2016 Jun;38(6):418-22.

4. Actinic prurigo of the lip: Two case reports.

Miranda AM. World J Clin Cases. 2014 Aug 16;2(8):385-90. Free Full Text.