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

.

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)

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.