Monday, October 16, 2017

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.

Thursday, September 28, 2017

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!






Wednesday, September 20, 2017

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

Diagnosis:  Working Dx:  Tinea amiantacea

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.  The other question I have is whether a scalp biopsy may be helpful. 


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.

Monday, September 18, 2017

Cheilitis Query


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?

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.

Tuesday, September 05, 2017

Man from India with Wide-Spread Vesicular Eruption


Presented by Dr. Bassem Ghali
Jagadguru Sri Shivarathreeswara University
Mysuru, India


The patient is a 60 yo man with a  pmhx of COPD who noted a recent eruption of vesicles on trunk, as well as his forehead and scalp. No fever. No other pertinent history. No itch. No pain. No new meds.
It started as blisters on the trunk, slightly itchy but no other symptoms, and not painful. The lesions opened up with clear fluid being expressed and leaving shallow ulcers. They have started become generalized with new lesions on the scalp and genitalia.

O/E:  Lesions appear like small bullae/vesicles on chest and abdomen, with clear fluid. In the center is what appears to be a black point, probably the hair follicle. There is no erythema on or around these lesions. 

Clinical Photo:

 Diagnosis:  What are your thoughts?

Friday, September 01, 2017

Hailey-Hailey Disease

The patient is a 38 year-old cook with a 14 year history of a pruritic, occasionally painful rash on his torso.  He used triamcinalone cream in the past without relief.  No family history aof a similar process.

O/E.:  There are plaques in axillary folds, on the chest, back, and popliteal fossae.Some advancing borders appear vesicular on close observation.  There is mild post-inflammatory hyperpigmentation in healed sites.

Clinical Photos from L. Axilla:
Laboratory:  An incisional biopsy was taken from the crusted/vesicular edge.

Tentative Diagnosis:  Benign Familial Pemphigus (Hailye-sHaily)

Thursday, August 03, 2017

Labial Melanocytic Macule


The patient is a 62 yo woman who presents with an 18 month history of a pigmented macule on the lower lip.  She has noted no change over time.  No history of skin cancer.

O/E:  There is a 6 mm in diameter dark tan macule on the lower lip.  Dermatocopic exam shows pigmented dots.

Photos:
Diagnosis:  This appears to be a Labial Melanocytic Macule.

Discussion:  There are only very few references that describe the dermatoscopic appearance of these lesions.  Those I found were solitary case reports.  The brown dots may indicate the lesion is in the active growth phase.  I offered to biopsy the lesion, but  told the patient it was likely a LMM and is shepreferred, we could follow her up in 3 months.