Dermatofibrosarcoma protuberans (DFSP)

What is dermatofibrosarcoma protuberans?

• Rare tumour 
• most likely to be of fibroblastic lineage

How does it present?

• it has smooth appearance resembling a keloidal scar
• its site on the trunk in young to middle-aged patients

What is the management

• excision with wide surgical margins 

Reference:

• Primary certificate of dermatology RACGP 

Benign lymphocytic infiltrates

What is benign lymphocytic infiltrates?

• The are benign infiltrates of lymphocytes occurring in the dermis
• They are categorised into B cell and T cell proliferations. 
• Lymphocytoma cutis is the name used for B cell infiltrates
• Jessner's lymphocytic infiltrate is an example of a T-cell pseudo lymphoma 

How does it present?

• Rare
• The presentation is often in the third or fourth decade of life, but may occur at any age 
• They present as smooth red apple,es, plaques or nodules, which may be multiple and coalesce
• The face is most commonly involved, including the ear lobes

What the skin biopsy shows?

• moderately dense infiltrate lymphocytes within the dermis 
• distinction from lymphoma is sometimes difficult

What is the management?

• typical treatment includes
• potent topical steroids 
• intralesional steroids 
• phototherapy 
• hydroxychloroquine 
• spontaneous resolution sometimes occur 
• recurrence is common 
• small number may progress to lymphoma 

Reference:

• Primary certificate of dermatology RACGP 

Cutaneous B-cell lymphoma (CBCL)

What is cutaneous B-cell lymphoma (CBCL)?

• It can be either primary disease of the skin or secondary from nodal (non-Hodgkin's) lymphoma
• It is rare and less common than mycosis fungicides
• Men are more commonly affected, most cases present in the sixth or seventh decade of life

How does it present?

• solitary or multiple nodules localised to one area of the body
• Usually pink or violaceous in colour, smooth, firm, non tender 
• favour neck and head region
• lymph nodes in other area may be involved 

What does it look like on skin biopsy?

• dense infiltrate of lymphocytes throughout the mid and lower dermis 
• B lymphocytes do not localise to the epidermis, hence, no scale

What is the treatment ? 

• Referral to a dermatologist or oncologist is recommended for staging and treatment 
• Treatment options 
• radiotherapy 
• surgery for localised lesion s
• chemotherapy 
• rituximab 

What is the prognosis?

• In general good prognosis > 90 % 5 year survival rate for the follicle centre and marginal zone b cell lymphoma 
• less so for the variant occurring on the legs 
• Secondary CBCL is associated with a poor prognosis 

Reference:

1. primary certificate of dermatology RACGP 

Actinic keratosis

Key points:

• estimated around 10% of them becomes Squamous cell carcinoma 
• thought to be an intraepidermal lesion 
• multiple treatment modalities, the most common one is cryotherapy
• biopsy lesion if it does not respond to treatment

What is actinic keratosis?

• They are keratotic lesions with malignant potential 
• They are considered intraepidermal, pre-cursor or early lesions of squamous cell carcinoma 
• lesions are most commonly found in the sun-exposed areas of elderly patients with fair skin types who have had significant sun exposure in their lifetime

What are the histological changes?

• epidermal cell dysplasia 
• dilated upper dermal blood vessels 
• degeneration of collagen and elastin in the dermis (solar elastosis)

Who's at risk?

• Celtic descents 
• Skin types 1 and 2 
• immunosuppressant therapy
• elderly

What are the differential diagnoses?

• BCCs
• lupus erythematous
• actinic porokeratosis
• SCCs

How does it present?

• usually in caucasians > 40 in sun exposed area
• actinic keratoses initially present as a poorly defined area of redness or telangiectasia
• over time, the lesion becomes more defined and develops a thin, adherent, yellowish or transparent scale
• with time, the adherent scale becomes progressively thicker and yellow in colour 

How is it diagnosed?

• most of the time by clinical examination 
• advance lesions may require biopsies to differentiate from squamous cell carcinoma
• the histologic hallmark is a disordered epidermis with intraepidermal keratinocyte atypia 

What is the prognosis?

• small number of actinic keratoses will progress to SCC, and the trouble is we don't know which one is going to progress and which one is not 
• We think the thicker ones are more worrisome 
• SCC that develop on the ear, the scalp, or at the vermilion border are more likely to metastasise, so actinic keratoses in the above areas need to be treated aggressively

What treatment is available ?

• cryotherapy
• imiquimod
• Efudix 
• photodynamic therapy
• emollients containing keratolytics e.g. 2-4% salicylic acid in sorbolene cream
• Tretinoin 
• Laser

Why are there so many treatments available?

• None work 100 % 

Why do lesions recur after treatment?

1. The lesion may have been treated inadequately
2. Wrong diagnosis: could be BCCs, SCCs, Bowen's lupus erythematous or psoriasis. Therefore, failed treatment usually means biopsy

Reference:

• Habif: skin disease diagnosis and treatment third edition

Anal pruritus

Key points:

1. Anal pruritus is estimated to affect up to 5% of the population, with a male to female ratio of 4:1

2. In up to 25% of cases the anal pruritus is idiopathic

3. Even mild degrees of faecal soiling, which the patient may not be aware of, may be enough to cause an itch-scratch cycle

4. Dietary associations with pruritus anti include caffeine, alcohol, chocolate, tomatoes, spices and citrus fruit

5. Important to educate the patient about the recurring, benign nature of this irritating condition and to ensure adherence to the following simple, yet essential, measures to eliminate irritants and resolve symptoms

6. Management strategies

• Normalisation of bowel motions
• Cleaning after defaecation: rubbing or scrubbing the area should be actively discouraged
• Clothing
• Soaps and cleansers: do not use soaps
• Do not scratch: easy to say than done. May need help with topical steroid. Suggested regimen: start with methylprednisolone fatty ointment --> reducing to a moderate potency preparation such as betamethasone validate and then 1% hydrocortisone cream. Not curative. May need to return to high potency steroid from time to time 
• topical capsaicin in a 0.006% preparation (only available in a compound pharmacy. needs to be mixed with white paraffin )

References:

http://www.racgp.org.au/download/documents/AFP/2010/June/201006maclean.pdf

Male baldness

Key points:

1. 5AR converts testosteron to dihydrotestosterone, inhibition of 5AR improves hair growth and slows hair loss.

2. Finasteride (a type 2 5AR inhibitor) and dutasteride (type 1 and 2 isoenymes) are used to treat Androgenic alopecia

3. Main side effects of 5AIRs are effects on sexual function, breast enlargement and a possible increase in the risk of prostate cancer

4. Early onset of AGA is a strong predictor of early onset of severer coronary heart disease and metabolic syndrome

5. Hair thinning usually on ly becomes noticeable after losing 50 % or more of scalp hair

6. The typical history for a man with AGA is gradual onset of thinning after puberty. There is a gradual thinking of hair on the crown and vertex of the scalp, and frontal recession

7. When discussing treatment, emphasis

• no treatment will completely reverse the process
• the response to treatment is quite variable 
• some people will not respond to particular treatments

8. Treatment options 

• no treatment 
• hair piece
• medical treatment 
• topical minoxidil 2-5%
• oral finasterid 1 mg daily 
• oral dutasteride 0.5 mg daily (not approved for hair loss use yet in Australia)
• surgery 

Reference

http://gplearning.racgp.org.au/content/AFP/16Apr/Clarke.pdf

Dermatology

Topics related to Dermatology:


Ingrown toenail

Pruritus ani

Male baldness

actinic keratosis

punch biopsy

shave biopsy

BCC

Cutaneous B cell lymphoma 

Benign lymphocytic infiltrates

Dermatofibrosarcoma protuberans

Rosacea

Rosacea

• Epidemiology
• 2-3% of general population 
• Rosacea tends to occur in adults over the age of 30 years.
• In groups aged younger than 35 years or older than 50 years, men and women are affected equally, however, there is a predominance in women in the 36-50 year age group.
• Most common in fair skinned, anglo-celts
• Cause
• multifactorial and exact mechanisms are not well understood
• Genetics may play a role
• Neurovascular dysregulation and augmented immune detection and response
• infection:  the face mite demuxed folliculorum, an obligatory parasite of human pilosebaceous follicles, has been identified in elevated numbers in patients with rosacea
• Clinical features
• commonly affects the central convex areas of the face(cheers, nose, chin and forehead)
• Diagnosis can be made using the following features
• flushing 
• erythema
• inflammatory lesions 
• telangiectasia 
• Differential diagnoses of rosacea
• Acne vulgaris
• Seborrhoeic dermatitis
• Perioral dermatitis
• steroid induced acneiform eruption 
• lupus erythematousus-discoid, systemic or subacute cutaneous 
• Cutaneous sarcoidosis of the nose 
• Tinea faciei
• Essential telangiectasia
• Carcinoid syndrome 
• Drug reaction 
• polymorphous light eruption 
• atypical infections 
• contact dermatitis 
• Lupus vulgaris (cutaneous tuberculosis)
• Acne agminata
• Dermatomyositis
• Polycythaemia rubra vera
• Superior vena cava obstruction 
• Treatment: 
• Education 
• Avoid precipitants
• sun screen and hats
• 
  
• Oral agents
• tetracycline 500 mg bd 
• doxycycline 50 mg - 100 mg / day (intermittent use is preferable)
• erythromycin 500 mg bd 
• erythromycin ethyl succinate 800 mg bd
• Topical agents
• metronidazole 
• erythromycin 
• brimonidine 0.33% gel 
• azelaic acid (available as a 20% lotion or 15% gel) 
• other agents
• diclofenac
• isotretinoin for refractory cases
• clonidine, spironolactone, beta blockers,  naloxone and ondansetron
• ivermectin 
• Recurrence is routine 
• Complications 
• depression 
• ocular rosacea (symptoms include tearing, conjunctival hyperaemia, foreign body sensation, burning, stinging, dryness, itching, light sensitivity and blurred vision)
• lymphoedema
• salivary gland involvement --> reduce in salivary secretions and dry mouth 

References:

http://medicinetoday.com.au/system/files/pdf/medicine_today/article/MT2015-01-034-CHEE.pdf

Burns

I am scared of burns. I don't know what to do but after seeing burns for a few times, I start to get some understandings of it. I tend to follow the following principles (hopefully most of the burns we see at GP clinic is just minor burns):

1.  Assess the whole person, follow the principle of ABC 

2.  Know when to refer, the burns unit is available 24/7 for you to get advice (please go to RCH website), refer 

• All full thickness burn
• Circumferential burns
• All burns to face, eyes, ears, hands, feet, genitalia, perineum or a major joint, even if less than 10 %
• Chemical burns
• Electrical burns
• Burns associated with significant fracture or other major injury 
• All inhalation or airway burns
• Burns in children under the age of 12 months
• It is out of your/your hospital's comfort zone 

3. Assess the TBSA of burns. In children, their palm size is around 1 % of their body surface area. In adults, you can use your palm to gauge

4. Categorize burns into the following 3 categories and treat accordingly:

• superficial: only epidermis is involved
• superfical partial thickeness: dermis is involved, forms blister, fixomull 
• superficial deep thickness: hist white slough, red mottled, sluggish capillary return
• Full thickness: dry, charred whitish, absent capillary return 

5. 

superficial burn --> can leave it open, in infants  use non adherent dressing

partial thickness burn --> mepilex Ag Tm with crepe bandage or acticoat 3/7

Full thickness burn --> refer 

Reference:

1. RCH burns guideline 

Melanoma

Condition

• Melanoma

Definition

History

• Risk factors for melanoma
• Older age
• Men > Women
• History of sun burn (Melanoma is more associated with episodic intense sunburns than more continuous sun exposure).
• past history of melanoma increases risks by 10 fold
• non melanoma skin cancer may increase the risk fourfold 
• Family history: first-degree relative doubles the risk of a person developing melanoma

Examination

• examine the whole skin surface under good lighting 
• if melanoma is suspected, the patient should be examined for enlarged lymph nodes in the appropriate draining area (e.g. axial or groins)

Investigation 

• excisional biopsy if possible: 
• stage 0 < 0.1mm
• stage 1 < 2 mm without ulceration or up to 1mm with ulceration 
• stage 2 > 2mm 
• Stage 3: spread to lymph nodes
• stage 4: distant spread

Management

• excisional biopsy 
• often requires referral

Key points:

• In Australia, it has been shown that up to 75% of patients detect their own recurrences.
• No evidence that earlier detection by routine scans or examinations improves the outcome.
• Instruct patient to gain awareness of lesions on their skin and report any persisting symptoms promptly.
• Sun protection when the ultraviolet (UV) index is 3 or above to prevent further skin damage and subsequent skin cancers

References:

Check program 2015 September Cancer

Approach to Scabies

key points:

1. Usually present with intense itching
2. Caused by Sarcoptes scabiei. The female mite burrows beneath the skin in order to lay eggs. The eggs hatch into tiny mites and spread out over the skin and live for only about 30 days
3. The mite antigen, in its excreta, causes a hypersensitivity rash.
4. Diagnosis is by microscopic examination of skin scrapings or by response to treatment
5. Usually spread through close contacts. Children need to be kept away from school until they complete the course of treatment.
6. According to therapeutic guideline, the treatment of choice is permethrin 5% leave on for at least 8 hours or benzyl benzoate for 24 hrs repeat after 1 week. Usually permethrin is better tolerated and less drying.
7. In kids less than 6 months, the current recommendation from therapeutic guideline is still permethrin 5 % but it also stated that permethrin is currently not licensed to be used for this purpose.
8. Other treatment for children under 6 months: sulphur 10%in white paraffin once daily for 2-3 days or chromatin 10% topically once daily for 2 -3 days
9. In immunocompromised patients, it can become crusted scabies, essentially means huge number of mites in the body known as norwegian scabies. Try to get expert opinion, treat with ivermectin 200 mcg/kg as a single dose plus topical treatment.

References:
John murtagh 5th edition
eTG

Treatment for head lice

Head lice is a common problem is school aged children.
GPs need to have an approach in dealing with this problem. Give clear instruction to parents of how to deal with it. Usually diagnosis is via clinical examination. Visualisation of nits and live lice in the hair is diagnostic.

Key points:

1. It is caused by the louse pediculus humanus capitis.
2. Patients can present with significant itching on the scalp and neck.
3. Wet combing: apply generous amount of hair conditioner and comb with fine toothed comb. The conditioner stunt the lice and stops them from crawling for 20 minutes. The method only has around 40 % successful rate.
4. permethrin 1% topically, leave for a minimum of 10 minutes; repeat treatment in 7 days (there are other treatment options, please see therapeutic guideline). Use the wet combing method the day after treatment to check for live lice. If live lice are found despite treatment, that means the lice are resistant to the product used.
5. In between treatments, use the wet combing method twice to remove all eggs less 1.5 cm from the scalp.
6. Wet combing should be repeated weekly for few weeks to ensure complete clearance.
7. Bed sheets and linens should be washed with hot water 60 degrees.
8. Treat all household members
9. Notify school but can still go to school after treatment
10. Treatment resistant: use a different product or ivermectin 200 mcg/kg as a single dose with fatty food, repeat in 7 days.

References:
- eTG
- John murtagh 5th edition

Nail and hair disorder

Hair and nail disorder is something that is not very well covered in medical school. I don't even know where to start. I am hoping to have a simple approach to common hair and nail disorders which come through the door. Below is my attempt to understand hair and nail disorders a little more.

I found that John Murtagh's General practice to be a good point to start esp. the key facts and checkpoints:

• There are two types of hair: terminal hair, which is coarse and well pigmented and vellum hair, which is fine, soft and relatively unpigmented. 
• Alopecia is a generic term for hair loss
• Hair loss (alopecia) generates considerable anxiety and the fear of total hair loss should be addressed with the patient and a realistic prognosis given. 
• Androgenic alopecia is the most common cause of human hair loss, affecting 50% of men by age 40 and up to 50% women by age 60
• In telogen effluvium, the traumatic event has preceded the hair loss by about 2 months (peak loss at 4 months)
• Although severe stress could precipitate alopecia areata, day to day stressors are not considered to be a trigger. Stress seems to be a consequence of alopecia rather than the cause of it 
• Hair loss can be patchy or diffuse where it involves the entire scalp. 
• Patchy loss - alopecia aerate and trichotillomania 
• Generalised loss - telogen effluvium, systemic disease, drugs
• Alopecia areata has a poor prognosis if it begins in childhood, if there are several patches and there is loss of eyebrows or eyelashes. 
• Scarring alopecia can be an indicator of lupus erythematousus or lichen planus

Causes of diffuse hair loss

• Androgenetic alopecia
• Telogen effluvium
• Postpartum telogen effluvium 
• Alopecia areata 
• Drugs - cytotoxic and others
• Hypothyroidism
• Nutritional 
• Iron deficiency 
• Severe dieting
• Zinc deficiency
• Malnutrition 
• Post febrile state
• Anagen effluvium

Reference:

John Murtagh's general practice 5th edition 

Paediatric skin rash

It is a very common condition, and it is probably the most difficult to diagnose and manage. Fortunately, most of the time, they are self limiting.

Dr. Adrian Bonsall tried to put an end to this confusion. He developed this algorithm which was published in the Royal Children's Handbook.

This algorithm is quite self explanatory.

Professor Robin Marks also made an attempt in tackling this issue. He covers more than paediatric skin rash. His approach was referenced in John Murtagh's general practice.

He stated that most common dermatological problems fall into one of seven categories. If the rash dose not fall into these 7 categories, the person should be seen by a consultant dermatologist.

• Infections
• Bacterial: impetigo
• Viral 
• Warts
• Herpes simplex, herpex zoster
• Pityriasis rosea
• Exanthemata
• Fungal
• Tinea
• Candidiasis
• Pityriasis versicolor
• Acne
• Psoriasis
• Atopic dermatitis (eczema)
• Urticaria 
• Acute and chronic 
• Papular
• Pediculosis
• Scabies
• Insect bites
• Sun-related skin cancer
• Drug-related eruptions 

Dermatoscopy

Key points:

- Skin cancer is common in Australia and GPs need to be competent in assessing skin lesions.

- The use of a dermatosope in clinical practice has been shown to increase diagnostic accuracy and is considered the standard of care in assessing patients with pigmented skin lesions.

- All visible lesions that cannot be confidently diagnosed should be examined with a dermatoscope.

- Dermatoscope is more than a magnifying lens and light source. By eliminating reflection from the skin surface, the dermatoscope allows better visualisation of the patterns formed by pigment and blood vessels - critical features in the diagnosis of skin lesions. (Try to get one if you don't have one already)

- There are many different methods in analysing a pigmented lesions. (CASH, the ABCD method of dermatoscopy, the 7-point checklist, the Menzies method, the 3 point checklist, the revised pattern analysis and a short modification of revised pattern analysis called 'chaos and clues'.

- The method I learned is called Chaos and Clues.

- First, we need to learn how to describe pigmented structures, which are objectively defined using the following geometric terms:

• Line: a two dimensional continuous object with length greatly exceeding with 
• Pseudopod: a line with a bulbous end
• Circle: a curved line equidistant from a central point
• Clod: any well circumscribed, solid object larger than a dot; clods may take any shape
• Dot: an object too small to have a discernible shape 
• Lines are further classified into 5 types: reticular, branched, parallel, radial and curved, as these have diagnostic significance

- Blood vessels can be described the same way:

- Colour has great diagnostic significance in dermatoscopy. The main pigments are melanin and haemoglobin, and the colours produced are shown :

- The chaos and clues algorithm:

• The first step is to dermatoscopically assess the pigmented lesion for 'chaos', defined as asymmetry of structure or colour'. Chaos is assessed by pattern not shape. As perfect symmetry is biologically rare, some deviation from geometrical symmetry must be expected. It is helpful to imagine a piece of carpet that can be cut in any shape but which maintains uniform pattern.  It would be regarded as having no chaos regardless of how irregular the shape was and regardless of the presence of a little dust on one part. 
• If chaos are identified, look for clues. 
• As for all the algorithms, there are always exceptions: beware of dermatoscopic grey on head or neck, pigmented nodular lesions, parallel ridge pattern (palms or soles)

- As with many things in life, they don't come easily. It takes a lot of time to practice, practice and practice. If in doubt, do a biopsy. (Spoke to a surgeon in the past, he told me that he has never regretted taken out a normal appendix but he always regret on the ones which he didn't. Biopsy rarely results in major harms but melanoma kills.)

Reference:

1. Dermatoscopy in routine practice 'Chaos and Clues'. Australian Family Physician. 2012. 

seborrhoeic dermatitis

I saw a lady with seborrhoeic dermatitis today. Her persentation is very similar to the following photo:

I thought it is almost time to review the treatment of seborrhoea dermatitis.

1. Cause: unknown. Malassezia is an aetiology factor, hence, the use of anti fungal.
2. Infantile seborrhoeic dermatitis and adult seborrhoea dermatitis are two different conditions.

3. Scalp seborrhoeic dermatitis: treat with anti fungal shampoo or steroid lotion (apply on wet hair and leave overnight and wash it off in the morning)

4. Facial, flexural and scrotal seborrhoeic dermatitis

• Cleansed the skin thoroughly using non soap cleanser daily 
• Apply ketoconazole cream once daily for 2 to 4 weeks 
• Hydrocortisone cream can be used, apply twice daily for 1 - 2 weeks 
• LPC and Tar can be used instead of steroid. 

5. Finally just to remind myself of different classes of topical steroid:

References:

1. Australian therapeutic guideline: dermatology

2. Dermnet

Ingrown toenail (onychocryptosis)

Key points on ingrown toenail management:

1. Common presentation to primary care, especially in adolescent boys. It is typically located along the lateral edges of the great toenail and represents and imbalance between the soft tissues of the nail fold and the growing nail edge.

2. It is exacerbated by faulty nail trimming, constricting shoes and poor hygiene. Other risk factors include: male, 14-25 age group, isotretinoin use and thickening of the nail plate.

3. All patients should be instructed on correct foot and nail care. Foot hygiene includes foot baths, avoiding nylon socks and frequent changes of cotton or wool socks. It is important to fashion the toenails so that the corners project beyond the skin. (Figure 1)

4. Surgical nail excision is an easy procedure. GPs should learn how to do it.

5. There are many different ways of removing the lateral toenail. The principles are the same. Remove the part of the nail that is digging into the skin and prevent it from happening again. The following youtube video showed a technique that we use in our GP practice

6. https://youtu.be/XVDYb6ubt7I

7. The following video gives a good illustration of how to do a digital block: https://youtu.be/l2Zl15LFQWQ

8. I use the phenol ablative technique and it is well described in reference 2

9. The first time when you do it, it is better to have someone there to supervise. Once you have done it for a few times, you will be able to do it. It is not a difficult procedure.

Reference:
1. John Murtagh's General practice 5th edition
2. http://www.racgp.org.au/afp/2015/march/ingrown-toenails-the-role-of-the-gp/