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 

Tennis Elbow

Tennis elbow is quite a common presentation at general practice. The diagnosis is usually quite straight forward, occasionally, it is complicated by referred neck pain. Most of the time, the patient will be able to tell you the diagnosis is Tennis Elbow.


What is the management?

• There are many treatments available. Why ? Because none is effective. 
• Treatments include:
• physiotherapy: ultrasound, manual therapy
• NSAIDs: oral, topical. Some mild benefit with topical NSAID gel. 
• ESWT: no benefit. Cochrane recommended against it. 
• Surgery: no evidence. Final Resort. 
• Platelets rich plasma : no definite conclusion can be drawn. No benefit has been shown so far. 
• orthotic device : no definite conclusion can be drawn. 
• GTN patch: not mentioned in cochrane. Usually used in combination with an exercise program. 

I looked at cochrane review, there is no evidence for any particular treatment.

The following is a succinct summary from reference 1. 

Essentially, in acute stage, watch and wait. 

Subacute stage, refer to physiotherapy or structured exercise program. 

Chronic stage, can try cortisone injection. Refer for specialist opinion. 

• 

Patient handout: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/elbow-pain
(I read quite a few handouts online and I found the one from the victoria state government to be the most accurate and up to date)

References:

1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3781883/

2. http://www.cochrane.org/CD001821/MUSKEL_orthotic-devices-for-the-treatment-of-tennis-elbow

Acute sinusitis

I came across this article on Australian prescriber. It looks like all common management strategies that we use have little or no evidence.

Including: systemic steroid, sinus wash. There is small benefit using intranasal steroid in mild disease.

References:
https://www.nps.org.au/australian-prescriber/articles/acute-sinusitis

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

Autistic spectrum disorder

I have absolutely no interest in dealing with behavioural problems in kids, unfortunately, it is part of the job as a GP. There is a strong push from parents and schools to label these children with a diagnosis. Once they have a diagnosis, they can receive extra fundings and extra help at school. Having said that, it is also important to diagnose children with ASD early so that there is a better chance for them to function independently in the future.

What is Autism?

• Lifelong neurodevelopment disability that affects how an individual communicates and interacts with people and their environment. There are difficulties in 2 primary areas. 
• social interaction 
• repetitie behaviours and interests

How common is autism ?

• Australia 1/160 - 1/100
• USA & Europe 6-7/1000

What cause autistic spectrum disorder?

• exact cause is unknown 
• multifactorial is the keyword in exam 
• some genetic factors in play
• monozygotic twins 60%
• dizygotic 3 %
• More common in males than females 4:1
• More common in certain chromosomal disorders e.g. Fragile X syndrome 
• Increased in neurogenetic disorders e.g. tuberous sclerosis 

• NOT CAUSED BY VACCINATION

How do children with Autistic spectrum disorder present?

• Parents usually aware before 18 months 
• Most common parental concerns include delayed speech and behaviour problems

What is the Role of GP?

• Identify problem early and refer 
• Need to conduct a full history and physical exam
• Appropriate referrals e.g. hearing/vision, intervention services+/-therapy, paediatrician
• ongoing multidisciplinary management and review

What are some absolute indications for immediate refer?

• No Babbling, pointing or other gestures by 12 months
• No sharing of interests in objects with another person
• No single words by 16 months
• No 2 word spontaneous phrases by 24 months
• Any loss of language or social skills at any age

Any screening tools available ?

• M-Chat (16-30 months) Free to download online. Just type M-Chat on google.

What are some key elements in intervention?

• The earlier the better, the more the better
• early intervention between 15-25 hours a week
• Multidisciplinary supportive individualized
• In collaboration with family 
• Strategies to be able to generalise skills
• Develop functional, spontaneous communication 
• Reduction of maladaptive behaviours
• Teach functional adaptive skills
• Opportunity for neurotypical peer interaction 
• Clear Goal setting, predictability and routine 
• Continual review 

Does medication help?

• In general, medication does not help. 
• It is mainly used to treat other co-morbidities e.g. ADHD, insomnia, anxiety etc

What is the prognosis?

• 10 % adults with ASD live independently 

References: 

• Austism spectrum disorder by Dr. Gillian Brooks from diploma of child health webcast 2016