Module 4C Psoriasis and other Papulosquamous diseases

We conclude this module looking at a few unusual diseases. PRP has been mentioned before but Grover's disease is increasingly common in the elderly with these red scaly itchy papules around the chest and lower back and under the breasts. Darier's disease is it's histological big brother with marked acantholysis but the papules are the same except there are many more of them! Look at the distribution of the lesions in Darier's and the summer exaccerbation. Note it is an autosomal dominant disease. Keep an eye out for Lichen sclerosus. Consider it with any itchy red persistent perivulval rash. Look for the atrophy and the white sclerosis sometimes with a bit of haemorrhage into the affected genital skin.

There is a podcast on this module that you can listen to either before you do the reading of the module or after, whichever you prefer!
Podcast on Grover's disease and Lichen sclerosus. Just click on the little arrow next to the loudspeaker motif on the left side of the bar to start the podcast.

View this YouTube video on the images in module 4C



Grover's disease
Once you recognise this condition you will be surprised how common it is in Australia. The lesion is an itchy scaly red papule on the trunk and back just under the breasts. The itch is very troublesome and the condition is made worse by excess sweating. It does tend to wax and wane a bit.

It responds to topical steroids initially try moderate strength such as Celestone M cream but then go to Advantan and ultimately diprosone or elecon creams if the patient is not settling. Minimise excess sweating. Appropriate cotton clothing and air conditioning help. The other name for the condition is transient acantholytic dermatosis. The acantholysis bit comes from the histological feature of splits in the epidermis and inflammation at the dermoepidermal junction. Unfortunately the condition is often persistent rather than transient as its name tries to suggest!

Check out Dermnet on Grovers disease

An itchy trunkal rash particularly in the elderly.

Close up of the red scaly papules

Grovers histology with localised acantholysis

Lichen sclerosis et atrophicus is an autoimmune disease of genital epithelium seen in both males and females, giving rise to sclerotic, sometimes telangiectatic, skin that will tear and break easily and be quite uncomfortable and itchy, especially in females. Rarely you can have extragenital lichen sclerosis. The condition in children may be misdiagnosed as sexual abuse. It requires a strong topical steroid such as Diprosone OV to suppress the inflammation for a month and then a weaker steroid such as hydrozole cream can be used in the longer term to keep the condition suppressed until hopefully it burns itself out.

Diagnosis In the genital area you may initially diagnose this as lichen simplex, a type of eczema but it does not give the atrophy and slight bleeding into the skin that lichen sclerosus does. Also LS and A can extend around the anus in a figure of 8 distribution so always examine the perianal skin.

Dermnet on Lichen sclerosus

A uTube video on the histopathology of Lichen sclerosus

Vulval lichen sclerosus with white epidermal atrophy and subdermal sclerosis. Note tearing of superficial capillaries

The rarer papular type on the body with a hint of violaceous colour.

Pictures of lichen sclerosus in a young girl and an older woman

Pityriasis rubra pilaris
I was not going to discuss this condition again but as several of you have submitted cases to the VCM I thought I better. In essence it starts like psoriasis in the scalp and then slowly progresses distally. If you catch it early look for the follicular papules seen in image 3. Often they are best seen on the back of the neck or the hands. Typically there is ectropion and palmar and plantar keratoderma.



Listen to a podcast on PRP

See this Consultation on PRP 

Note the clear islands of sparing of normal skin

Note the imminent ectropion and the marked facial involvement

Note the follicular basis of the lesions. Often this is lost as they all join up.

PLEVA Pityriasis Lichenoides et Varioliformis Acuta! This is a rare papulosquamous disease that presents with polymorphous papules usually in covered areas on the trunk or on the inner aspects of the arms but spares sun exposed areas. The papules are usually a red brown colour and can become purpuric,scaly and sometimes break down and have a necrotic surface.It is probably of viral aetiology as we see it in outbreaks periodically.The lesions can resolve with both hyper and hypopigmentation and crops can arise for months.

Erythromycin seems to help possibly because of it's anti inflammatory effects and sunlight and artificial UV exposure also seem to settle it down. Methotrexate has been used for very severe cases but how that ties in with a viral aetiology is beyond me!

Dermnet on PLEVA

Also look at this Consultation on PLEVA

Noted the scabbed papules on the trunk, a typical area.

Histology showing a psoriasiform epidermal disease

Annotated

Dariers Disease This condition is red and scaly and really looks like a bad version of Grovers disease! You have the same itchy keratotic papules but they are found closely packed together in areas such as the groins and behind the ears. The groin image almost shows the tears in the epidermis you see in Hailey-Hailey disease as Darier's also shows some acantholysis or separation of cells in the epidermis.

Treatment This condition is treated with oral retinoids particularly Neo Tigason (Acetretin or Etretinate) at 25-50mgs daily. Note this drug is teratogenic in females. Darier's is worse in the summer months and is often complicated by secondary staph infection of the groins and face requiring Dicloxacillin orally or topical Bactroban (Mupirocin)cream. A strong topical steroid cream also helps even in the flexures in this condition. The disease is inherited as an autosommal dominant disorder.

Dermnet on Darier's disease

Note the grouped papules, oozing and secondary infection

Grouped follicular papules , oozing and infection

Note the fissuring in the groin skin and the thick grouped papules

Below is a little piece from Journal Watch on the T cell type infiltrates in both Psoriasis and Atopic Eczema. The arguement has been are both conditions antigenically mediated or are their intrinsic epithelial defects as well?

Psoriasis and Atopic Dermatitis Are Triggered by Responses to Specific Antigens And intrinsic epithelial abnormalities have a role.

Curiously, psoriasis and atopic dermatitis (AD) rarely occur in the same patient. Psoriasis is driven by Th1 and Th17 helper T cells, while AD is driven by Th2 cells. Investigators studied three patients with both AD and psoriasis. Histologic examination of skin samples from AD and psoriasis lesions showed characteristics of the respective diseases. Psoriasis plaques contained large numbers of Th1 and Th17 cells secreting cytokines -interferon and interleukin (IL)-17. The AD samples contained relatively higher numbers of Th2 and Th22 cells secreting IL-4 and IL-22.

Koebner reactions can cause psoriasis in traumatized skin. After a patch test challenge with house-dust antigen, the invading T cells were mostly Th2 cells, and two patients developed eczema (but not psoriasis). In nickel patch tests, the infiltrate in nickel-sensitive patients was dominated by Th1 and Th17 cells, and many T cells in T-cell clones that were established from extracted lymphocytes reacted with nickel. Only a few cells extracted from psoriasis lesions reacted with nickel in these same patients.

Based on these results, the authors conclude that intrinsic epithelial abnormalities are not involved in the pathogenesis of AD or psoriasis. Instead, they propose that T cells migrate into skin in response to distinct but different antigen triggers.

In patient 1, previous adalimumab treatment had cleared the psoriasis but with a flare of the AD; now, both conditions responded to ustekinumab. Both diseases responded to cyclosporine in patient 2. Treatment and response were not specified for patient 3.

Comment: These findings support the current party line regarding the role of various T-cell types in atopic dermatitis and psoriasis. I do not see how the findings rule out "intrinsic epithelial abnormality" in either condition. In fact, good evidence supports the role of such abnormalities in both AD (in filaggrin expression and tight junctions) and psoriasis (intrinsic hyperproliferation). Interestingly, the researchers did not detect mutations in filaggrin genes in these patients.

— Mark V. Dahl, MD
Published in Journal Watch Dermatology July 20, 2011
CITATION(S):
Eyerich S et al. Mutual antagonism of T cells causing psoriasis and atopic eczema. N Engl J Med 2011 Jul 21; 365:231.

Psoriasis nails

Psoriasis in coloured skin

Ultraviolet therapy with narrow band UVB is very useful in guttate and extensive thin plaque psoriasis

Some extemporaneous preparations for scalp psoriasis

UNG COCOIS Co.

Coconut oil 30%

Salicylic acid 2 to 4%

Precipitated sulphur 2 to 4%

Coal tar solution 6.25 to 12%

Emulsifying ointment to 100%

 

Dithranol 0.3-1%

Salicylic acid 5%

Emulsifying wax 10%

Liquid Paraffin to 100%

Apply 10mins then wash off. Increase to 30min if tolerable.

If the consistency is too thick then loosen up in hot water. It is best to put the mixture into a plastic “tomato sauce” squeezy bottle (Boston round), so it can be applied easily to the scalp.

 

3% LPC 3%sulfur 3%ac sal in UEA overnight.

 

Resorcinol 2% Salicylic acid 3% Coal tar solution 10% Quillaia Tincture 10% Glycerine 10% Soap Spirit to 200mL

Extensive pustular psoriasis on very red and tender skin

Localised psoriasis on the penis Use hydrozole cream only.

Click here to take survey

Have a look at these images of Psoriasis, Tinea or Eczema. Guess the correct answer. No individual results are recorded. I will go over the images in a webinar. It is just a way of getting a group response to some images.

Have a look now at the images in the Image Diagnosis section and filter for Module 4. Try to answer each of these as best you can before looking at the answer.

When you have completed your reading for this module 4 by the Thursday of week 1 go to www.aidexams.com  and take the Module 4 MCQs Your results in this test will be recorded to help me frame the  teleconference.

At the end of week 2, before the second Thursday teleconference , you should also take this Written online assessment on Module 4 in www.aidexams.com NB Answer all 5 questions before pressing Submit at the bottom of the test and keep a Word file of your answers. The password or OPD is world

View the Module 4 Webinar below