Aquagenic syringeal acrokearatoderma

Aquagenic syringeal acrokeratoderma:
It is eccrine gland disease.
Pathogenesis is poorly understood
Stratum corneum dysfunction and aberration in eccrine gland structure can be possible for the disease presentation.

Clinical features:
Patient complains of appearance of whitish translucent Papules over palms after immersion of hand in water.
Once the contact with water is removed it disappears in 10-20 minutes.
Sometimes it is accompanied by sensation of tingling and tightness of palm.
Excessive wrinkling of skin over palms develop on repeated exposures
 Other sites:
Dorsal hands or fingers
Rarely sole is  involved

Associations:
Cystic fibrosis
Family history (+) in some cases
Hyperhidrosis

Conditions should be distinguished from hereditary papulotranslucent acrokearatoderma.
Histopathology is helpful to confirm diagnosis
On histopathology orthokeratosis and  dialated eccrine ducts are seen in aquagenic acrosyringeal kearatoderma while eccrine gland is normal in hereditary variant.

Treatment:
No standard treatment is available
Options:
1) Aluminum chloride hexahydrate 20% at night
2) Barrier emollients petroleum based
3) 12% ammonium lactate cream
4) Botulinum toxin injection
Biopsy should be performed immediately after immersion of hand in water.

This disease is rare but sometimes it may be under diagnosed.
The hand in bucket sign is very typical of this condition.

Fig:

Multiple whitish Papules over palm after water exposure 

Freckles

Freckles:
It is pigmentory condition.
It's also called ephelids.
Freckles are brownish pigmented Macules of various sizes over face.
Other sites: axilla and inguinal regions.
When these sites other than face  are involved then we suspect neurofibromatosis type 1.
Freckles are also present in one genodermatoses  called xeroderma pigmentosa.

Most patients have freckles only over face. In that case it's very easy to treat it.
Diagnosis is solely based on clinical examination.

Treatment:
1) Topical treatment:
A)Use of Depigmenting agents : Kojic acid
B) Phenol 80% over spots

2) lasers:
a)Frequency doubled Nd. YAG 532 nm
b) IPL (500-1000 nm )
c) Q switched ruby laser 694nm
d) 755nm alexandrite laser
e) 532 nm KTP laser

3)chemical peel
Side effects: post inflammatory hyper or hypo pigmentation.

Remember: freckles don't require treatment always.
When cosmetic concern is  there treatment options can be chosen.

Freckles do have 2.5 fold increased  risk of sunburn and poor response to tanning.

Difference between freckles and lentigo:
Freckles.                                Lentigo
1) Darkening on sun exposure No change
2) Presents in early childhood Elderly people
3) No increase in melanocyte Increase in melanocyte number
    Number


Use of sunscreen with spf 30+ is strongly recommended in these patients.

Freckles over cheeks and nose 

Moisturizer

Moisturizer:
It is to maintain moisture of your skin.
It is used very routinely by females.
But do you know which moisturizer is the best for you?
I will educate you about the mechanism behind moisturizer...

Types:
1) occlusive
2) humectant 3) emollient

Emollient = occlusive +  humectant

occlusive:
Beeswax, soybean oil
Humectants: urea, sugar, sorbitol.
Emollient: lanolin 

Melasma

Melasma :
It is a pigmentory disorder characterised by brownish Macules  over face especially cheeks and forehead.
Types:
1) Epidermal
2) Dermal
3) Mixed
Depending on the level of pigmentation into the skin.
Another classification is based on the location of skin affected
1) Malar
2) Mandibular
3) Centrofacial

Woods light is used to determine the type of melasma.
 Cause:
1)Increased estrogen levels in females especially pregnancy
2) prolonged sun exposure

When it happens after or during pregnancy it's called chloasma (pregnancy mask).

How to treat??
1) Topical treatment
2) Systemic
3) Laser or light therapy
4) Chemical peel

1) Topical treatment:
A) Depigmenting agents:
1) Kojic acid
2) Mulberry extract
3) Hydroquinone 4%

B) Retinoids
C) Steroids
Most  common topical treatment :

Kligman formula (Triple combination formula):
Hydroquinone+ tretinoin+ fluocinonide


1) Systemic treatment:
a) Capsule vitamin c 200mg 1OD for 2-3 months.
b) Tab tranexamic acid 250-500 mg 1OD

2) Lasers:
Nd Yag laser toning 1064 nm is good for maintenance.
Intense pulsed light therapy is another alternative.
3) Chemical peel:
a) Glycolic acid peel 30%

Peeling procedures have downtime so most of the patients don't prefer.


Mesotherapy with tranexamic acid is also tried in some centres.

Recurrence rate is high with any above treatment.
No standard treatment is available. It is all depends on individual skin type.

Advice :
1) Use of sunscreen with spf 50+ and UVA+++
2) Use of wide brimmed hat in mid-day sun
3) Use of retinoids at night time

Skin types

Skin types :

Bauman skin type indicator (BSI):
Main four types:
1) dry vs oily
2) pigmented vs non pigmented
3) sensitive vs irritant
4) wrinkled vs tight
With these basic types individual skin can fall in any of 16 types

skin catogerization is important while treating any skin disease
It is important while choosing over the counter cosmetic products.
So the following table will help you to define your skin type.

Oily sensitive skin is more prone to acne and or rosacea.
Pigmented skin should be protected from excessive sun exposure.
Dry skin is likely to develop eczema.

Fitzpatrick skin typing is universal method 

Rosacea

Rosacea:
It is chronic inflammatory disease characterized by erythema and pustules.
Histopathology:
1) Dialated dermal vessels
2) Sebaceous gland hyperplasia
3) Inflammatory cell infiltrate
Age group: 20-50 years ( middle age)
Cause: unknown
Exacerbating factors:
1) Spicy food
2) Angry mood / emotional stress
3) Hot weather
4) Hot shower bath for prolonged time
5) Exposure to sunlight
6) Alcohol
7) Exercise
8) Cosmetics
9) Hot drinks
10) Medications

Disease Association with acne is common.
Clinical features:
Sites affected: cheek, nose,forehead,chin
Earliest symptom: flushing
1)Redness over face :
Diffuse erythema over cheeks and nose
Sometimes accompanied by itching and burning sensation
2) prominent telangiectasia
3) Papule and pustules formation
4) sebaceous gland hyperplasia resulting in swelling.

Types of Rosacea:
1) Erythematotelangiectatic (ET)
2) Papulopustular Rosacea
3) Rosacea fulminans ((pyoderma faciale)
4) Rhinophyma ( nose deformity)




Approach to patient:
History of exacerbating factor
Clinical examination with dermatoscope:
For visualization of tiny capillaries

Treatment:
1) Topical treatment
2) Systemic treatment
3) Lasers

Acne vulgaris

Common age: adolescent age 12-25 years.

But it can affect newborns and old aged patients. 

Cause: hormone imbalance is the main cause for development of acne

Types:

1] comedones : white heads or black heads

(mild variety)

2] papulopustular acne are of moderate variety

3] nodulocystic acne is severe form of acne

4] acne fulminans and

5]pyoderma faciale 

last two forms of acne which require aggressive treatment.

How is this treated?

1] Topical treatments: 

Tretinoin 0.025% cream at bedtime is effective in most of patients.

Others: adapalene gel 0.1%, brevoxyl cream, niacinamide gel, clindamycin cream, glycolic acid cream

These creams are useful for mild to moderate acne.

2] systemic/ oral treatments:

Tab azithromycin 500mg once a day for 3 days in a week for 1-3 months

Tab doxycycline 100 mg once daily for 1-3 months.

Tab minocycline 50mg once daily 

Tab isotretinoin 0.5-1 mg/kg once daily for 1-6 months

(Isotretinoin treatment is very effective in reducing acne eruption but careful monitoring of liver function and lipid profile is advised). One more important note: Isotretinoin is contraindicated in pregnancy.

Nodulocystic acne can be treated with intralesional triamcinolone acetonide 10 mg/ml.

Treatment of acne fulminans:
Systemic steroids at the beginning to control inflammation and then start antibiotics or isotretinoin.