Disorders of Pigmentation 1

‫بسم ال ا لرحمن ا لرحيم‬ Pigmentation Disorders BY Dr.Ahmed Noureldin Ahmed MBBS, DCH ,DTM&H UMM-GHOILINA H.C

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Melanocyte .It is the melanin – producing cell► .It lies in between basal Keratinocytes ► It is a dendritic cell► The dendrites transfer Melanin to Keratinocytes, to► .protect their nuclei from the destructive effect of UVR The colour of the skin depends on the► ,rate of melanin production & distribution but not the number of melanocytes

Melanin is a brown-black, light-absorbing pigment, protecting the skin against ultraviolet rays The melanin system is composed of functional .units called epidermal melanin units Each unit consists of a melanocyte that supplies melanin pigment to a group of .keratinocytes Pigmentation is determined primarily by the amount of melanin transferred to the keratinocytes

Pigmentary Disorders A- Hypopigmentation Vitiligo- 1 Post-inflammatory Hypopigmentation- 2 Oculocutaneous Albinism- 3 Idiopathic Guttate Hypomelanosis-4 Hypopigmented Macules in Leprosy- 5

A- Hypopigmentation 1- Vitiligo An idiopathic, circumscribed hypomelanosis of skin and hair. It may be an autoimmune disease and is associated with other autoimmune diseases: pernicious anemia, DM, and Addison’s dis Peak incidence is 10 to 30 years of age. Occurs in all races but is most cosmetically disfiguring in darker-skinned people.

Vitiligo An idiopathic inflammatory Process that results in skin depigmentation consequent to loss of melanocytes from the epidermis and in whitening of hair that follows loss of . melanocytes from the bulb of follicles

Vitiligo It presents with milky-white patches of► varying sizes and shapes on any .body site There is no history of preceding► .inflammation Lesions are often symmetrical and► frequently involve the face, hands and .genitalia

.(Vitiligo ( cont The hair can also depigment► Trauma may induce new lesions.► ►Spontaneous repigmentation can occur and often starts around hair follicles giving .a speckled appearance Repigmentation is rare if a lesion has► persisted for more than 1 year or if the hair .is depigmented

(Vitiligo (cont

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Treatment It was an Egyptian, Professor Abdel Monem El Mofty, Department of Dermatology, Cairo University Medical School, who observed plants used in Egyptian folk medicine and began the development of modern photochemotherapy (PUVA( for vitiligo and psoriasis. In the 1940s, he used crystalline methoxsalen (Meladinine( followed by sunlight exposure to treat vitiligo

Treatment .spontaneous repigmentation may occur- 1 Topical Psoralens and topical Steroids- 2 Photochemotherapy ( PUVA( or Psoralens- 3 .and exposure to natural sunlight :Surgical Treatment- 4 a( Minigrafting b( Cultured Melanocytes (c( Permanent micropigmentation (Tattoing

Treatment ► Narrow-band UVB for repigmentation of generalised vitiligo ►new immunomodulatory agents, such as calcineurin antagonists, have recently been introduced as new promising tools to treat acquired hypopigmentary disorders

Post-inflammatory- 2 Hypopigmentation One of the most common . causes of pale skin It is much common in people with pigmented skin It may be seen as , a consequence of eczema acne or psoriasis Providing the skin disease is controlled , the pigmentation will recover slowly after many months

Post-inflammatory hypopigmentation .

Oculocutaneous Albinism- 3 autosomal recessive disorders affecting the . pigmentation of skin, hair and eyes It can affect all races☻ Melanocytes are in normal number but have♣ . abnormal function Clinically it presents with universal pale♠ . skin, pale blonde hair and a pinkish iris Photophobia, nystagmus and a squint are♥ . also present in most cases

Albinism .

Treatment involves obsessive protection against Sunlight to avoid sunburning and development of skin cancer

Idiopathic Guttate-4 Hypomelanosis This occurs most commonly in black African people and is of unknown .aetiology It presents with small (2-4 mm( asymptomatic porcelain-white macules, often on skin exposed to sunlight The borders are often sharply defined and angular . There is no effective treatment

☺ Asymptomatic Multiple scattered Hypopgmented macules on the legs Due to idiopathic hypomelanosis

Leprosy- 5 Both tuberculoid leprosy and indeterminate leprosy can present with anaesthetic patches .of depigmentation Loss of hair and decreased sweating may ► also be present in the lesions Skin lesions are numerous, varying in size and► ( form (macules, papules, plaques annular, rimmed lesion with punched-out,► hypopigmented anaesthetic centre is characteristic

Leprosy Leprosy (Hansen's disease( is caused by the acid-fast bacillus Mycobacterium leprae. Multiple asymmetrical hypopigmented Anaesthetic patches Are seen in Tuberculoid type

-Pityriasis Versicolor 6 well demarcated symmetric hypopigmented minimally scaly papules and plaques

Pityriasis Alba- 7 is a nonspecific dermatitis of unknown etiology that causes erythematous scaly patches. These resolve and leave areas of hypopigmentation . It is more prominent in dark-skinned patients It occurs predominantly in children 3-16 yrs Most cases persist for several months Several patches are usually observed and confined to the face , around the mouth and cheek, but can be seen on the trunk and limbs

Pityriasis Alba .

Treatment

►Pityriasis alba resolves spontaneously and may not require treatment . ►Apply Simple Emollient Cream(Aquaderm( 2-6 times/day( to retain moisture in the skin( ►A mild Tar Paste may be helpful for chronic cases on the trunk. ►Hydrocortisone 1% Cream , bid for a wk may be helpful . ►Photoprotection may be considered

LEUCOGO Cream ►Natural

skin care cream for depigmentation, to help in the regulation of Depigmentation ► Sufficient amount to be applied once daily at sun set ( layer on layer (

B- Hyperpigmentation 1- Freckles 2- Lentigos ( Solar Lentigo – Lentigo Maligna( 3-Chloasma 4- Postinflammatory Hyperpigmentation 5- Metabolic and endocrine effects 6- Peutz – Jegher Disease 7-Urticaria Pigmentosa 8- Acanthosis Nigricans 9- Mongolian Spots

1- Freckles(Ephelides( These appear in childhood as small brown macules after sun exposure. They fade in the winter months.

-Lentigos 2 These are a more permanent macule of pigmentation similar to freckles but they tend to persist in the winter

Solar Lentigo Flat hyperpigmented macules occur in older people on sun exposed areas Because of actinic damage

Lentigo Maligna Flat asymmetrical dark brown macules On the face ٍBiopsy of the lesion Revealed Lentigo Maligna

Treatment of Lentigos ►Liquid Nitrogen (Melanocytes freeze at -4ºC ►Krypton Laser ► Tretinoin cream( Retin A( and hydroquinone cream can lighten lentigines. Retin A is a keratolytic agent Acts by increasing epidermal cell mitosis and turnover while suppressing keratin synthesis. Important side effect is hypopigmentation, which reduces the appearance of lentigines. ►ٍSunscreen before exposure to sun ►Whitening cream ( Avalon( contains Hydroquinone 2% , Vit.E and sunscreen ►Unitone Cream with or without sunscreen

Chloasma- 3 These are brown symmetrical macules over the cheeks and forehead, and are most common in women. They can occur spontaneously but are also associated with pregnancy and the oral CP.

Postiflammatory- 4 hyperpigmentation Excessive scratching Of the vesicles of Chicken pox Leads to areas of Hyperpigmentation.

Metabolic and Endocrine- 5 Effects 1- Haemochromatosis 2-Cushing Syndrome 3- Addison’s Disease 4-Nelson’s Syndrome

Peutz-Jegher Syndrome- 6 This is an autosomal dominant genetic condition. It presents with brown macules of the lips and perioral region It is associated with gastrointestinal polyposis which almost never becomes malignan

P-J Syndrome ((cutaneous sign of systemic dis multiple lentigines on her lips . 2-4 mm hyperpigmented macules

Urticaria Pigmentosa- 7 ((Cutaneous Mastocytosis This presents most commonly with multiple pigmented macules in children. These lesions tend to become red, itchy and urticated if they are rubbed (Darier's sign( Occasionally lesions may blister and in the rare congenital, diffuse form of the disease the skin may become thickened and leather Skin biopsy shows an excess of mast cells in the skin

Urticaria Pigmentosa systemic symptoms are present such as wheeze, flushing, syncope or diarrhoea ► The condition spontaneously resolves after some years in children but is persistent in adults.

Urticaria Pigmentosa .

(Acanthosis Nigricans (A.N- 8 AN is characterized by symmetrical, hyperpigmented, velvety plaques that may occur in almost any location but most commonly appear on the intertriginous areas of the axilla, groin and posterior neck The posterior neck is the most commonly affected site in children The vulva is the most commonly affected site in females who are hyperandrogenic and obese

A.N .

Treatment of A.N The goal of therapy is to correct the underlying disease process. Correction of hyperinsulinemia often reduces the burden of hyperkeratotic lesions. Likewise, weight reduction in obesityassociated AN may result in resolution of the dermatosis. ►Topical Retinoids : promote shedding of hyperkeratotic skin. They are modifiers of keratinocyte adhesion, differentiation, and proliferation

(Treament of A.N ( Cont ► Topical Dx, → keratolytics (topical tretinoin(. ► Oral agents include etretinate and dietary fish oils. Dermabrasion and external radiation also may be used to reduce the hyperkeratosis ►20% Urea cream ►Alpha Hydroxyacid ►Salicylic acid

Mongolian Spots- 9 An asymptomatic bluish discoloration overlying the sacrococcygeal area is present at birth.

Principals of Topical Therapy Topical dermatologic treatments are used as cleansing agents, absorbents, antiinfective agents, anti-inflammatory agents, astringents (drying agents that precipitate protein(, emollients (skin softeners(, and keratolytics (agents that soften, loosen, and facilitate exfoliation of the squamous cell(

Principals of Topical therapy Topical Therapy consists of an active ingredient, an appropriate vehicle or base to deliver this, and often a preservative to maintain its shelf-life Creams : a semisolid mixture of oil and water held together by an emulsifying agent. A preservative such as parabens is added They are 'lighter' and rub in more easily than oint. They are useful for face and hand lesions ►They are less effective than ointments.

Ointment ►asemisolid , contain no water , based on oil or greases. ►They are the best treatment for dry flaky skin disorders Lotions ►These are based on a liquid vehicle such as water or alcohol. ►They are usually volatile giving a cooling effect which is a useful antipruritic ►They are useful for weeping skin conditions and on hairy skin ( e.g. the scalp (

Gels Semisolid preparations of high M/W polymers. ►They are non- greasy and liquify on contact with the skin ►They are useful for treating hairy skin (e.g. the scalp ( Pastes ►Contain a high percentage of powder in an ointment base. ►They are thick and difficult to remove from the skin. ( e.g. Lassar’s paste( used on plaques of psoriasis.

Emollients are composed of water in oil emulsions ► Emollients function in smoothing the rough skin, changing the skin's appearance, lubricating, replacing natural skin lipids, and providing occlusion. ►They fill the spaces between the corneocytes, thus providing improvement to defects in desquamation

Aerosol forms of betamethasone dipropionate and triamcinolone acetonide are available but are seldom used because they offer no advantage over creams,lotion and solutions

Solution are homogenous mixtures of two or more substances.

(Hydroquinone ( HQ ► is one of the most widely prescribed skinlightening agents in the world . ►It is the most effective inhibitor of melanogenesis. ►tretinoin is used to enhance the efficacy of HQ ►Other depigmented agents are kojic , azelaic acid, mulberry , Melatoonin and glabridin ( which is the main ingredient of Licorice extract , inhibit tyrosinase activity of melanocytes(

Guidelines for Topical Steroids • The face should be treated with mild steroid • Potent Steroids should be used for short courses. • You can use very potent steroid on the palm and soles , as the skin is much thicker. • Regular use of Emollients may lessen the need for steroid use . • Only use steroid on inflamed skin. • Use mild steroids in flexures

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