مـصـر Egypt

3 Dr. Shoukry A. Sakr

•Consultant of Dermatology & Cosmetology. •Consultant of Andrology & Marital Relations. •Cairo university , Cairo , Egypt مـصـر Egypt

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OBAGI MEDICAL CENTER KUWAIT 2006-2009 9

Dr. SHOUKRY SAKR ENCYCLOPEDIA WWW.SHOUKRYSAKRDERMAPEDIA.COM SKIN STRUCTURE

SKIN STRUCTURE SKIN STRUCTURE

Epidermis Vein

Dermis Artery

Sweat gland Hair follicle Nerve receptor Sebaceous gland 12 Hypodermis EPIDERMAL CELLS

13 EPIDERMAL CELLS

14 EPIDERMAL CELLS

15 THE DERMIS

THE DERMIS

THE DERMIS

THE DERMIS

Shaft

Sebaceous gland

Papilla Sheath

Follicle THE DERMIS

FUNCTIONS OF THE SKIN

1. The skin is the largest body organ about 6 square meters 2. Body temperature regulation i.e. thermoregulatory function 3. Prevents loss of important body fluids e.g. plasma. 4. Preventing toxic materials to enter into within the tissues. 5. Protects the body from harmful influences of factors like UVR and radiations by (melanocytes) that produce melanin which gives skin , nail and hair color. 6. Works as excretory organ : toxic substances expelled through sweat. 7. Supports and maintains the internal organs of the body. 8. Immune & infection control function. 9. Sensory organ for the pain, heat , cold , touch & pressure. 10. Manufacturer of vitamin "D" under the influence of UVR . 11. Skin is a mirror which reflects expressions as fear or joy . The internal diseases may appear to have cutaneous manifestations.

12. Skin22 texture , color and smell has a very important role in the sexual relations. PERFORATING DERMATOSES

23 PERFORATING DERMATOSES

By Dr. Shoukry Sakr

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PERFORATING DERMATOSES

25 PERFORATING DERMATOSES - Definition : Its is a group of disorders in which altered components of skin are eliminated via the by TRANSEPIDERMAL ELIMINATION (TEE) in which a material from the dermis is extruded through the epidermis to the exterior with little or no disruption of the surrounding structures -Types : (I) Acquired reactive perforating dermatoses : Primary Perforating Disorders when occuring in a diabetic patient or a patient with chronic renal failure undergoing hemodialysis 1- 2- Reactive perforating collagenosis 3- Elastosis perforans serpiginosa 26 4 - PERFORATING DERMATOSES

(II) Disorders sometimes associated with TEE : 1- annulare 2- NBLD 3- Rheumatoid nodule 4- Sarcoidosis 5- Lichen nitidus 6- Infectious : & T.B. 7- Pseudoxanthoma elasticum 8- Solar elastosis 27 PERFORATING DERMATOSES

9- Chondrodermatitis nodularis 10- Porokeratosis of Mibelli 11- Intralesional steroid 12- 13- Osteoma cutis 14- Myxoid cyst 15- Alopecia mucinosis 16- Papular mucinosis 17- Cutaneous amyloidosis

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(I) PRIMARY PERFORATING DISORDERES

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Perforating dermatoses : 1ry 1. Reactive perforating collagenosis(RPC) 2. Elastosis perforans serpiginosa(EPS) 3. Perforating folliculitis(PF) 4. Kyrle's disease(KD) 5. Acquired Perforating Dermatosis (APD) : Occur in renal failure&/or mellitus 2ry 1. Perforating GA 2. PXE 3. lipoidica 4. Chromomycosis 5. Exogenous agents: 1. ID Cs 2. Ca in drilling mud 31 PERFORATING DERMATOSES 1- Kyrle disease 2- Perforating folliculitis 3- Elastosis perforans serpiginosa 4- Reactive perforating collagenosis 5- Acquired perforating dermatosis

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33 PERFORATING DERMATOSES

1- KYRLE’S DISEASE

34 PERFORATING DERMATOSES - It may be associated with : D.M. , renal failure , hepatic insufficiency , congestive heart failure , hypothyroidism & hyperlipoproteinemia

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• The primary lesion is a small papule with silvery scale. • This eventually enlarges to form a redbrown papule or nodule with a central keratin plug. Some, but not all, of the lesions appear to be follicular. These may coalesce to form larger keratotic plaques. Koebnerization is not ordinarily described as such, but a striking linearity of lesions sometimes is noted. • Site: legs, arms , head and neck . Involvement of palms and soles is rare. • Ocular changes, including keratotic lesions of conjunctiva and cornea, have been described in a single case report. 36 PERFORATING DERMATOSES

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• A partially parakeratotic plug, which fills epidermal depression. It may or may not involve a hair follicle. • Marked acanthosis of surrounding epithelium . Within plug there is admixed basophilic debris. Keratinous material extends focally to basilar layer and may contact dermis. At this point, necrotic cellular material and degenerated connective tissue are noted to undergo transepidermal elimination. • A surrounding mixed infiltrate usually is present, including neutrophils, lymphocytes, and epithelioid histiocytes (macrophages).

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2- PERFORATING FOLLICULITIS

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3- ELASTOSIS PERFORANS SERPIGINOSA (PERFORATING SERPIGINOSA ELASTOSIS)

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• rare skin disorder in which abnormal elastic tissue fibre passes from dermis to epidermis (TEE). • Three forms: 1. Idiopathic EPS: unknown origin, possibly genetic 2. Reactive EPS: associated e’Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, , acrogeria, perforating , pseudoxanthoma elasticum. Perforating diseases that may occur in conjunction with EPS. 3. Drug-induced EPS: affects 1% of people treated with Dpenicillamine45 PERFORATING DERMATOSES

• D-penicillamine induced dermatoses: 1. cutis laxa 2. 3. pseudoxanthoma elasticum (PXE), 4. elastosis perforans serpiginosa (EPS), 5. Ecchymoses 6. lymphangiectasis. 7. Bullous diseases: Pemphigus,pemphigoid,EBA 8. CTD:SLE,dermatomyocitis 46 PERFORATING DERMATOSES

EPS most commonly appears during early adulthood (20-30 years) but may been seen in early childhood or late in life. • more common in males than females (ratio 4:1). • C/P: – cluster of small reddish papules 2-5 mm – often grouped in linear, circular or serpiginous (snake-like) patterns. – Each lesion may have a central pit, which is sometimes filled by a crusty or scaly plug. – asymptomatic but can be pruritic. • Sites : back of neck (70%), arms (20%), face (11%), legs (6%) and trunk (3%).

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• DD: – Dermatophytosis – Perforating collagenosis – Perforating granuloma annulare • Treatment: spontaneously resolves without complications after a few years. – no cure for EPS. – topical tazarotene(effective in 2pt). – Imiquimod:effective treatment after 10 weeks of therapy.

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4- REACTIVE PERFORATING COLLAGENOSIS

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1- Familial (AR) more in infancy 2- Acquired in association with D.M. & renal failure

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5- ACQUIRED PERFORATING DERMATOSIS

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• irregular, epidermal hyperplasia with hypergranulosis. Centrally, there is a thick, hyperkeratotic and parakeratotic plug containing basophilic material that is embedded in an epidermal invagination59 . PERFORATING DERMATOSES

Dilated follicular infundibulum filled with keratinous and necrotic debris . The follicular epithelium is disrupted in one point, and degenerated, basophilic dermal collagen is seen entering the perforation

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• Connective tissue fibers and cellular debris are extruded through the epidermis via a spiraling path.

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acid orcein- Giemsa stain: Elastic fibers are black.

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HYPERKERATOSIS LENTICULARIS PERSTANS (FLEGEL’S DISEASE)

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COLLOID MILIUM

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AINHUM (CONGENITAL CONSTRICTION)

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PSEUDOINHUM (AUTOAMPUTATION)

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PERFORATING NECROBIOSIS LIPODICA

77 PERFORATING DERMATOSES • oval violaceous patch that expands slowly. The advancing border is red and central area turns yellow-brown. The central area and has a waxy surface; telangiectasias become prominent • HP : degeneration of collagen, granulomatous inflammation of subcutaneous tissues and of blood vessels, capillary basement membrane thickening, and obliteration of vessel lumina. • TEE of degenerated collagen through hair follicle in is rare, clinically manifesting as multiple hyperkeratotic plugs on surface of NL lesion . • The most commonly affected site in NL is leg( 85%). Other locations, however, may include hands, fingers, forearms, face, and scalp. The presence of multiple lesions is very rare

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Degenerated collagen

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Infiltrate of inflammatory cells with foamy cells encircling the altered connective tissue

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Transfollicular elimination of necrotic material (Trichromestain)

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83 OTHER DERMATOSES

PERFORATING GRANULOMA ANNULARE

84 OTHER DERMATOSES • Rare • young adults and is more common in women • C/P:lesions pass through four stages: i) erythematous or skin colored papules ii) pustular-like lesions that exude thick, creamy, or clear viscous material iii) umbilicated or crusted papular-nodular lesions and iv) atrophic There are two presentations of PGA: a localized form with lesions mainly in upper limbs and palms and a more frequently seen, generalized form in abdominal area, trunk, upper and lower limbs. The generalized form has a chronic course with 37% lasting > 10 y .

85 OTHER DERMATOSES

• DD: 1. reactive perforating collagenosis 2. papulonecrotic tuberculid 3. ulcerated rheumatoid nodule 4. perforating sarcoidosis 5. ulcerated or perforating necrobiosis lipoidica diabeticorum (NLD) 6. giant molluscum contagiosum . 86 OTHER DERMATOSES

87 OTHER DERMATOSES

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GRANULOMATOUS ROSACEA (LUPOID ROSACEA)

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• variant of rosacea with a chronic and non-relapsing course, which presents with brownish-red papules -on an infiltrated erythematous base, features which alone are enough for diagnosis of the disease . Clinically, it may be differentiated from classic rosacea by lesion location, which generally occur on the lateral surface of the face and in the neck below the mandible, and histopathologically by the presence of epithelioid granulomas 92 OTHER DERMATOSES

93 OTHER DERMATOSES

94 هذا ماأعلم وهللا أعلى وأعلم والعلم كله عند هللا

95 Thank you Dr. SHOUKRY SAKR

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