Perforating Dermatoses
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مـصـر Egypt 3 Dr. Shoukry A. Sakr •Consultant of Dermatology & Cosmetology. •Consultant of Andrology & Marital Relations. •Cairo university , Cairo , Egypt مـصـر Egypt 5 6 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 24 PERFORATING DERMATOSES PERFORATING DERMATOSES 25 PERFORATING DERMATOSES - Definition : Its is a group of disorders in which altered components of skin are eliminated via the epidermis 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- Kyrle disease 2- Reactive perforating collagenosis 3- Elastosis perforans serpiginosa 26 4 - Perforating folliculitis PERFORATING DERMATOSES (II) Disorders sometimes associated with TEE : 1- Granuloma annulare 2- NBLD 3- Rheumatoid nodule 4- Sarcoidosis 5- Lichen nitidus 6- Infectious granulomas : leprosy & T.B. 7- Pseudoxanthoma elasticum 8- Solar elastosis 27 PERFORATING DERMATOSES 9- Chondrodermatitis nodularis 10- Porokeratosis of Mibelli 11- Intralesional steroid 12- Calcinosis cutis 13- Osteoma cutis 14- Myxoid cyst 15- Alopecia mucinosis 16- Papular mucinosis 17- Cutaneous amyloidosis 28 PERFORATING DERMATOSES (I) PRIMARY PERFORATING DISORDERES 29 PERFORATING DERMATOSES 30 PERFORATING DERMATOSES 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 diabetes mellitus 2ry 1. Perforating GA 2. PXE 3. Necrobiosis 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 32 PERFORATING DERMATOSES 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 35 PERFORATING DERMATOSES • 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 37 PERFORATING DERMATOSES 38 PERFORATING DERMATOSES 39 PERFORATING DERMATOSES • 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). 40 PERFORATING DERMATOSES 2- PERFORATING FOLLICULITIS 41 PERFORATING DERMATOSES 42 PERFORATING DERMATOSES 43 PERFORATING DERMATOSES 3- ELASTOSIS PERFORANS SERPIGINOSA (PERFORATING SERPIGINOSA ELASTOSIS) 44 PERFORATING DERMATOSES • 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, scleroderma, acrogeria, perforating granuloma annulare, 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. anetoderma 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%). 47 PERFORATING DERMATOSES • 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. 48 PERFORATING DERMATOSES 49 PERFORATING DERMATOSES 50 PERFORATING DERMATOSES 51 PERFORATING DERMATOSES 4- REACTIVE PERFORATING COLLAGENOSIS 52 PERFORATING DERMATOSES 1- Familial (AR) more in infancy 2- Acquired in association with D.M. & renal failure 53 PERFORATING DERMATOSES 54 PERFORATING DERMATOSES 55 PERFORATING DERMATOSES 5- ACQUIRED PERFORATING DERMATOSIS 56 PERFORATING DERMATOSES 57 PERFORATING DERMATOSES 58 PERFORATING DERMATOSES • 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 60 PERFORATING DERMATOSES • Connective tissue fibers and cellular debris are extruded through the epidermis via a spiraling path. 61 PERFORATING DERMATOSES acid orcein- Giemsa stain: Elastic fibers are black. 62 PERFORATING DERMATOSES HYPERKERATOSIS LENTICULARIS PERSTANS (FLEGEL’S DISEASE) 63 PERFORATING DERMATOSES 64 PERFORATING DERMATOSES 65 PERFORATING DERMATOSES COLLOID MILIUM 66 PERFORATING DERMATOSES 67 PERFORATING DERMATOSES 68 PERFORATING DERMATOSES AINHUM (CONGENITAL CONSTRICTION) 69 PERFORATING DERMATOSES 70 PERFORATING DERMATOSES 71 PERFORATING DERMATOSES 72 PERFORATING DERMATOSES PSEUDOINHUM (AUTOAMPUTATION) 73 PERFORATING DERMATOSES 74 PERFORATING DERMATOSES 75 PERFORATING DERMATOSES 76 PERFORATING DERMATOSES 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 atrophies 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 necrobiosis lipoidica 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 78 PERFORATING DERMATOSES Degenerated collagen 79 PERFORATING DERMATOSES Infiltrate of inflammatory cells with foamy cells encircling the altered connective tissue 80 PERFORATING DERMATOSES Transfollicular elimination of necrotic material (Trichromestain) 81 PERFORATING DERMATOSES 82 PERFORATING DERMATOSES 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