Pigmented lesions


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Oral mucosa reflects the health of the whole human body at a first glance.If any disorder is present in the system it will first appear in oral cavity. Here is an overview of certain pigmented lesions.

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  • Find histology Lever
  • Look for image of industrial hyperpigmentation
  • Examples of bronze diabetes & addison’s diseaes
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  • Pigmented lesions

    1. 1. CONTENTS Introduction Classification Endogenous Exogenous References Conclusion
    2. 2. INTRODUCTION The word pigment is derived from latin word meaning “colour or colouring”Normal skin pigmentation is influenced by: Degree of vascularity Amount & location of melanin Presence of carotene Thickness of the horny layer
    3. 3. Overproduction Over population Sun Benign nevi, malignantexposure,drugs,hormones melanoma
    4. 4. PIGMENTED LESIONS Diffuse & bilateral Focal Adult onset Red-blue-purple Blue-grey Brown Systemic No Blanching systemic •Addisons •Amalgam •Melanotic •Varix • Drug disease •Hemagioma Tattoo macule •Heavy induced •Foreign •Pigmented • Post infl Non Body tattoo nevus metal •Kaposis ammatory •blanching •Blue nevus •Melanoma • Smokers •Melano sarcoma • melanosis Thrombus acanthoma •HematomaAdel Kauzman, Marisa Pavone, Nick Blanas. Pigmented Lesions of the Oral Cavity: Review, DifferentialDiagnosis, and Case Presentations. Journal of the Canadian Dental Association.November 2004, Vol. 70, No. 10
    5. 5. PIGMENTED LESIONS ENDOGENOUS Hemoglobin Varix, hemangioma, Kaposi sarcoma, Circulating erythrocytescoursing through patent vessels. angiosarcoma, hereditary hemorrhagic telangiectasia•consequence of blood Hemosiderinextravasation- trauma or a defect Ecchymosis, Petechia, Thrombosed•generalized hemosiderin tissue Varix, Hemorrhagic Mucocele,deposition-Hemochromatosis Hemochromatosis PIGMENTED Melanotic Macule, Nevus, Melanoma, Basilar Melanosis With Incontinence
    6. 6. Exogenous Pigmentation of Oral MucosaSource Color Disease ProcessSilver amalgam Gray, black Tattoo,iatrogenic traumaGraphite Gray, black Tattoo, traumaLead, mercury, Gray Ingestion of paintbismuth or medicinalsChromogenic Black, brown, Superficialbacteria green colonization
    7. 7. HEMANGIOMA Childhood-hamartoma; adult-varicosity Etiology : congenital & traumatic colour-depth of vascular proliferation Tongue (intrinsic muscles), lip, buccal mucosa & palate ( Raised, nodular Benign vascular hamartomas-range from- flat reddish blue macule to a nodular blue tumefaction Congenital- strawberry nevus
    8. 8.  Port wine stain involves facial skin is flat &magenta I colour. Skull radiograph: vessel wall calcification yield bilamellar radiopaque tracks “Tram line calcification” Bubbly or honeycomb trabeculated appearance Overlying cortex is expanded and thinned, but complete cortical disruption and invasion into soft tissue are not present Diascopy Intraluminal clots form- palpable and do not blanch
    9. 9. Sunburstappearance
    10. 10.  Calcified nodules/phleboliths- radiographically evident 85% of childhood-onset hemangiomas spontaneously regress after puberty Conventional surgery, laser surgery, cryosurgery Sclerosing agents - 1% sodium tetradecyl sulfate (intralesional injection)- .05 to 0.15 ml/cm3 Cutaneous port-wine stains - subcutaneous tattooing or by argon laser
    11. 11. Differential diagnosis: Mucocele & Ranula: soft &fluctuant Aneurysm: pulse are detected
    12. 12. Histopathology Cavernous -large dilated vascular channels lined by endothelial cells without a muscular coat Cellular/capillary-endothelial proliferation, vascular lumina are very small Intramuscular –deep lesions
    13. 13. Sturge Weber syndrome Encephalotrigeminal angiomatosis Port wine stain (nevus flammeus) – leptomeninges of cerebral cortex Mental retardation, hemiparesis, seizures Ocular lesions Calcification d/d- angioosteohypertrophy syndromePort wine stain + varices + hypertrophy of bone
    14. 14. Hemangioma Vascular malformationDescription Ab endothelial cell Ab blood vessel proliferation developmentElements Inc no of capillaries Mix of artery, vein, capillaries (AV shunt)Growth Rapid congenital, Grows throughout ceases pubertyBoundaries Circumscribed;rarely Poorly circumscribed affects boneThrill & bruit absent presentInvolution Spontaneous Does not involuteResection Easy Difficult, surgical haemorrhageRecurrence Uncommon Common
    15. 15. Blue rubber bleb nevussyndrome Bean’s syndrome Multiple small & large cavernous hemagioma Skin & GI tract + mouth Childhood/young adulthood Life threatening-blood loss->anemia & Fe deficiency
    16. 16. Varix Pathologic dilatations of veins or venules are varices or varicosities Site- ventral tongue- tortuous ,serpentine blue, red, and purple elevations Progressively prominent with age degenerative change in the adventitia of the venous wall-painless & non haemorrhagic interfere with mastication
    17. 17.  focal dilatation-varix Elders, lower lip, raised Blue/red/ purple; surface mucosa -lobulated or nodular Diff diagnosis-hemangioma-age-etiology- lip or cheek biting-growth potential-same histology-thrombi-organization & canalization Same t/t
    18. 18. ANGIOSARCOMA Malignant vascular neoplasm. Arise anywhere in body Colour: red ,blue or purple . Rapidly proliferative present as nodular tumor. can arise from blood or lymph vessel endothelial cells / pericytic cells of vasculature. Treated by radical excision
    19. 19. KAPOSI SARCOMA Tumor of putative vascular origin HHV-8 2 forms-elderly men ( oral mucosa & skin of lower extremities) , (2) children in equatorial africa ( lymph nodes)-aggressive & lethal Slow progressive, less metastasis Oral tumors - red, blue, and purple-hard
    20. 20. KAPOSI SARCOMA + HIV Oral lesions - posterior hard palate Begin as flat red macules of variable size and irregular configuration Numerous isolated and coalescing plaques Eventually- increase in size ->nodular growths- entire palate, protruding below the plane of occlusion Facial gingiva
    21. 21.  D/d-pyogenic granuloma, giant cell granuloma Bacillary angiomatosis-bartonella henselae-rare in oral mucosa Early stage-no t/t; later-electrocautery/excision Intralesional 1% vinblastine sulfate-less post injection pain-multiple biweekly injections Proliferating spindle cells with mild pleomorphism + plump endothelial cells extravasation of erythrocytes + hemosidren granules
    22. 22. HEREDITARY HEMORRHAGIC TELANGIECTASIA/Rendu-Osler-Weber syndrome Multiple round or oval purple papules measuring less than in 0.5cm in diameter. Genetically transmitted disease Site: vermilion &mucosal surfaces of lips as well as on the tongue &buccal mucosa. multiple microaneurysms, owing to a weakening defect in the adventitial coat of
    23. 23.  Same lesion in nasal mucosa-epistaxis differential diagnosis-petechial hemorrhages (platelet disorder)-macular, red/blue, not genetic CREST syndrome Selective embolization electrocautery(series of procedures) using local anesthesia
    24. 24. Ephelis Increase in melanin pigment synthesis by basal-layer melanocytes, without an increase in the number of melanocytes Freckle Vermilion border of lips ( lower lip). Lesion is macular ranging from small to few cms Solar exposure M=f Adults, asymptomatic
    25. 25. Oral Melanotic Macule Intraoral counterpart Oval or irregular outline, Brown or black, gingiva, palate, buccal mucosa. Once they reach a certain size, they do not tend to enlarge further Differential diagnosis - nevus, early superficial spreading melanoma, amalgam tattoo, and
    26. 26.  Biopsy melanin-containing dendritic cells are seen to extend high into a thickened spinous layer. melanoacanthoma Surgery –no predisposition to melanoma Myxoma syndrome-soft tissue myxoma + endocrinopathy Laugier –Hunziker syndrome/phenomenon- acquired disorder + lips,oral, finger+ subungual melanocytic streaks
    27. 27. Nevocellular Nevus/nevomelanocytic nevus/pigmented nevus Benign proliferations of melanocytes Nevus cells - localized to basal layer- junction of epithelium and basement membrane+ connective tissue Minimal proliferation Macular, flat and brown, regular outline Junctional nevi
    28. 28. Melanocytes form clusters at the epitheliomesenchymal junction proliferate down into the connective tissue Dome shaped appearance Compound nevi Lose their continuity with surface epithelium+ cells become localized - deeper connective tissues Intradermal nevi (skin) Intramucosal (mouth)
    30. 30. Blue nevus Melanocytic cells reside deep in the connective tissue and because the overlying vessels dampen the brown coloration of melanin, yielding a blue tint More spindle shaped + more pigment Macular or nodular; brown intraorally Palate, gingiva, buccal mucosa & lips
    31. 31. Malignant Melanoma Sun exposure->malar region Facial cutaneous melanomas –macular/ nodular Brown -black -blue, with zones of depigmentation. Jagged irregular margins-nevi(smooth outline) Elder, M>F
    32. 32. “Lentigo maligna melanoma” or “hutchinson’s freckle” Facial skin lesions – atypical melanocytic hyperplasia /melanoma in situ. Melanocytic tumor cells spread laterally and superficially Radial growth phase Good prognosisNodular-deeper invasion-vertical growth-poor
    33. 33.  Breslow method, by which millimeter depths of invasion are measured (depth correlating with prognosis Oral mucosa -anterior labial gingiva, ant. Hard palate. They may be focal or diffuse and mosaic
    34. 34. Staging  Stage 1-primary tumor only  Level 1- melanoma in situ without evidence of invasion/microinvasion +nt  Level 2-invasion upto lamina propria  Level 3- deep skeletal tissue-muscles  Stage 2- metastatic to regional lymph node  Stage 3- distant node metastasisGondivkar et al. Primary malignant melanoma-casereport & review of literature.quitesscence int vol 7; jan2009
    35. 35.  Excision with wide margins CT & MRI-Rule our metastases- submandibular & cervical nodes Immunosuppressive drugs
    36. 36. Drug induced Melanosis Quinoline, hydroxy quinoline,amodiaquine minocycline Site :hard palate (large &localized) or multifocal, throughout the mouth. Oral contraceptives &pregnancy are associated with hyperpigmentation of facial skin- periorbital &perioral region -melasma or chloasma. Flat lesions, nail bed & skin
    37. 37. Physiological pigmentation Asian ,black people, dark skinned Caucasians diffuse melanosis of facial gingiva lingual gingiva& tongue may exhibit multiple, diffuse & reticulated brown macule basilar melanosis, evolves in childhood Multifocal or diffuse pigmentation of recent onset – investigated-endocrinopathic disease.
    38. 38.  Symmetric distribution No gender predliction, any age Does not alter normal architecture Degree of intraoral pigmentation –may not correspond cutaneous coloration No change in intensity
    39. 39. Café au Lait Pigmentation Color of coffee with cream Small ephelis-like macules to broad diffuse Late childhood and multiple Neurofibromatosis- nodular and diffuse pendulous neurofibromas - skin and (rarely) in the oral cavity Basilar melanosis without melanocyte proliferation McCune Albright syndrome
    40. 40. Smoker’s Melanosis Ant labial gingiva, Buccal mucosa, lateral tongue, palate, floor of mouth No cause-and-effect relationship Melanogenesis is stimulated by tobacco smoke products
    41. 41.  Brown, flat, and irregular, geographic or maplike basilar melanosis with melanin incontinence No premalignant potential Cosmetics
    42. 42. Pigmented Lichen Planus Erosive lichen planus + diffuse melanosis Increased production of melanin by the melanocytes Accumulation of melanin laden macrophages in the superficial connective tissue
    43. 43. Endrocrinopathic Pigmentation Addison’s diseaseGranulomatous infection of cortex/ autoimmune cortical destruction Adrenocortical insufficiency Steroid hormones decrease Feedback loop stimulated Excess secretion of ACTH Hypotension and hypoglycemia,stimulation of MSH
    45. 45.  Skin may appear tanned- bronzing of the skin and patchy melanosis Gingiva, palate, and buccal mucosa – blotchy Increased hormone-dependent melanogenesis-> accumulation of melanin granules Serum steroid & hormone investigation Disappear-therapy for endocrine disease
    46. 46. HIV oral Melanosis Hyper pigmentation of skin ,nails &mucous membrane. Buccal mucosa is frequently affected site gingiva,palate,tongue may also be involved. Etiology remains undetermined Diffuse multifocal macular brown pigmentations
    47. 47. Peutz-Jeghers Syndrome hereditary intestinal polyposis syndrome Benign hamartomatous polyps in the gastrointestinal tract hyperpigmented macules on the lips and oral mucosa Rectal bleeding, abdominal pain
    48. 48. BROWN HEME-ASSOCIATED LESIONS Ecchymosis- large size-Traumatic-lips & face, uncommon buccal mucosa Traumatic event(fellatio,cheek bite,prostho app) Erythrocyte extravasation into the submucosa Bright red macule /swelling if a hematoma forms brown coloration within a few days Hemoglobin is degraded to hemosiderin
    49. 49.  Retrauma- observed for 2 weeks-resolution Hemorrhagic diathesis-ecchymosis as d/d Anticoagulant drugs -cheek or tongue Hereditary coagulopathic disorders & chronic liver failure-prothrombin time and partial thromboplastin time, clotting time- prolonged
    50. 50. Petechia Pinpoint hemorrhage less than 2cm Autoimmune or idiopathic thrombocytopenic purpura (ITP), disorders of platelet aggregation, aspirin toxicity and myelosuppressive chemotherapy
    51. 51.  Oral –soft palate (10-30)-suction Does not blanch on compression Excessive suction of soft palate against the posterior tongue -self inflicted -pruritic palate at theonset of a viral or an allergic pharyngitis “click” their palate t/t-stop activity-regression in 2 weeks Platelet studies
    52. 52. Hemochromatosis bronze diabetes. tetrad of liver cirrhosis Male predliction
    53. 53.  tissue – iron - Prussian blue-elevated serum level Medical referral
    54. 54. Amalgam Tattoo solitary or focal pigmentation macular bluish gray - even black buccal mucosa, gingiva, or palate vicinity of teeth- large amalgam restorations Accidental impregnation-metal flecks- mucosa extraction sockets- healing phase- connective tissue while re-epithelialization
    55. 55.  Radiographs fine brown granular stippling of reticulum fibers, particularly around vessel walls giant cell reaction-uncommon, infiltrate +nt No t/t required, biopsy when distant to tooth
    56. 56. Graphite Tattoo Palate Traumatic implantation from a lead pencil, school children Macular, focal, and gray or black
    57. 57. Hairy Tongue Unknown etiology dorsum ,particular middle &posterior one third. papillae are elongated- appearance of hairs hyperplastic papillae (filiform) become pigmented by colonization of chromogenic bacteria-brown to green colour to black
    58. 58.  tea &coffee-diffuse discolouration t/t- brush the tongue and avoid tea and coffee for a few weeks Recurrence + nt
    59. 59. Pigmented Neuroectodermal tumor of infancy Benign neoplasm-neural crest cells Infants younger than 6 months Maxilla > mandible> skull Non ulcerated, dark pigmented mass Ill defined radiolucency with developing teeth Less metastasis
    60. 60. Heavy-Metal Ingestion Occupational hazard Ingested pigments tend to extravasate from vessels in foci of increased capillary permeability such as inflamed tissues. Free marginal gingiva-gingival cuff, resembling eyeliner-gray-black app Behavioral changes, neurologic disorders, and
    61. 61. Plumbism Lead poisoning-paints,glazes,cooking vessels,batteries,exhaust of automobile Oral manifestation: metallic taste, excessive salivation,dysphagia.Burtonian line is seen when exposure to lead is high &oral hygiene is poor at gingival margin. chelating agent such as edta or penicillamine.
    62. 62. Mercurialism Pink disease, swift disease,acrodynia. Acute or chronic increase in saliva, itching sensation, metallic taste, salivary glands & lymph nodes are swollen. Tongue is enlarged ,painful,ulcerated
    63. 63.  Color: diffuse grayish pigmentation of alveolar mucosa, gums are deeper hue than normal, teeth may exfoliate due to marked periostitis. Management: bed rest ,atropine to lessen saliva flow. Administration of BAL (British anti-lewisite)& dimercaprol
    64. 64. Argyria Exposure to silver compound Cause: local &systemic absorption of silver compound. Oral manifestation: Diffusely distributed through out gingival &mucosal tissue. Management: source of contact be eliminated.
    65. 65.  Gold- Chrysiasis may be induced by parenteral administration of gold salts, usually for the treatment of rheumatoid arthritis
    66. 66. Nevus of Ota Congenital melanosis bulbi Blue hyperpigmentation of face Involves 1 & 2 branches of trigeminal nerve Melanocytes trapped in upper 1/3 of dermis
    67. 67. Melanin = primary pigment producing brown coloration Tyrosine – tyrosinase –melanin- this occurs in the melanosomes of melanocytes Then the melanosomes are transferred from the melanocyte to a group of keratinocytes called the epidermal melanin unit Variations in skin color is related to the number of melanosomes, the degree of melanization, and the distribution of the epidermal melanin unit
    68. 68. Pigmentary Demarcation Lines Can be divided into five categories: Group A- lines along the outer upper arms with variable extension across the chest Group B-lines along the posteromedial aspect of the lower limb Group C-Paired median or paramedian lines on the chest, with midline abdominal extension Group D-medial, over the spine Group E-bilaterally symmetrical, obliquely oriented, hypopigmented macules on the chest
    69. 69. Pigmentary DemarcationLines More than 70% of blacks have one or more lines These are much less common in whites Type B lines often appear for the first time during pregnancy
    70. 70. Normal Pigmentation Normal skin pigmentation is influenced by: -the degree of vascularity -the amount & location of melanin -the presence of carotene -the thickness of the horny layer
    71. 71. Melanin Production The amount produced is dependent on:-genetics-the amount and the wavelengths of ultraviolet light received-the amount of melanocyte-stimulating hormone(MSH) secreted- the effect of melanoccytestimulatingg chemicals like furocoumarins (psoralens)
    72. 72. HemosiderinHyperpigmnetation Pigmentation due to deposits of hemosiderin occurs in:-purpura-hemochromatosis-hemorrhagic diseases-stasis ulcers** difficult to distinguish from postinflammatory dermal melanosis clinically
    73. 73. PostinflammatoryHyperpigmentation Any inflammatory condition can cause either hypopigmentation or hyperpigmentation Also may be a complication of chemical peels, dermabrasion, laser therapy, or liposuction Histologically, there is melanin in the upper dermis and around upper dermal vessels, located primarily in macrophages (melanophages)
    74. 74. Postinflammatoryhyperpigmenation  Postinflammatory hyperpigmentation following resolution of lymphocytoma cutis on the cheek of a black child
    75. 75. IndustrialHyperpigmentation Occurs in coal miners, anthracene workers, pitch workers, etc Pigmentation of the face may occur from the incorporation in cosmetics of derivatives of coal tar, petrolatum, or picric acid, mercury, lead, bismuth, or furocoumarins (psoralens)
    76. 76. Systemic Diseases Syphilis, malaria, pellagra, and diabetes Addison’s disease- diffuse melanosis pronounced in the axillae and palmar creases, and nipples and genitals, and buccal mucosa Diabetes produces diffuse bronzing of the skin ** patients with virilizing adrenal tumors usually develop hyperpigmentation and hypertrichosis
    77. 77. Systemic Diseases Nelson’s syndrome (a  Vitamin B12 deficiency pituitary MSH-producing tumor)  Kwashiorkor Pheochromocytoma  Vitamin A deficiency Hemochromatosis  Primary biliary cirrhosis Amyloidosis (triad= Scurvy hyperpigmentation, Pregnancy pruritis, xanthomas) Menopause Porphyria cutanea tarda
    78. 78. Hemochromatosis Characterized by: Usually are present:  Gray-brown  Cirrhoisis mucocutaneous  Hypogonadism hyperpigmentation  Liver cirrhosis  Diabetes mellitus  hepatomegaly
    79. 79. Hemochromatosis  Skin pigmentaion is  The actual pigmentation usually generalized is caused by increased  But, more pronounced basal-layer melanin on face, extensor aspect  Mucous memebranes are of the forearms, backs of pigmented in up to 20% the hands, and the of patients geniocrural area  Koilonychia is present in  Iron is deposited in the 50% skin  Localized ichthyosis in  Iron is present as 40% granules around blood  Alopecia is common vessels and sweat glands and within macrophages
    80. 80.  Dx:  Elevated plasma iron and Occurs mostly in men in IBP their sixties  High serum ferritin Women who have without an obvious cause genetic should prompt hemochromatosis can investigation for both have full phenotypic hemochromatosis and expression PCT Extremely rare in the  Etiology is either an young inborn error of Neonatal metabolism or excessive hemochromatosis has number of blood been associated with transfusions intrauterine infections ie  AR gene for heredity cytomegalovirus hemochromatosis is Adults with linked to the HLA-A locus hemochromatosis are on chromosome 6p susceptible to Yersinia
    81. 81. Hemochromatosis-tx Phlebotomy until satisfactory iron levels are found Extracorporeal chelation has also been used successfully Associated DM requires medical tx Long-term complications are cirrhosis and then hepatomas
    82. 82. Melasma Brown patches, sharply  Tends to affect the darker- demarcated, typically on complected the malar prominences and  It may also be found on the forehead forearms The three clinical patterns  Occurs at pregnancy and at are: centrofacial, malar, menopause mandibular  It may also be seen in Increased pigment may ovarian disorders and other simultaneously occur endocrine disorders around the nipples and external genitalia  Most frequently 90% of the time seen in women, 10% in men
    83. 83.  Tx- avoid sunlight, and aMelasma complete sun block with broad-spectrum UVA coverage should be used daily Strong association with the  Kligman’s formula use of birth control pills or (Triluma) dilantin  > then 4% hydroquinone Discontinuing the may be needed contraceptives rarely clears  Side effects of this is the pigmentation, and it ochronosis and satellite may last for years after pigmentation discontinuing them.  Jessner’s solution, Melasma of pregnancy glycolic acid peels,azelaic usually clears within a few acid, kojic acid, and months of delivery cystamine and buthionine sulfoximine are other options
    84. 84. Melasma
    85. 85. Melasma
    86. 86. Melasma
    87. 87. Acromelanosis Progressiva AKA acropigmentation  By age 4 or 5 the A progressive pigmentary perineum, extremities, disorder first described in a and areas of the head Japanese infant and neck were involved Characterized by diffuse  Epileptiform seizures black pigmentation on the dorsum of all the fingers occurred and toes  History revealed Pigmentation became consanguinity progressively more widespread and more pigmented
    88. 88. Pigmented Anomalies of theExtremities Acropigmentation of Dohi  Patients develop Found to affect individuals progressive pigmented & from Europe, India, depigmented macules Caribbean  Often mixed in is a First described in Japan in reticulate pattern 12 patients  Many believe this to be a AKA dyschromatosis variation of symmetrica hereditaria or symmetrical acropigmentation of dyschromatosis of the Kitamura extremities
    89. 89. Reticular Pigmented Anomaly  Clinically it looks smoothof the Flexures  Pigmententation is A rare pigmentary adult- reticular; at the onset disorder periphery, discrete, AKA Dowling-Degos brownish black macules disease or dark dot disease surround the partly Should be considered confluent central whenever acanthosis pigmented area nigricans is in the  Typically, axillae, differential & pt is not inframmary folds, and obese and is known not to intercrural folds are have any internal involved malignancy  There are frequently pits, sometimes pigmented , about the mouth
    90. 90. Reticular Pigmented Anomalyof the Flexures It begins age 20 to 30  Many authors believe it is a spectrum of reticulate yrs and progresses acropigmentation of gradually Kitamura Unknown etiology  Another manifestation of this disorder is familial- AD with variable rocacea-like dermatitis penetrance and with warty keratotic expressivity, and plaques on the trunk and limbs delayed onset  There is no treatment
    91. 91. Histology Distinctive elongation, tufting, and deep hyperpigmentation of therete ridges, with protrusion of similar tufts even from the sides of the follicles
    92. 92. Reticulate Acropigmentationof Kitamura AD  One report of a pt with bony abnormalities Characterized by linear consisting of absence of palmar pits and terminal phalanges of the pigmented macules 1-4 second, third, and fourth mm in diameter on the toes volar and dorsal  Some tx success has been aspects of the hands reported using axelaic acid and feet ointment
    93. 93. Dermatopathia PigmentosaReticularis Consists of a triad of  An autosomal generalized reticulate hyperpigmentation, dominant inheritance noncicatricial alopecia, and pattern has been onychodystrophy reported. Other associations: adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and nonscarring blisters on dorsa of hands and feet.
    94. 94. Dermatopathia PigmentosaReticularis
    95. 95. Transient Neonatal PustularMelanosis  Histologically, there are intracorneal or Infants develop 2- subcorneal aggregates of 3mm macules, predominantly pustules, and ruptured neutrophils, but pustules at birth, eosinophils may also be found predominantly  Dermal inflammation is involving the face composed of an Pigmentation may last admixture of neuts and for weeks or months eos after the pustules are  Differential dx: ETN, healed neonatal acne, & acropustulosis of infancy
    96. 96. Transient Pustular NeonatalMelanosis
    97. 97. Transient Neonatal PustularMelanosis
    98. 98. Peutz-Jeghers  Macules may also occur around the mouth, on the central face, backs of the hands, especially the  Characterized by fingers, and on the toes hyperpigmented macules and tops of the feet. on the lips and oral  Associated polyposis mucosa and polyposis of the small intestine involves the small intestine preferencely  Dark brown or black  But, hamartomatous macules appear typically on the lips, especially the polyps of the stomach lower lip, in infancy or and colon may occur childhood  Symptoms of  Similar lesions may hamhartomas of the appear on buccal small intestine may cause mucosa, tongue, gingiva, repeated bouts of and genital mucosa abdominal pain and vomiting, and intussusception
    99. 99. Peutz-Jeghers Syndrome Cosmetic tx of labial  Syndrome is inherited and macules has been transmitted as a simple accomplished with the use mendelian dominant trait of a 694-mm ruby laser  Sporadic noninherited incidence of malignancy cases may occur within the polyps is 2-3%  The gene (STK11) has been Incidence of GI malignancy localized to 19p13.3 is low, but increased  19p13.3 is believed to be a incidence of other kinds of tumor suppressor gene cancer-breast, and gynecologic malignancies in women
    100. 100. Peutz-Jeghers Syndrome  A protein-losing  Cronkhite-Canada enteropathy may syndrome should be develop and is associated considered in dx with the degree of  Characterized by intestinal polyposis melanotic macules on  Onset is after age 30 yrs the fingers and gastrointestinal polyposis  Sporaically occurring,  Also generalized , benign condition uniform darkening of the  Hypogeusia is the skin, extensive alopecia, dominant initial and onychodystrophy symptom  The polys that occur are  Diarrhea and ectodermal usually benign adenomas changes may follow and may involve the  75% of cases have been whole GI tract reported in Japan
    101. 101. Peutz-Jeghers syndrome  Lip lentigenes in an adolescent with Peutz-Jeghers syndrome
    102. 102. P-J syndrome
    103. 103. Pathology
    104. 104. Reihl’s Melanosis Photosensitivity,  Characteristic feature is phototoxic dermatitis spotty light to dark brown pigmentation Begins with pruritis,  Most intense on the erythema, and forehead, malar regions, pigmentation, gradually behind the ears, on the spreads, then becomes sides of the neck, on other stationary sun-exposed areas Melanosis occurs mostly in  Also circumscribed women and develops over telangiectasia and months temporary hyperemia
    105. 105. pathogenesis Sun exposure following  Has been reported in perfume or cream patients with AIDS and A photocontact Sjogren’s syndrome dermatitis  No good treatments One report of a  The cause of the sensitivity positive patch test needs to be determeined results to lemon oil,  Hyperkeratosis and geraniol, and pigmentation disappear hydroxycitronellal spontaneously
    106. 106. Tar Melanosis An occupational  Small, dark, lichenoid, dermatosis occurring follicular papules become among tar handlers after profuse on the extremities, years of exposure namely the forearms Severe, widespread itching  Bullae are sometimes develops, followed by observed reticular pigmentation,  Represents a telangiectases, and a shiny photosensitivity or appearance of the skin phototoxicity induced by There is a tendency for tar hyperhidrosis
    107. 107.  AD inheritance  Histologically- increase inFamilial melanin in the basal cellProgressive patches  Characterized by layer, especially at the tips of the rete ridges of hyperpigmentation,Hyperpigmentation present at birth,  Pigmented granules are increasing in size and scattered diffusely number with age throughout the  Hyperpigmentation epidermal layers appears in the  Differentiated from other conjunctivae and the hyperpigmentations by buccal mucosa over time presence of bizarre,  Eventually large portions sharply marginated of skin and mucous patterns of membranes become hyperpigmented skin involved
    108. 108. Universal AcquiredMelanosis(Carbon Baby) Ruiz-Maldonado  EM showed a negroid reported a case of a pattern in the Mexican child, born melanosomes of the white, who epidermal melanocytes progressively became black and keratinocytes Developed  Melanocytes were not pigmentation of the increased in number palms, soles, mucous membranes
    109. 109. Zebralike Hyperpigmentation Alimurung et al reported  Hyperpigmenation was an unusual pattern of linear and symmetrical, hyperpigmentation in a involving the trunk and black male infant with extremities congenital defects (ASD,  Increased number of dextrocardia, auricualr melanocytes in the bands of hyperpigmentation atresia, deafness. And  Pigmentary anomaly fades growth retardation) with time spontaneously  May be a varient of incontinentia pigmenti
    110. 110. PeriorbitalHyperpigmentation1.) Familial periorbital 2.) Erythema melanosis (AD) dyschromicum Usually involves all four perstans is a rare cause eyelids, may extend to 3.) Familial dark circles around the eyes, involve the eyebrows frequently seen in and cheeks individuals of Mediterranean ancestry
    111. 111. Metallic Discolorations Pigmentation from deposition of fine metallic particles in the skin Metal may be carried to skin from the blood stream or may permeate into it from surface applications
    112. 112. Argyria  Local tx with a silver- containing product may produce argyria  Examples: conjunctivae, Localized or widespread from eye drops; a wound slate-colored pigmentation from sulfadiazine cream, Due to silver in the skin earlobes from silver Most noticeable in parts earings; and from silver exposed to sunlight acupuncture needles  Can also occur from Tissue silver may stimulate melanocytes occupational exposure, usually siversmiths Initially discoloration is  In localized exposures, hardly perceptible, having only a faint blue color, but the appearance may be a slate-gray color develops separated by many years with time from the exposure
    113. 113. Histology Systemic and localized argria have the same features Normal appearing skin under low power Fine black granules in the basement zone of the sweat glands,blood vessel walls, d-e junction, and arrector pili muscles Unstained biopsy section by darkfield illumination demonstrates silver granules outlining basement membrane of the epidermis and the eccrine sweat glands
    114. 114. Bismuth Rarely associated with deposition of metallic particles in gums when used IM or orally Also known as the bismuth line Presence of stomatitis or peridontitis increased the risk Generalized cutaneous discoloration, in addition to oral mucous membrane and conjunctival pigmentation resembling argyria has occurred but has not be reported in the last 50 years
    115. 115. Lead Chronic lead poisoning can produce a “lead hue” with lividity and pallor Deposit of lead in the gums may occur and is known as the “lead line”
    116. 116. Iron In the past, soluble iron compounds were used in the treatment of allergic contact dermatitides In eroded areas iron was sometimes deposited in the skin, like a tattoo Use of Monsel’s solution can produce similar tattooing
    117. 117. Gold  Chrysiasis may be induced by parenteral administration of gold salts, usually for the treatment of rheumatoid arthritis  More commonly recognized in white patients  A mauve, blue, or slate/gray pigmentation develops initially on the eyelids, spreading to the face, dorsal hands, and other areas  Severity is related to the total dose received, rare < a dose of 20 mg/kg of elemental gold  Pigment is accentuated in light-exposed areas, and sun protected areas do not demonstrate gold  Localized chrysiasis has been induced by the Q- switched ruby laser tx in a patient on parental gold therapy
    118. 118. Mercury Mercurial pigmentation in the skin is rare, especially since the use of mercurials has been strictly controlled Most common presentation is subcutaneous nodules that result from accidental implantation of elemental mercury from a thermometer into skin
    119. 119. Canthaxanthin Orange-red pigment canthaxanthin is present in many plants ( notably algae and mushrooms) and in bacteria. Crustaceans, sea trout, and feathers When ingested for the purpose of simulating a tan, its deposition in the panniculus imparts a golden orange hue to the skin Stools become brick red and the plasma orange, and golden deposits appear in the retina
    120. 120. Dye Discoloration Blue hands from accidental dyeing were reported by Albert in 1976 A man’s hands were dyed as a result of warming them in his armpits while wearing a new blue flannel shirt The dye was insoluble in water, but soluble in sweat
    121. 121. Rubeosis A rosy coloration of the face occurring in young people with uncontrolled diabetes mellitus May be associated with xanthochromia to produce a “peaches and cream” complexion
    122. 122. Vitiligo Usually begins in childhood or young adulthood 50% of cases begin before age 20 Prevalence ranges from 0.5% to 1% Females are disproportionately represented among patients seeking medical care, it is not known if it is actually more common in females or simply because they more often bring it to their physicians attention
    123. 123. Clinical Features An acquired pigmentary anomaly of the skin Manifested by depigmented white patches surrounded by a normal or a hyperpigmented border There may be intermediate tan zones or lesions , halfway between the normal skin color and depigmentaton-so-called trichrome vitiligo Hairs in vitiliginous areas usually become white also Rarely, the patches may have a red, inflammatory border Patches are of various sizes and configurations
    124. 124. Types Localized or focal(including segmental) Generalized Universal Acrofacial
    125. 125. Vitiligo Generalized is the most common Involvement is symmetrical Most commonly involving the face, upper chest, dorsal aspects of the hands, axillae, and groin Tendency for skin around orifices to be affected (eyes,nose, mouth, ears, nipples, umbilicus, penis, vulva, anus) Lesions also favor areas of trauma (elbows and knees)
    126. 126. Generalized Vitiligo  Involvement of perineal and inguinal skin  Note the distinct borders
    127. 127. Acral Vitiligo
    128. 128. Symmetric, Acral Vitiligo Left: pre-PUVA treatment Right:same pt shows perifollicular pattern of repigmentation during PUVA therapy
    129. 129. Segmental Vitiligo  Rapidly progressing segmental vitiligo
    130. 130. Segmental Vitiligo  Segmental vitiligo of the eyebrow and eyelashes
    131. 131. Segmental Vitiligo  Segmental vitiligo on the arm , neck, and chest  Note areas of spontaneous follicular repigmentation  Left upper back with partial spontaneous repigmentation
    132. 132. Universal Vitiligo Applies to cases where the entire body surface is depigmented
    133. 133. Focal Vitiligo  May affect one nondermatomal site  Or asymmetrically affect a single dermatome  This form is treatment resistant, has an earlier onset, and is frequently associated with other autoimmune phenomena  It represents 5% of adult vitiligo and 20% of childood vitiligo  Trigeminal area is most commonly affected
    134. 134. Acrofacial Vitiligo Type affecting the distal fingers and the facial orifices
    135. 135. Vitiligo  Local loss of pigment may occur around nevi and melanomas, the so-called halo phenomenon  Vitiligo-like leukoderma occurs in 1% of melanoma patients  In those previously dx with melanoma, it suggests metastatic disease  Paradoxically, patients who develop leukoderma have a better prognosis than patients without it  Halo nevi are more common in patients with vitiligo  Lesions are hypersensitive to UV light and burn easily when exposed to the sun
    136. 136.  Ocular abnormalities are increased in patients with vitiligo  Vitiligo occurs in 13% of Iritis and retinal pts with the autoimmune pigmentary polyendocrinopathy- abnormalities candidiasis-ectodermal 8% of pts with idiopathic dystrophy (APECED) uveitis have vitiligo or  Familial aggregation is poliosis seen- up to 30% of Most frequent vitiligo pts have an associations are with affected relative-it is not other “autoimmune” inherited as AD or AR diseases((IDDM, trait, but has a pernicious anemia, multifactorial genetic Hashimoto’s thyroiditis, basis Graves’ disease, Addison’s disease, and AA)
    137. 137. Childhood Vitiligo Shows an increase in  Poor response to PUVA segmental therapy presentation More frequent autoimmune or endocrine anomalies High incidence of premature graying in females
    138. 138. Vitiligo  Completely depigmented oval ivory white areas with convex hyperpigmentated borders
    139. 139. Vitiligo  Vitiligo with depigmentation of the lips
    140. 140. Henne Induced Vitiligo
    141. 141. Occupational Vitiligo  All the intermediates in Thiols, phenolic the biosynthesis of compounds, catechol, melanin are phenolic derivatives of catechol, compounds, therefore mercaptoamines, and postulated that several quinones produce accumulation of these depigmentation within the melanocyte Seen in pts who work in may damage or kill the rubber garments or wear cell. gloves containing an  Clinical pattern may be antioxidant, monobenzyl similarto vitiligo, but ether of hydroquinone lesions tend to be concentrated in areas of contact with the incriminated substance
    142. 142. Occupational Vitiligo Many phenolic compounds can produce leukoderma, with or without antecedent dermatitis Examples: paratertiary sulfhydryls; monobenzyl ether of hydroquinone One source is phenolic antiseptic detergents used in hospitals Adhesives and glues containing them may be found in shoes, wristbands, and adhesive tape, and rubber products used in brassieres, girdles, panties, or condoms may also be at fault Self-sticking bindis (the cosmetic used by many Indian woman on the forehead) has been reported to induce leukoderma from the adhesive material
    143. 143. Chemical Depigmentation  Chemical depigmentation due to a germicidal detergent  Pts usually improve with discontinuation of the offending agent
    144. 144. Pathogenesis Three possible mechanisms have been proposed as inducing vitiligo are autoimmunity, neurohumoral factors, and autocytotoxicity No mechanism has been conclusively proven
    145. 145. Histology  There is complete loss of melanocytes  Usually there is no inflammatory component
    146. 146. Differential Morphea Lichen sclerosis Pityriasis alba Tinea versicolor tertiary pinta
    147. 147. Treatment  Fair-skinned pts may manage their disease  Spontaneous with sunblock repigmentation occurs in  Sun protection is no more than 15% to 25% mandatory in all pts with of cases vitiligo because of the  Response is slow loss of protection from  PUVA may actually UV radiation in the worsen the appearance depigmented skin initially by pigmenting  Topical steroids may be surrounding skin useful on focal or limited  Cover-up lesions strategies(topical dyes,  Mid to super high- make-up, self-tanning potency steroids are creams) often required on trunk and acral lesions with the strength tapered as the lesions respond
    148. 148.  Systemic steroids lead to temporary Treatment repigmentation, this is usually lost as the steroidal agents are tapered  Trioxsalen, at a dose of up PUVA therapy is the to 20-40mg, is taken a few most common treatment hours before natural sun for generalized vitiligo exposure Topical application of 8-  Risk of phototoxicity is methoxypsoralen at a low,so this can be done at concentration of 0.05% home to 0.01%, followed by  Ocular protection must be UVA exposure worn from the ingestion of Topical PUVA is used for the drug through the whole focal or limited lesions tx day Inadverrtent burns with blistering are frequent during tx
    149. 149.  Most commonly, 8-  Two-three tx’s/week are methoxyporalen is used done Initially tx is  20% of pts total QOD(because of the repigmentation delayed erythema of occurs;30% to 40% have PUVA), increased to QD partial response once dose is defined  Acral, periorificial, and 1hr to 30 mins before segmental lesions UVA exposure , 8- respond less well methoxypsoralen  Darker-skinned pts have 0.5mg/kg is ingested a better response, since Initial UVA dose is 1 or 2 they tolerate higher UV J/cm squared, which is doses gradually increased; 5-  Repigmentation may MOP has an aefficccacy begin after 15-25 equal to that of 8-MOP tx’s;significant and less risk of improvement may take phototoxicity 100-300 tx’s
    150. 150.  If there is no follicular repigmentation after 3-6  Phenylalanine/UVA(PAU months or approx 50 tx’s VA) is much less effective PUVA should be abated than PUVA CI to PUVA:  UVB tx alone with 311- photosensitivity, nm irradiation is porphyria, liver disease, associated with a higher SLE rate of acute Surgical tx’s can be phototoxicity but may be applied to limited lesions successful if all other tx modalities  UVA plus topical steroids have been exhausted is superior to either agent Epidermal grafting, alone, but is successful autologous minigrafts, only 24-36% of the time and transplantation of after 9 months cultured and noncultured melanocytes
    151. 151.  If > 50% of the body surface area is affected by vitiligo, the pt can consider depigmentation This tx is permanent Monobenzone 20% is applied BID for 3-6 months to residual pigmented areas Up to 10 months may be required One in six pts will experience acute dermatitis, usually confined to the still-pigmented areas
    152. 152. Vitiligo  Partial repigmentation of lesions of vitiligo on the leg of a 14-year- old child at the end of the summer of sun exposure
    153. 153. Vitiligo  Partial repigmenation of vitiligo following psorralen-ultraviolet light (PUVA) therapy
    154. 154. Vitiligo  Permanent repigmentation after 2 years of photochemotherapy (tripsoralen followed by sunlight exposure)
    155. 155. Vogt-Koyanagi-HaradaSyndrome Characterized by bilateral uveitis, symmetrical vitiligo, alopecia, white scalp hair, eyelashes and brows(poliosis, and dysacousia(diminished hearing) Occurs in thirties Initial or meningoencephalitic phase occurs with prodromata of fever, malaise, headache, nausea, and vomiting Also may have psychosis, paraplegia, hemiparesis, aphagia, and nuchal rididity Recovery is usually complete
    156. 156. VKHS  Second phase(ophthalmic-auditory stage) is characterized by uveitis, dreased visual acuity, photopobia, and decreased hearing(50%)  The convalescent phase begins 3weeks to 3 months after it begins to improve
    157. 157. Alezzandrini’s Syndrome Extremely rare syndrome characterized by a unilateral degenerative retinits This is followed several months later by ipsilateral vitiligo on the face and ipsilateral poliosis Deafness may also be present
    158. 158. Alezzandrini’s Syndrome
    159. 159. Leukoderma Postinflammatory leukoderma may result from inflammatory dermatoses ie: Pityriasis rosea, psoriasis, herpes zoster, secondary syphilis, and morphea, sarcoidosis, tinea versicolor, mycosis fungoides, scleroderma, and pityriasis lichenoides chronica, and leprosy Other causes: burns, scars, postdermabrasion, and intralesioal steroid injections
    160. 160. Leukoderma  Postinflammatory hypopigmentation in a 4-month-old black child with atopic dermatitis
    161. 161. Leukoderma  Postinflammatory hypopigmentation following resolution of guttate psoriasis
    162. 162. Pityriasis alba  Ill-defined hypopigmented oval patches are generally seen on the face, upper arms, neck, and shoulders of affected persons  It can be differentiated from vitiligo by its fine adherent scale, partial hypopigmentation, and distribution
    163. 163. Pityriasis alba  White, slightly scaly patches with indistinct borders on a child’s cheek
    164. 164. Postinflammatoryhypopigmentation
    165. 165. Albinism A partial or complete congential absence of pigment in the skin, hair, and eyes (oculocutaneous albinism), or the eyes alone (ocular albinism) Cutaneous phenotype of the various forms is broad, but the ocular phenotype is reasonably constant in most forms The ocular phenotype includes decreased visual acuity, nystagmus, pale irides that transilluminate, hypopigmented fundi, hypoplastic foveae, and lack of stereopsis
    166. 166. Albinism  This pt has light skin, yellowish white hair, and a lack of pigmentation in nevi
    167. 167. Oculocutaneous Albinism 1 OCA 1 results from mutations in the tyrosinase gene Affected pts are homozygous for the mutant gene or are compound heterozygotes for different mutations in the tyrosinase gene AR Two forms: 1) OCA 1A & OCA 1B (indistinguishable at birth) OCA 1 is most severe with complete absence of tyrosinase activity and complete absence of melanin in the skin and eyes Visual acuity is decreased to 20/400 OVA 1B tyrosinase activity is reduced but not absent. Pts may show increase in skin,hair, eye color with age and can tan
    168. 168. OCA 1 OCA 1B was originally called “yellow mutant” albinism Temperature sensitive OCA (OCA 1-TS) results from mutations in the tyrosinase gene that produce an enzyme with limited activity < 35 degrees C and no activity below this temp. pts have white hair, skin, andeyes at birth, at puberty dark hair develops in cooler acral areas
    169. 169.  Top:albinism with white hair, pale skin, and translucent irides Bottom:ophthalmoscopi c view of a pt with albinism demonstrates a pale fundus, poor macular development, and prominent choroidal vasculature
    170. 170. Oculocutaneous Albinism 2 Prevalence of 1:15,000 Pts were named “tyrosinase-positive” albinos AR and mutations occur in the P gene P gene codes a membrane transport protein that is present in the melanosome membrane Cutaneous phenotype of OCA 2 pts is broad, ranging from nearly normal pigmentation to virtually no pigmentation Pigmentation increases with age, and visual acuity improves with age Prader-Willi and Angelman syndromes are caused by deletions in the P gene; 1% of pts with these syndromes also have OCA 2
    171. 171. Oculocutaneous Albinism 3 AR-caused by mutations in the tyrosine-related protein 1 (TRP-1), located on chromosome 9 OCA 3 has been described only in black pts and is characterized by light brown hair, light brown skin, blue/brown irrides, nystagmus, and decreased visual activity Brown rather than black melanin is formed
    172. 172. Ocular Albinism There are multiple forms of ocular albinism OA 1 may be present with lighter than expected skin It is X-linked Female carriers have “mud-splattered” fundi Macromelanosomes are found in the skin, so skin bx may be a helpful tool Many cases of AR ocular albinism have been reclassified as OCA 1 or OCA 2
    173. 173. Syndromes Associated withAlbinism Chediak-Higashsi Syndrome Hermansky-Pudlak Syndrome Griscelli Syndrome(partial albinism with immunodeficiency) Elejalde Syndrome Cross-McKusick-Breen Syndrome Cuna Moon Children
    174. 174. Classification ofOculocutaneous Albinism
    175. 175. Selenium Deficiency Selenium deficiency in the setting of total parental nutrition can lead to pseudoalbinism Skin and hair pigmentation return to normal with supplementation
    176. 176. Waardenburg’s Syndrome Four genotypic variants  Pts have features of exist: piebaldism, with white Types 1 & 3 are caused by forelock, mutations in the PAX gene hypopigmentation, on chromosome 2 premature graying, Type 2 is caused by synophrys, congenital deafness, a broad nasal mutations in the MITF root, and ocular changes gene on chromosome 3, including heterochromia and type 4 due to irides mutations in the ENDRB gene on chromosome 13  Apparently, melanoblasts fail to reach the target sites during embryogenesis
    177. 177.  Rare, AD with variablePiebaldism phenotype, presenting at birth  White forelock, patchy absence of skin pigmenation  Depigmented lesions are static and occur on the anterior and posteroir trunk, mid upper arm to wrist, mid-thigh to mid- calf, and shins  A characteristic feature is the presence of hyperpigmented macules within the areas of lack of pigmentation and on normal skin
    178. 178. Piebaldism
    179. 179. Piebaldism  Segmental white patch on the neck with a tuft of white hair present from birth
    180. 180. Piebaldism  White forelock and patch of unpigmented skin in a young girl with piebaldism
    181. 181. Piebladism The white forelock arises from a triangular or diamond-shaped midline white macule on the frontal scalp or forehead The medial portions of the eyebrows, and eyelashes may be white Histologically, melanocytes are completely absent in the white macules Etiology is a mutation in the c-kit protooncogene Phenotypic differences seen in families is caused by different locations of mutations in the gene The white lesions may respond to surgical excision
    182. 182. Idiopathic GuttateHypomelanosis symmetrica progressiva  AKA leukopathica  Very common aquired disorder affecting women more frequently than men  Usually occurs after age 40  Lesions occur on the shins and forearms; are small (6 or 8mm), rarely become very numerous ( a dozen or two at most), and never occur on the face or trunk  Lesions are irregularly shaped and very sharply defined, like depigmented ephelides, and are only of cosmetic significance
    183. 183. Idiopathic GuttateHypomelanosis