Mathurada Kongkabpan, MD. Trichotilomania Impulse control disease. Build up tension before pulling, pleasure/relief after pulling out of hair Characterized by repetitive pulling of hair resulting in alopecia. Age of onset (mean) : 10 13 yrs. PE : nonscarring alopecia, hair broken at different lenghts. Site : scalp, eyelash, eyebrow, pubic hair. (most more than one site) Pharmacologic Rx Monotherapy : SSRI +/- olanzapine, risperidone, pimozide Psychotherapeutic intervention Cognitive-behavioral Rx (important role, effective>clomipramine.) Insight-oriented psychotherapy. Hypnotherapy : particularly in pediatric 7 c- slid e Differential Diagnosis Lichen planus Leukoplakia Candidiasis 8 c- slid e Oral Lichen Planus Idiopathic inflammatory dis. of skin, hair, nails, mucous membranes 40-60% of pt. with LP have oral lesion 7 types: milky-white papules, reticulate p., hypertrophic LP, atrophic LP, erosive/ulcerative LP, bullous LP, desquamative gingivitis 9 c- slid e Oral Lichen Planus Most common: reticular pattern Slightly raised whitish linear lines in lace-like pattern Buccal mucosa, lips,tongue,gingival involvement Asso. chronic liver dis.: HCV Oral ulceration-potential malignant: SCC % 10 c- slid e Oral Lichen Planus Spontaneous remission oral LP-long time Mean duration of oral LP about 5 years Erosive form rarely spontaneous resolves Oral ulceration-potential malignant: SCC 0.5-5% 11 c- slid e Treatment Topical corticosteroid: superpotent Topical immunomodulators eg. tacrolimus Intralesional corticosteroids Multiple well defined erythematous plaques with hemorrhagic crust arrange in linear and bizarre shape on distal of Rt.forearm and dorsum of Rt.hand Dx. Jellyfish dermatitis DDx. 1.Irritant contact dermatitis 2.Allergic contact dermatitis Jellyfish dermatitis Nematocyst or stinging capsules contain toxin Early irritant toxic reaction in all exposed persons,delayed hypersensitivity reaction in a small percentage Acute ; urticaria,angioedema,anaphylaxis Leaving PIH in a streaky flagellate fashion Some ; new lesions appear,severe pruritic lichenoid papules and plaque(linear,zigzag, whip-like pattern) Treatment: -1 st aid =soaking in sea water,vinegar,NOT alcohol -topical potent steroids,antihistamine, systemic steroids is controversial -intralesional steroids are the most effective for delayed type hypersensitivity reaction Multiple well defined,round,edematous violaceous plaque and erythematous rim with some flaccid bullae on top distributed on lower back Dx.Bullous fixed drug eruption DDx. 1. EM 2. SJS 3. Exaggerated arthropod bite reaction Fixed Drug Eruption Commonly found on lips,face,hands,feet,genitalia Develop in 1 to 2 wks. after a first exposure. Subsequent exposures,appear in 24 hrs. A challenge or provocation test with suspect drug may useful in diagnosis The most common ass.c FDE - sulfonamides - NSAIDs - barbiturates - tetracyclines - cabamazepines Three form 1. Solitary: small(5-8mm.),skin-color papule on face esp. nose, upper lip, cheek of adult. 2. Multiple: adolescent, numerous, small papule predilection around nasolabial fold,forehead,chin, preauricular 3. Desmoplastic: a solitary ring-shaped shiny lesion and is usually excised because it can look like a basal cell carcinoma.basal cell carcinoma Trichoepithelioma Desmoplastic trichoepithelioma Associated disease Broooke-Spiegler (epithelioma adenoides cysticum) syndrome: AD trait, development of multiple skin appendage tumors such as cylindromas, trichoepitheliomas, and spiradenomas. Rombos syndrome : atrophoderma,milia, hypotrichosis,BCC,peripheralvasodilation SLE Myasthenia gravis Treatment excision cryotherapy Electrodesiccation CO2 laser 25 c- slid e 26 c- slid e Differential Diagnosis Pyogenic granuloma Hemangioma Kaposis sarcoma Bacillary angiomatosis 27 c- slid e Pyogenic Granuloma Rapidly growing, friable, red papules or polyp of skin or mucosa that freq. ulcerates Most common in children, young adults Etio: unknown Reactive neovasculization : trauma, irritation 28 c- slid e Clinical findinds: Begins as small (5-8 mm) vascular papules Rapidly enlarges over several wks to dull red, dome-shaped, smooth, nontender papules Location : face, neck (more common) but can occur anywhere usu. solitary 29 c- slid e Histology: Lobular capillary hemangioma Exophytic Collaretes of hyperplastic epithelium 30 c- slid e Treatment: Shave excision with curettage, electrodesiccation Surgical excision 31 c- slid e Prognosis: Small risk of recurrence after removal Untreated lesions tend to enlarge slowly, ulcerate and bleed Xeroderma pigmentosum Ddx. - Cockayne syndrome - XP-CS AR Defect in the nucleotide excision repair systems at the level of endonuclease enz. function 7 genetically different complementation groups (A to G) associated with site of impairment in global genomic nucleotide excision repairs Affected gene = XPA-XPG or DNA polymerase era Action spectrum : nm. Clinical Skin freckle : predominated on sun-exposed skin ; all patients - 50 % -> sunburn parchment-like skin - increase risk of CA : BCC,SCC,melanoma Eye - 40% - photophobia, keratitis,corneal opacities - retina sparing - ectopion,endopion,complete loss of lids Neuro most severe form = De Sanctis- Cacchione syndrome ( XP + microcephaly+progressive MR + hyporeflexia,areflexia,+achilles tendon shortening leading to quadriparesis ) Increase internal malignancy times : CA Brain, lung, oral cavity, KUB,breast,pancreasetic, gastric,renal, leukemia management -early diagnosis -sun protection: UV radiation, sun screen, cloth -avoid smoking -cancer : treat as normal - Oral retinoid : prevent new neoplasm 0.5 MKD -eye : UV glasses,methyl cellulose eyedrop Verrucous brown papules coalesce to form well demarcated papillomatous plaques in linear configuration. Epidermal nevus DDx. - Linear psoriasis - Linear LP - Lichen striatus 5 type 1.localized (single) 2.systematized epidermal nevus = diffuse distribution 3.nevus unius lateralis = unilateral 4.Ichthyosis hystrix = 2 extremities linear Trunk -> transverse 5. ILVEN same as localized but inflammation Clinical -Verrucous brown papules coalesce to form well demarcated papillomatous plaques in linear configuration. -linear configurations are common on the limb as is distribution in Blaschkos lines -extensive lesions may be associated with abnormalities in other system (Epidermal nevus syndrome ) Epidermal nevus sybdrome -N= neuro ( MR,seizure) -E=eye (coloboma,cataract) -V=vascular (CVS) -U=urogenital (horse-shoe kidney) -S=skeleton (bone cyst,deformity) Treatment 1.Topical -retinoid -Vit D -diprosalic acid -peeling : chemical, dermabration 2.Surgery -excision -laser, electric surgery Two Question Questions What are associated conditions? Whatre your further investigations? Most likely diagnosis? Sister Mary Josephs nodule from metastatic mucinous cystadenocarcinoma of the pancreas Asscociated conditions neoplastic cell metastatic through lymph ducts and venous routes to umbilical metastasis The most common primary site is the stomach in men and is the ovary in women Men stomach 30%, rectum, colon, small bowel 25% pancreas 18% Lung 3% Prostate 2% Unknow 17% Women ovary 34% endometrium 12% Stomach 9% rectum, colon, small bowel 12% pancreas 8% Cervix 5% Breast 3% Gall bladder 3% Unknow 9% investigations Biopsy : the diagnosis of metastatic carcinoma hinges on histopathologic evaluation of involved skin. Imaging Studies: Use MRI CT The prognosis generally poor with an average survival time of 11 months. prognosis erythrasma Common superficial bacterial infection. Corynebacterium minutissimum (G+Rod) Predilection sites; Toe web spaces, genitocrural, axillary and inframammary regions. Woods lamp; Coral-red fluorescence caused by coproporphyrin III. Whats the name of this abnormality? What is the condition that can be found this abnormality? Apparent Leukonychia White discoloration that fades with pressure Nail plate transparency is maintained 3 different types - Terrys nails : hepatic disorder - Muehrckes nails - Half-and-half nails : renal disorder Muehrckes nails Narrow paired transverse bands parallel to the lunula Abnormality of the nail vascular bed Common in patients receiving systemic chemotherapy and hypoalbuminemia (eg. in nephrotic syndrome, liver disease, malnutrition) What is the most likely diagnosis? Where are the sites of gene mutation? Hereditary hemorrhagic telangiectasia ( Osler Weber Rendu syndrome ) AD. Mutation genes are ENG and ALK-1. Results in decrease level of endoglin,ALK1 Triad of the disease are - telangiectasia - recurrent epitaxis - +ve family history Diagnostic criteria Definite :if 3 criteria are present Possible or suspect :if 2 criteria are present Unlikely :if fewer than 2 criteria 1. Epistaxis : spontaneous, recurrent nose bleeding. 2. Telangiectasia : multiple at characteristic site Lips Oral cavity Fingers nose 3. Visceral lesions e.g. Gastrointestinal telangiectasis bleeding Pulmonary AVM Hepatic AVM Cerebral AVMs Spinal AVM 4. Family history: first degree relative with HHT. Clinical manifestrations. Nasal : recurrent epistaxis ~ 80% (10 yr-old). Skin : macular telangiectasia ~2 mm. Most common at face, lips, nose, tongue, ears, hands, feet and upper body. Pulmonary, CNS, GI tract : AVM The prognosis is good. The prognosis depends on the degree of systemic involvement. 10% of patient die from complications of their disease. Differential diagnosis CREST syndrome Rothmund Thomson Ataxia telangiectasia Rosacea Essential telangiectasia Management Investigations. CBC, platelet count. serum iron. UA. Stool exam, occult blood. CXR, CT/MRI for r/o AVM. Gastroscopy or colonoscopy. Biopsy may helpful. Treatment. Mild case : no treatment is necessary. Symptomatic treatment e.g. E/C or laser surgery for individual lesion. surgical procedures or embolization. Estrogen and/or progesterone. Aminocarproic acid : inhibit fibrinolysis via inhibition of plasminogen activator substances. Counseling. Fin de jour.