Dermatology therapy essentials - part 6 pptx

K K-M syndrome ᭤ Kasabach-Merritt syndrome Kaltostat ᭤ Alginates Kaposi sarcoma ᭤ Kaposi’s sarcoma Kaposi varicelliform eruption ᭤ Eczema herpeticum Kaposi’s dermatosis ᭤ Xeroderma pigmentosum Kaposi’s sarcoma Synonym(s) Kaposi sarcoma ; multiple idiopathic hem- orrhagic sarcoma Definition Neoplasm of endothelial origin, involving the skin, mucosal surfaces, and internal organs Pathogenesis Unclear whether a hyperplastic disease or a true neoplasm; Herpes hominis virus-8 (HHV-8) linked to all subtypes; co-factors: immunosuppression, genetics, country of residence, and male sex Clinical manifestation Classic subtype: usually affects older men of Mediterranean or eastern European Kaposi’s sarcoma. Violaceous papules and plaques on the lower extremity PART11.MIF Page 321 Friday, October 31, 2003 10:45 AM 322 Kaposi’s sarcoma-like granuloma backgrounds; sometimes arises in chroni- cally edematous extremities; violaceous patches, plaques, or nodules on the lower extremities, which can be painful and can ulcerate African endemic subtype: primarily affects boys and men; appears same as classic subtype or in a more deadly form involving bones and lymph system Iatrogenic subtype: seen in kidney and liver transplant patients on immunosuppressive drugs; usually regresses after immunosuppressive drug stopped AIDS-related subtype: lesions often appear on the upper body, including the oral cav- ity, head, neck, back, and in viscera; begin as discrete, red or purple patches that are bilaterally symmetric and initially tend to involve the lower extremities; patches become elevated, evolving into nodules and plaques; sometimes arise as a large infiltrat- ing mass or as multiple, cone-shaped, fria- ble tumors Differential diagnosis Pyogenic granuloma; tufted angioma; melanocytic nevus; melanoma; cavernous hemangioma; angiokeratoma; metastasis; myofibromatoma; arteriovenous malforma- tions Therapy None indicated for indolent skin tumors in elderly patients; localized disease: cryotherapy; radiation therapy; surgical excision or laser ablation; intralesional vinblastine chemotherapy; disseminated disease: vinblastine 3.5–10 mg IV weekly, or chemotherapy combinations, with vinblastine, bleomycin, and doxorubicin; AIDS-associated disease: antiviral therapy References Geraminejad P, Memar O, Aronson I, Rady PL, Hengge U, Tyring SK (2002) Kaposi's sarcoma and other manifestations of human herpesvi- rus 8. Journal of the American Academy of Der- matology 47(5):641–655 Kaposi’s sarcoma-like granuloma ᭤ Granuloma gluteale infantum Kaposi’s varicelliform eruption ᭤ Herpes simplex virus infection Kasabach-Merritt syndrome Synonym(s) K-M syndrome ; consumptive thrombocytopenia ; giant hemangioma syndrome Definition Thrombocytopenia caused by sequestra- tion and destruction of platelets in a large vascular lesion, usually a cavernous hemangioma Pathogenesis Vascular lesion cause platelet trapping and activation, with consumption of coagulation factors Clinical manifestation Presents as a reddish-brown skin plaque or nodule that progresses to a large violaceous mass; petechiae, bruising, and bleeding; high-output cardiac failure; may occur in cavernous hemangioma, Kaposi heman- gioendothelioma, or tufted angioma Differential diagnosis Coagulation abnormality of other cause; angiosarcoma; port-wine stain; congenital hemangiopericytoma; kaposiform heman- PART11.MIF Page 322 Friday, October 31, 2003 10:45 AM Keloid 323 K gioendothelioma of infancy and childhood; teratoma; lymphatic malformation; venous malformation; infantile fibrosarcoma; infantile myofibromatosis; congenital hemangiopericytoma; epithelioid heman- gioendothelioma Therapy Prednisone; interferon; hematologic agents such as epsilon aminocaproic acid, aspirin, and dipyridamole, pentoxifylline, and cryo- precipitate References Hall GW (2001) Kasabach-Merritt syndrome: pathogenesis and management. British Journal of Haematology 112(4):851–862 Kawasaki disease Synonym(s) Mucocutaneous lymph node syndrome ; Kawasaki syndrome; acute febrile mucocutaneous lymph node syndrome Definition Acute systemic vasculitis associated with a febrile illness; skin and mucous membrane involvement Pathogenesis May be caused by a ubiquitous infectious agent in certain genetically predisposed individuals Clinical manifestation Prolonged fever; polymorphous exanthem; swelling and induration of the hands and feet, with subsequent desquamation; non- exudative conjunctival injection; hemor- rhagic, dry, fissured lips; “strawberry tongue”; non-suppurative cervical lymphadenopathy; myocarditis and pancardi- tis; coronary artery abnormalities; arthral- gias and arthritis; urethritis with sterile pyuria; aseptic meningitis; diarrhea, vomiting, abdominal pain; hydrops of the gall- bladder; auditory abnormalities; testicular swelling, pneumonitis Differential diagnosis Viral exanthem; erythema multiforme; scarlet fever; rubeola; staphylococcal scalded skin syndrome; Stevens-Johnson syndrome/toxic epidermal necrolysis; leptospirosis; Rocky Mountain spotted fever; acrodynia; juvenile rheumatoid arthritis; polyarteritis nodosa Therapy Intravenous immunoglobulin (IVIG), 2 g per kg, as a single infusion over 10–12 hours ଙ ; aspirin 80–100 mg per kg per day PO in 4 divided doses until the fever has abated for several days References Rowley AH, Shulman ST (1999) Kawasaki syndrome. Pediatric Clinics of North America 46(2):313–329 Kawasaki syndrome ᭤ Kawasaki disease Kelley-Seegmiller syndrome ᭤ Lesch-Nyhan syndrome Keloid Synonym(s) Cheloid Definition Overgrowth of fibrous tissue that usually develops at the site of a skin injury, where PART11.MIF Page 323 Friday, October 31, 2003 10:45 AM 324 Keratinous cyst the tissue extends beyond borders of the original wound, usually does not regress spontaneously, and tends to recur after excision Pathogenesis Probable genetic factors; imbalance between the anabolic and catabolic phases of healing process; more collagen produced than degraded Clinical manifestation Rubbery or hard, reddish-brown papule or nodule, with regular margins; some with clawlike pseudopods extending beyond the areas of trauma, projecting above the level of the surrounding skin; no spontaneous regression; lesion become less red over many months or years; most common loca- tions: earlobes, face, neck, lower extremities, breast, chest, back, and abdomen Differential diagnosis Hypertrophic scar; squamous cell carcinoma; dermatofibroma; dermatofibrosar- coma protuberans; fibromatosis; North American blastomycosis Therapy Triamcinolone 10–20 mg per ml intralesional; cryotherapy; silicone gel sheet; com- pression dressing; superficial orthovoltage radiation therapy; surgical excision with postoperative interferon or imiquimod References Shaffer JJ, Taylor SC, Cook-Bolden F (2002) Keloi- dal scars: a review with a critical look at thera- peutic options. Journal of the American Academy of Dermatology46(2):S63–97 Keratinous cyst ᭤ Pilar cyst Keratoacanthoma Synonym(s) Self-healing squamous cell carcinoma ; self- healing epithelioma Definition Low-grade malignancy of the piloseba- ceous epithelium, characterized by rapid growth over a few weeks to months, fol- lowed by spontaneous resolution over several months Pathogenesis Possible etiologic factors: sun exposure, trauma, human papilloma virus, genetic factors, and immunosuppression Clinical manifestation Solitary, firm, round, skin-colored or reddish papule rapidly progressing to dome- shaped nodule, with a smooth shiny surface and a central keratinous plug; occurs on sun-exposed areas of face, neck, and dorsum of the upper extremities; spontaneous involution after many months Differential diagnosis Squamous cell carcinoma; basal cell carcinoma; wart; seborrheic keratosis; inverted follicular keratosis; atypical fibroxan- thoma; Merkel cell carcinoma; metastasis; sporotrichosis; coccidioidomycosis; North American blastomycosis; prurigo nodularis Therapy Surgical excision ଙ ; radiation therapy; methotrexate 25 mg per ml intralesional, repeated every 2–3 weeks for up to 5 treatments; fluorouracil 50 mg per ml intralesional, repeated every 2–3 weeks for up to 5 treatments References Schwartz RA (1994) Keratoacanthoma. Journal of the American Academy of Dermatology 30(1):1–19 PART11.MIF Page 324 Friday, October 31, 2003 10:45 AM Keratodermia 325 K Keratoconjunctivitis sicca ᭤ Sjögren syndrome Keratoderma Synonym(s) Keratodermia Definition Skin disorder consisting of a surface that appears horny or scaly References Ratnavel RC, Griffiths WA (1997) The inherited palmoplantar keratodermas. British Journal of Dermatology 137(4):485–490 Keratoderma blennorrhagica Definition Hyperkeratotic and pustular condition of the palms and soles associated with Reiter disease References Shupack JL, Stiller MJ, Haber RS (1991) Psoriasis and Reiter's syndrome. Clinics in Dermatology 9(1):53–58 Keratoderma hereditaria mutilans ᭤ Vohwinkel syndrome Keratoderma palmoplangtaris diffusa with periodontosis ᭤ Papillon-Lefèvre syndrome Keratoderma palmoplantaris striata ᭤ Striate keratoderma Keratoderma palmoplantaris transgradiens ᭤ Mal de Meleda Keratodermia ᭤ Keratoderma Keratoderma. Scaly plaques on the plantar aspects of the feet PART11.MIF Page 325 Friday, October 31, 2003 10:45 AM 326 Keratoelastoidosis Keratoelastoidosis ᭤ Acrokeratoelastoidosis Keratolysis plantaris sulcatum ᭤ Pitted keratolysis Keratolytic winter erythema Synonym(s) Winter erythrokeratolysis ; erythrokeratolysis hiemalis ; Oudtshoorn skin Definition Form of ichthyosis characterized by cycli- cal erythema, hyperkeratosis, and recurrent and intermittent peeling of the palms and soles, particularly during winter Pathogenesis Unknown; autosomal dominant trait Clinical manifestation Palmoplantar erythema with skin scaling; more pronounced in winter months Differential diagnosis Erythrokeratodermia variabilis; progres- sive symmetric erythrokeratodermia; Gir- oux-Barbeau erythrokeratodermia with ataxia; Greither disease; ichthyosis linearis circumflexa; psoriasis; mycosis fungoides; lupus erythematosus; lamellar ichthyosis; gyrate erythema; atopic dermatitis Therapy Emollients References Danielsen AG, Weismann K, Thomsen HK (2001) Erythrokeratolysis hiemalis (keratolytic winter erythema): a case report from Denmark. Jour- nal of the European Academy of Dermatology & Venereology 15(3):255–256 Keratoma plantarum sulcatum ᭤ Pitted keratolysis Keratomycosis nigricans palmaris ᭤ Tinea nigra Keratosis diffusa fetalis ᭤ Ichthyosis fetalis Keratosis follicularis ᭤ Darier disease Keratosis follicularis et parafollicularis serpiginosa ᭤ Elastosis perforans serpiginosa PART11.MIF Page 326 Friday, October 31, 2003 10:45 AM Keratosis pilaris 327 K Keratosis follicularis serpiginosa ᭤ Elastosis perforans serpiginosa Keratosis follicularis spinosa of Unna ᭤ Lichen spinulosus Keratosis follicularis spinulosa ᭤ Lichen spinulosus Keratosis, inverted follicular ᭤ Inverted follicular keratosis Keratosis, lichenoid ᭤ Lichenoid keratosis Keratosis palmaris et plantaris Synonym(s) Palmoplantar keratosis ; palmoplantar keratoderma Definition Heterogeneous group of disorders characterized by scaling and thickening of palms and soles References Ratnavel RC, Griffiths WA (1997) The inherited palmoplantar keratodermas. British Journal of Dermatology 137(4):485–490 Keratosis palmaris et plantaris with carcinoma of the esophagus ᭤ Tylosis Keratosis palmo-plantaris circumscripta ᭤ Tyrosinemia II Keratosis pilaris Synonym(s) Lichen pilaris; keratosis suprafollicularis; pityriasis pilaris Keratosis pilaris. Acuminate, follicular papules on the cheek PART11.MIF Page 327 Friday, October 31, 2003 10:45 AM 328 Keratosis pilaris atrophicans Definition Disorder of follicular keratinization, characterized by follicular keratotic papules Pathogenesis Autosomal dominant trait; arises from excessive accumulation of keratin at the follicular orifice Clinical manifestation Multiple accuminate follicular keratotic papules, sometimes with surounding erythema, most common on lateral arms, thighs and cheeks; association with ichthyosis vulgaris and atopic dermatitis; worse in dry climates and in the winter months; tends to improve with age Differential diagnosis Lichen spinulosus; folliculitis; milia; phryn- oderma; ichthyosis; pityriasis rubra pilaris; Darier disease; lichen planus Therapy Emollients; tretinoin; alpha hydroxy acids; corticosteroids, topical, medium potency References Lateef A, Schwartz RA (1999) Keratosis pilaris. Cutis 63(4):205–207 Keratosis pilaris atrophicans Synonym(s) Ulerythema ophryogenes; keratosis pilaris rubra atrophicans faciei; keratosis pilaris atrophicans faciei; folliculitis ulerythema reticulata; honeycomb atrophy; atrophoderma vermiculatum; ulerythema acnei- forme; atrophoderma reticulatum Definition Group of clinically related disorders characterized by follicular keratotic papules, vari- able perifollicular inflammation, and end- stage atrophy Pathogenesis Unknown; hereditary component Clinical manifestation Keratosis pilaris rubra atrophicans faciei (ulerythema ophryogenes) variant: follicular papules with erythematous halo, located over the lateral eyebrows; beginning shortly after birth and diminishing with age Atrophoderma vermiculatum variant: onset between age 5 and 12 years; follicular keratotic papules with surrounding erythema; evolving into atrophic pits in a reticulate honeycomb pattern Differential diagnosis Keratosis pilaris; folliculitis; acne vulgaris; milia; pityriasis rubra pilaris Therapy Keratolytics such as lactic acid 5% cream, urea 10% cream, or salicylic acid 2–5% cream or gel applied twice daily; alpha hydroxy acids; isotretinoin ᭤ Ulerythema ophryogenes References Frosch PJ, Brumage MR, Schuster-Pavlovic C, Bersch A (1988) Atrophoderma vermiculatum. Case reports and review. Journal of the Ameri- can Academy of Dermatology 18(3):538–542 Keratosis pilaris atrophicans faciei folliculitis ulerythema reticulata ᭤ Keratosis pilaris atrophicans Keratosis pilaris rubra atrophicans faciei ᭤ Keratosis pilaris atrophicans ᭤ Ulerythema ophryogenes PART11.MIF Page 328 Friday, October 31, 2003 10:45 AM Ketoconazole 329 K Keratosis rubra congenita ᭤ Lamellar ichthyosis Keratosis rubra figurata ᭤ Erythrokeratodermia variabilis Keratosis supracapitularis ᭤ Knuckle pads Keratosis suprafollicularis ᭤ Keratosis pilaris Keratosis-ichthyosis- deafness syndrome ᭤ KID syndrome Ketoconazole Trade name(s) Nizoral Generic available Ye s Drug class Azole antifungal agent Mechanism of action Inhibition of fungal cell membrane ergos- terol synthesis Dosage form 200 mg tablet Dermatologic indications and dosage See table Common side effects Dermatologic: skin eruption, pruritus Gastrointestinal: nausea and vomiting, diarrhea, abdominal pain Neurologic: somnolence, dizziness, leth- argy, headache, nervousness Laboratory: elevated liver enzymes Serious side effects Dermatologic: anaphylaxis Gastrointestinal: hepatic failure Ketoconazole. Dermatologic indications and dosage Disease Adult dosage Child dosage Eumycetoma 400 mg PO daily for months to years Not established Histoplasmosis 200–400 mg PO daily for 6–12 months 5–10 mg per kg PO daily for 6–12 months North American blastomycosis 400–800 mg PO daily for a minimum of 6 months 5–7 mg per kg PO daily for 6 months Protothecosis 200–400 mg PO daily for 2–6 weeks Not established South American blastomycosis 200–400 mg PO dailyfor 6–12 months 5–10 mg per kg PO daily for 6–12 months Tinea versicolor 400 mg PO for 1 dose; repeat in 7 days 6.6 mg per kg PO for 1 dose; repeat in 7 days PART11.MIF Page 329 Friday, October 31, 2003 10:45 AM 330 KID syndrome Endocrine: adrenal insufficiency Laboratory: leukopenia, hemolytic anemia Drug interactions Amiodarone; amitriptyline; antacids; bar- biturates; buspirone; carbamazepine; cyclosporine; digoxin; glyburide/met- formin; H-2 blockers; protease inhibitors; phenytoin; pimozide; quinidine; rifampin; statins; sulfonylureas; tacrolimus; theophyl- line; vinca alkaloids; warfarin Contraindications/precautions Hypersensitivity to drug class or component; achlorhydria; fungal meningitis; cau- tion in patients with hepatic insufficiency or with use of other potentially hepatotoxic medications References Rheney CC, Saddler CM (1998) Oral ketoconazole in cutaneous fungal infections. Annals of Phar- macotherapy 32(6):709–711 KID syndrome Synonym(s) Keratosis-ichthyosis-deafness syndrome Definition Disorder characterized by keratitis, ichthyosis-like keratoderma, and deafness Pathogenesis Unknown Clinical manifestation Vascularizing keratitis, with recurrent cor- neal ulcerations; congenital erythrokerato- derma; reticulated hyperkeratosis of the palms and soles; sensorineural deafness; may develop chronic infections, scarring alopecia, squamous cell carcinoma, and neuromuscular disease Differential diagnosis Congenital ichthyosiform erythroderma; lamellar ichthyosis; epidermolytic hyperkeratosis; Netherton’s syndrome Therapy Emollients; cyclosporine 2% ophthalmic solution for keratitis References Langer K, Konrad K, Wolff K (1990) Keratitis, ichthyosis and deafness (KID)-syndrome: report of three cases and a review of the literature. British Journal of Dermatology 122(5):689–697 Kikuchi’s disease ᭤ Kikuchi’s syndrome Kikuchi’s syndrome Synonym(s) Kikuchi’s disease; Fujimoto’s disease; Kikuchi-Fujimoto disease; histiocytic necrotizing lymphadenitis Definition Benign, self-limited disorder characterized by fever, chills, weight loss, and lymphadenopathy Pathogenesis Possibly hypersensitivity reaction to infectious agent Clinical manifestation Painless lymphadenopathy; mainly of the cervical lymph nodes; constitutional signs and symptoms: fever, chills, sore throat, myalgias; skin lesions including red plaques, facial erythema, crusted papules and nodules, ulcerated papules; spontaneous resolution in 1–4 months, with recur- rences PART11.MIF Page 330 Friday, October 31, 2003 10:45 AM [...]... tattoo; contact mucositis; drug-induced or chemicalinduced hyperpigmentation; traumatic melanonychia of the toenails; Peutz-Jeghers syndrome; physiologic melanoplakia and melanonychia Therapy Frequency-doubled Q-switched Nd:YAG laser, or HGM K1 krypton laser, or 532-nm diode-pumped vanadate laser References Veraldi S, Cavicchini S, Benelli C, Gasparini G (1001) Laugier-Hunziker syndrome: a clinical,... disease Therapy Tretinoin; isotretinoin; acitretin; vitamin A 100,000 units PO daily for 30 days, repeated after a 1-month rest period 335 References Harman M, Aytekin S, Akdeniz S, Derici M (1998) Kyrle's disease in diabetes mellitus and chronic renal failure Journal of the European Academy of Dermatology & Venereology 11(1):87–88 K L L-tryptophan-induced eosinophilia-myalgia syndrome ᭤ Eosinophilia-myalgia... ultrastructural study of four cases and review of the literature Journal of the American Academy of Dermatology 25(4): 63 2 63 6 Launois-Bensaude syndrome ᭤ Benign symmetric lipomatosis Lawrence syndrome ᭤ Berardinelli-Seip syndrome Ledderhose disease ᭤ Plantar fibromatosis Leiomyoma Synonym(s) None Definition Benign soft-tissue neoplasm that arises from smooth muscle Pathogenesis Unknown; three subtypes: Piloleiomyoma:... granulomatosa Therapy No therapy if asymptomatic; brushing of tongue with toothbrush to remove embedded debris References Fisher BK Linzon CD (1997) Scrotal glans penis (glans penis plicatum) associated with scrotal tongue (lingua plicata) International Journal of Dermatology 36( 10): 762 – 763 997 Lipodermoid ᭤ Dermoid cyst Lipoglycoproteinosis ᭤ Lipoid proteinosis Lipogranulomatosis subcutanea ᭤ Rothman-Makai... meningitis; malaria Therapy Mild disease: doxycycline; amoxicillin; erythromycin; severe disease: penicillin G 20–24 million units IV per day, divided into 4 doses for 5–10 daysଙ References Vinetz JM (2001) Leptospirosis Current Opinion in Infectious Diseases 14(5):527–538 Lesch-Nyhan disease ᭤ Lesch-Nyhan syndrome Lesch-Nyhan syndrome Synonym(s) Kelley-Seegmiller syndrome; Lesch-Nyhan disease Definition... upper part of the trunk; stellate-shaped, round or oval, uniformly tan-brown to black macule; slowly increasing in number and in size; lesions sometimes coalesce to form larger patches Ink spot lentigo: reticulated pattern, resembling spot of ink; limited to sun-exposed areas; single ink-spot lentigo among an extensive number of solar lentigines; PUVA lentigo: persistent, pale brown macule appearing 6. .. Differential diagnosis X-linked ichthyosis; congenital ichthyosiform erythroderma; Conradi disease; Netherton syndrome; trichothiodystrophy; erythrodermic psoriasis; generalized seborrheic dermatitis; Rud syndrome; SjögrenLarsson syndrome Therapy Emollients; alpha hydroxy acids; tretinoin; acitretin References Lacour M, Mehta-Nikhar B, Atherton DJ, Harper JI (19 96) An appraisal of acitretin therapy in children... Wiskott-Aldrich syndrome; acrodermatitis enteropathica; Rosai-Dorfman disease; xanthoma disseminatum; candidiasis; listeriosis; herpes simplex virus infection; varicella; infantile acropustulosis; leukemia; lymphoma; myeloma Therapy Localized skin involvement: high potency topical corticosteroids Extensive skin involvement: topical nitrogen mustard; photochemotherapy Multisystem disease: chemotherapy... Differential diagnosis Melanocytic nevus; lentigo maligna; melanoma; ephelides; actinic keratosis; seborrheic keratosis; traumatic tattoo; phytophotodermatitis Therapy Frequency-doubled Q-switched Nd:YAG laser, or HGM K1 krypton laser, or 532-nm diode-pumped vanadate laser; hydroquinone, with or without tretinoin References Schaffer JV, Bolognia JL (2000) The clinical spectrum of pigmented lesions Clinics... Peutz-Jeghers syndrome; nevi-atrial myxoma-myxoid neurofibromata-ephelides (NAME or LAMB) syndrome ᭤ Lentigo LEOPARD syndrome Synonym(s) Cardiocutaneous lentiginosis syndrome; multiple lentigines syndrome; generalized lentiginosis; centrofacial lentiginosis; lentiginosis profusa syndrome; lentiginosis- LEOPARD syndrome Multiple brown macules on the forearm L 344 Leprechaunism Therapy Cosmetically disfiguring . Kitamura Klein-Waardenburg syndrome ᭤ Waardenburg syndrome Klippel-Trenaunay syndrome ᭤ Klippel-Trenaunay-Weber syndrome Klippel-Trenaunay-Weber syndrome Synonym(s) Klippel-Trenaunay syndrome; Angio-osteo- hypertrophy;. infections. Annals of Phar- macotherapy 32 (6) :709–711 KID syndrome Synonym(s) Keratosis-ichthyosis-deafness syndrome Definition Disorder characterized by keratitis, ichthyosis-like keratoderma, and. vinblastine chemotherapy; disseminated disease: vinblastine 3.5–10 mg IV weekly, or chemotherapy combinations, with vinblastine, bleomycin, and doxorubicin; AIDS-associated disease: antiviral therapy