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S Saethre-Chotzen syndrome ᭤ Acrocephalosyndactyly Sailor’s neck ᭤ Actinic elastosis Salivosudoriparous syndrome ᭤ Auriculotemporal syndrome Salmon patch Synonym(s) Stork bite ; angel’s kiss ; nevus simplex ; erythema nuchae Definition Vascular malformation, present in infancy, consisting of ectatic capillaries Pathogenesis Possible persistent fetal circulatory pattern Clinical manifestation Pink-to-red macule or patch on the nape of the neck, glabella, forehead, upper eyelid, or nasolabial region; present at birth in about 40% of cases Differential diagnosis Hemangioma; Sturge-Weber syndrome; child abuse; insect bite reaction Therapy No therapy indicated References Mallory SB (1991) Neonatal skin disorders. Pedi- atric Clinics of North America 38(4):745–761 Salmonellosis Synonym(s) Typhoid fever ; paratyphoid fever Definition Infection caused by bacteria in the genus Salmonella, usually contracted by eating contaminated food PART19.MIF Page 513 Friday, October 31, 2003 12:22 PM 514 San Joaquin Valley fever Pathogenesis Infection caused by salmonellae, gram-neg- ative, rod-shaped bacteria of the family Enterobacteriaceae; most common sources of bacteria: beef, poultry, eggs Clinical manifestation Skin signs: light red papules (rose spots) occurring in crops on trunk during second to fourth week of illness; erythema nodo- sum, Sweet’s syndrome, pustular dermati- tis and generalized erythroderma (ery- thema typhosum) Gastrointestinal signs: loose stool or watery diarrhea; abdominal pain; mild hepat- osplenomegaly Differential diagnosis Viral gastroenteritis; shigellosis; ingestion of preformed toxins (“food poisoning”); campylobacter infection; cryptosporidio- sis; cyclospora infection; escherichia coli infection; listeriosis; vibrio infection Therapy Antibiotics only for patients with severe disease or those at high risk of invasive dis- ease: ciprofloxacin; amoxicillin ᭤ Typhoid fever References Stutman HR (1994) Salmonella, shigella, and campylobacter: common bacterial causes of in- fectious diarrhea. Pediatric Annals 23(10):538– 543 San Joaquin Valley fever ᭤ Coccidioidomycosis Sandworm disease ᭤ Cutaneous larva migrans Sanfilippo syndrome Synonym(s) Mucopolysaccharidosis type III-A ; muco- polysaccharidosis type III-B ; mucopoly- saccharidosis type III-C Definition Inherited metabolic storage disease from a deficiency of either heparan sulfate sulfami- dase, N- acetyl-alpha-D-glucosaminidase, acetyl-CoA alpha-glucosamide N- acetyl- transferase, or N- acetyl-alpha-D-glu- cosamine-6-sulfatase Pathogenesis Autosomal recessive trait; deficiency of either heparan sulfate sulfamidase, or N- acetyl-alpha-D-glucosaminidase, or acetyl- CoA alpha-glucosamide N- acetyltrans- ferase, or N- acetyl-alpha-D-glucosamine-6- sulfatase, resulting in accumulation of mucopolysaccharides in the lysosomes of the cells in the connective tissue Clinical manifestation Onset of symptoms from age 2–6 years; organs most involved: bone, viscera, con- nective tissue, and brain; regression of psy- chomotor development and neurologic signs, including severe mental retardation, hyperactivity, autistic features, and behav- ioral disorders; thickened facial features; coarse hair; hirsutism; genu valgum; short neck; progressive deterioration and death, usually before age 20 years Differential diagnosis Hunter syndrome; Hurler syndrome; Scheie syndrome; Gaucher’s disease; Niemann- Pick disease Therapy None References Yogalingam G, Hopwood JJ (2001) Molecular ge- netics of mucopolysaccharidosis type IIIA and PART19.MIF Page 514 Friday, October 31, 2003 12:22 PM Say syndrome 515 S IIIB: Diagnostic, clinical, and biological impli- cations. Human Mutation 18(4):264–281 Sao Paulo fever ᭤ Rocky Mountain spotted fever Sarcoidosis Synonym(s) Angiolupoid sarcoid ; Besnier-Boeck-Schau- mann disease ; Boeck's sarcoid Definition Chronic multisystem disease, characterized by noncaseating epithelioid granulomas Pathogenesis May result from exposure of a genetically susceptible host to specific environmental agents, such as infectious organisms, alu- minium, zirconium, talc, pine tree pollen, and clay, that the immune system is unable to effectively clear Clinical manifestation Skin: asymptomatic, red-brown macules and papules commonly involving the face, periorbital, nasolabial folds, extensor sur- faces of extremities; round-to-oval, red- brown-to-purple, infiltrated plaques, the center of which may be atrophic; non- tender, firm, oval, flesh-colored or viola- ceous nodules on extremities or trunk (Darier-Roussy sarcoidosis); inflitration of scars Pulmonary system: involvement in most patients; dyspnea; dry cough; chest tight- ness or pain Lymphatic system: palpable lymph nodes Ocular involvement: anterior uveitis, asso- ciated with fever and parotid swelling (uve- oparotid fever) Neurologic system: central nervous system involvement sometimes fatal; seventh cra- nial nerve palsy most frequent finding; mis- cellaneous findings: myocardial involve- ment, arthritis, proximal muscle weakness, renal failure Differential diagnosis Tuberculosis; lymphoma; pseudolym- phoma; foreign body granuloma; drug reac- tion; granuloma annulare; granuloma faciale; lichen planus; lupus erythematosus; leprosy; syphilis; psoriasis; tinea corporis; necrobiosis lipoidica Therapy Cutaneous involvement: triamcinolone 3 mg per ml intralesional Severe, recalcitrant disease: methotrexate; azathioprine; hydroxychloroquine Symptomatic systemic disease: pred- nisone ଙ References Vourlekis JS, Sawyer RT, Newman LS (2000) Sar- coidosis: developments in etiology, immunolo- gy, and therapeutics. Advances in Internal Medicine 45:209–257 Sarcoma botryoides ᭤ Rhabdomyosarcoma Savill’s syndrome ᭤ Sulzberger-Garbe syndrome Say syndrome ᭤ Barber-Say syndrome PART19.MIF Page 515 Friday, October 31, 2003 12:22 PM 516 Scabies Scabies Synonym(s) Seven-year itch Definition Contagious infestation of the skin by arach- nid mite Sarcoptes scabiei, var hominis Pathogenesis Causative organism is mite, Sarcoptes sca- biei; disease spreads through direct and prolonged contact between hosts; possible transmission through fomites, such as infected bedding or clothing, but less likely; delayed type IV hypersensitivity reaction to mites, eggs, or scybala (packets of feces) which causes intense pruritus Clinical manifestation Intense pruritus, particularly at night; slightly elevated, pink-white, linear, curved, or s-shaped line (burrow), located in webbed spaces of fingers, flexor surfaces of wrists, elbows, axillae, belt line, feet, and scrotum in men and areolae in women; bur- rows on the palms and soles in infants; vesi- cles; red papules on penile shaft Nodular variant: pink, tan, brown, or red nodules lasting for weeks Crusted (Norwegian) variant: occurs in immunocompromised and institutional- ized patients; minimally pruritic, hyperk- eratotic, crusted plaques over large areas; nail dystrophy; scalp lesions Differential diagnosis Atopic dermatitis; dermatitis herpeti- formis; pityriasis lichenoides; lichen pla- nus; insect bite reaction; contact dermati- tis; psoriasis; ecthyma; impetigo; xerotic eczema; transient acantholytic dermatosis; linear IgA bullous dermatosis; seborrheic dermatitis; erythroderma from other causes such as Sézary syndrome and pem- phigus foliaceus; Langerhans cell histiocy- tosis; fiberglass dermatitis; dyshidrotic eczema; pityriasis rosea; animal scabies; pediculosis; delusions of parasitosis; meta- bolic pruritus Therapy Permethrin; ivermectin; prednisone for severe symptoms References Wendel K, Rompalo A (2002) Scabies and pedicu- losis pubis: an update of treatment regimens and general review. Clinical Infectious Diseases 35(Suppl 2):S146–151 Scalded skin syndrome ᭤ Staphylococcal scalded skin syn- drome Scalp and head syndrome ᭤ Adams-Oliver Syndrome Scalp cyst ᭤ Pilar cyst Scabies. Scaly and fissured papules and plaques in the finger web spaces PART19.MIF Page 516 Friday, October 31, 2003 12:22 PM Schamberg’s progressive pigmented purpura 517 S Scarlatina ᭤ Scarlet fever Scarlet fever Synonym(s) Scarlatina Definition Bacterial infection caused by toxin-produc- ing group-A beta hemolytic streptococci Pathogenesis Eruption caused by erythemogenic toxin as consequence of local production of inflam- matory mediators and alteration of the cutaneous cytokines Clinical manifestation Abrupt onset of fever, headache, vomiting, malaise, chills, and sore throat, with rash appearing after 1–4 days; exudative tonsilli- tis a common site of infection; mucous membranes usually bright red; scattered petechiae and small, red papules on soft palate; during first days of infection, white membrane coating on tongue through which edematous, red papillae protrude (white strawberry tongue); after white membrane sloughs, tongue red with promi- nent papillae (red strawberry tongue); exanthem consisting of fine red, punctate papules, appearing within 1–4 days follow- ing the onset of illness; first appear on upper trunk and axillae and then general- ize, with accentuation in flexural areas; may appear more intense at dependent sites and sites of pressure, such as the buttocks; sand- paper feel to affected skin; transverse areas of hyperpigmentation with petechiae in the axillary, antecubital, and inguinal areas (Pastia lines); flushed face with circumoral pallor; rash fades with fine desquamation after 4–5 days Differential diagnosis Viral exanthem, including rubella, rubeola, fifth disease; toxic shock syndrome; Kawa- saki syndrome; lupus erythematosus; drug reaction Therapy Penicillin VK ଙ ; benzathine penicillin G; penicillin allergy – cephalexin, erythromy- cin References Chiesa C, Pacifico L, Nanni F, Orefici G (1994) Re- current attacks of scarlet fever. Archives of Pediatrics & Adolescent Medicine 148(6):656– 660 Scarring pemphigoid ᭤ Cicatricial pemphigoid Schamberg disease ᭤ Benign pigmented purpura Schamberg’s progressive pigmented purpura ᭤ Benign pigmented purpura Scarlet fever. Bright red tongue with prominent papillae PART19.MIF Page 517 Friday, October 31, 2003 12:22 PM 518 Scheie syndrome Scheie syndrome Synonym(s) Mucopolysaccharidosis type I-H/S ; muco- polysaccharidosis type I-S ; Hurler-Scheie syndrome Definition Inherited metabolic storage disease arising from a deficiency of alpha-L-iduronidase Pathogenesis Autosomal recessive trait; deficiency of alpha-L-iduronidase, which results in accu- mulation of mucopolysaccharides in the lysosomes of the cells in the connective tis- sue Clinical manifestation Onset of symptoms from age 2–4 years; signs and symptoms similar to those of Hurler syndrome, but milder, with slower progression; lichenified, dry, thick skin with diminished elasticity; increased pig- mentation on the dorsum of the hands; sclerodermalike changes; hypertrichosis of the extremities; pale colored hair; mild skeletal deformation and deformity of the hands; growth sometimes normal; aortic stenosis or regurgitation sometimes present; hepatosplenomegaly; intelligence usually normal Differential diagnosis Hurler syndrome; Hunter syndrome; Gau- cher’s disease; Niemann-Pick diseae; osteo- genesis imperfecta Therapy None References Schiro JA, Mallory SB, Demmer L, Dowton SB, Luke MC (1996) Grouped papules in Hurler- Scheie syndrome. Journal of the American Academy of Dermatology 35(5 Pt 2):868–870 Schenck's disease ᭤ Sporotrichosis Schilder-Addison syndrome ᭤ Addison-Schilder disease Schnitzler syndrome Synonym(s) Schnitzler’s syndrome Definition Disorder characterized by chronic urti- caria, in association with recurrent fever, bone pain, and IgM monoclonal gammopa- thy Pathogenesis May be related to deposition of the IgM paraprotein, leading to immune complex deposition and complement activation Clinical manifestation Chronic urticaria; individual episode usu- ally resolves within few hours; fevers per- sist for up to 24–48 hours; relapsing arthralgias; bone pain involving tibia, femur, ileum, and vertebral column; myal- gias; fatigue; weight loss Differential diagnosis Urticarial vasculitis; lupus erythematosus; adult Still disease; Waldenström mac- roglobulinemia; chronic hepatitis B infec- tion Therapy Acute disease flare: prednisone PART19.MIF Page 518 Friday, October 31, 2003 12:22 PM Scleredema adultorum of Buschke 519 S References Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E (2001) The Schnitzler syn- drome. Four new cases and review of the litera- ture. Medicine 80(1):37–44 Schnitzler’s syndrome ᭤ Schnitzler syndrome Schönlein-Henoch purpura ᭤ Henoch-Schönlein purpura Schwannoma ᭤ Granular cell tumor Schweninger and Buzzi, idiopathic anetoderma of ᭤ Anetoderma Scleredema Synonym(s) Scleredema adultorum; scleredema adul- torum of Buschke; scleredema diabeti- corum; scleredema diabeticorum of Bus- chke Definition Disorder characterized by nonpitting, indu- rated plaques and histological evidence of dermal mucin deposition Pathogenesis Increased procollagen synthesis Clinical manifestation Group 1 subtype: precedes febrile illness, particularly upper respiratory tract strepto- coccal infection; usually clears in 6 months to 2 years Group 2 subtype: no prior history of febrile illness; insidious onset of skin lesions; at risk of developing paraproteinemias, including multiple myeloma Group 3 subtype: prior history of diabetes mellitus, usually adult onset and insulin dependent, unremitting course; ill-defined, woody, nonpitting, indurated plaques; ery- thema, hyperpigmentation, and/or a peau d’orange appearance; usually located on face, neck, trunk, or upper extremities Differential diagnosis Scleroderma; lichen myxedema; general- ized myxedema; sclerema neonatorum; amyloidosis; cellulitis; erysipelas Therapy No effective therapy References Tate BJ, Kelly JW, Rotstein H (1996) Scleredema of Buschke: a report of seven cases. Australasian Journal of Dermatology 37(3):139–142 Scleredema adultorum ᭤ Scleredema Scleredema adultorum of Buschke ᭤ Scleredema PART19.MIF Page 519 Friday, October 31, 2003 12:22 PM 520 Scleredema diabeticorum Scleredema diabeticorum ᭤ Scleredema Scleredema diabeticorum of Buschke ᭤ Scleredema Scleredema of Buschke ᭤ Scleredema Sclerema ᭤ Sclerema neonatorum Sclerema neonatorum Synonym(s) Sclerema Definition Disorder of the subcutaneous fat in debili- tated neonates, resulting in generalized sub- cutaneous plaques Pathogenesis Prematurity, hypothermia, shock, and met- abolic abnormalities increases saturated-to- unsaturated fatty acid ratio, possibly as a result of enzymatic alteration, allowing pre- cipitation of fatty acid crystals within lipocytes; occurs with prematurity, pneu- monia, septicemia, respiratory distress syn- drome, congenital heart defects, gastroen- teritis, and intestinal obstruction Clinical manifestation Firm, violaceous subcutaneous plaques appearing suddenly, first on thighs and but- tocks and then spreading; may affect all parts of the body except palms, soles, and genitalia; temperature instability; restricted respiration; difficulty in feeding; decreased spontaneous movement Differential diagnosis Scleredema; scleroderma; subcutaneous fat necrosis of newborn; neonatal cold injury Therapy No specific therapy for skin disorder; insti- tution of therapy specific for the underly- ing disease References Fretzin DF, Arias AM (1987) Sclerema neonato- rum and subcutaneous fat necrosis of the new- born. Pediatric Dermatology 4(2):112–122 Scleroderma Synonym(s) None Definition Group of disorders characterized by skin thickening secondary to increased dermal collagen production ᭤ Progressive systemic sclerosus References Haustein UF (2002) Systemic sclerosis-scleroder- ma. Dermatology Online Journal 8(1):3 Sclerodermoid fasciitis ᭤ Eosinophilia-myalgia syndrome PART19.MIF Page 520 Friday, October 31, 2003 12:22 PM Scrub typhus 521 S Sclerodermoid myalgia ᭤ Eosinophilia-myalgia syndrome Scleroma ᭤ Rhinoscleroma Scleromyxedema ᭤ Papular mucinosis Scleromyxedema-like illness of hemodialysis ᭤ Nephrogenic fibrosing dermopathy Scleromyxedema-like illness of renal disease ᭤ Nephrogenic fibrosing dermopathy Sclerosing angioma ᭤ Dermatofibroma Sclerosing epithelial hamartoma ᭤ Trichoepithelioma Sclerosing hemangioma ᭤ Dermatofibroma Sclerosing periphlebitis of the lateral chest wall ᭤ Mondor’s disease Scrofuloderma ᭤ Cutaneous tuberculosis Scrotal tongue ᭤ Lingua plicata Scrub typhus Synonym(s) Tsutsugamushi disease; tsutsugamushi fever Definition Acute, febrile, infectious illness caused by Rickettsia tsutsugamushi, characterized by rash and systemic signs and symptoms Pathogenesis Caused by Rickettsia tsutsugamushi (Rick- ettsia orientalis), acquired when infected chigger bites and inoculates pathogens Clinical manifestation High, severe headache, myalgia; ocular pain; wet cough; malaise; injected conjunc- tiva; eruption begins as a red, indurated PART19.MIF Page 521 Friday, October 31, 2003 12:22 PM 522 Scurvy papule that eventually enlarges to 8–12 mm, vesiculates, and ruptures, developing necro- sis; 5–8 days later, onset of centrifugal- spreading macular eruption on trunk, sometimes becoming papular Differential diagnosis Tularemia; leptospirosis; typhoid fever; other rickettsial infections; viral exanthem; dengue fever Therapy Doxycycline ଙ ; chloramphenicol – 500 mg PO 4 times daily for 7–14 days References Baxter JD (1996) The typhus group. Clinics in Dermatology 14(3):271–278 Scurvy Synonym(s) Vitamin C deficiency syndrome Definition Vitamin C deficiency disease manifested by gingival lesions, hemorrhage, arthralgia, loss of appetite, and listlessness Pathogenesis Vitamin C deficiency, after at least 3 months of severe or total lack of vitamin C, result- ing in defective collagen synthesis and defective folic acid and iron utilization Clinical manifestation Perifollicular hyperkeratotic papules, sur- rounded by a hemorrhagic halo; hairs are twisted like corkscrews and may be frag- mented; submucosal gingival bleeding; sub- periosteal hemorrhage causes painful bones of the legs and elsewhere; arthralgia; ano- rexia; listlessness; conjunctival hemor- rhage; poor wound healing Differential diagnosis Vasculitis; physical abuse; coagulation abnormalities with leukemia; platelet abnormalities; deep vein thrombosis; thrombophlebitis Therapy Ascorbic acid 800–1000 mg per day PO for at least 1 week, then 400 mg per day until recovery complete References Hirschmann JV, Raugi GJ (1999) Adult scurvy. Journal of the American Academy of Dermatol- ogy 41(6):895–906 Scutula Definition Dense masses of mycelium and epithelial debris forming yellowish, cup-shaped crusts, seen in the favus form of tinea capi- tis References Qianggiang Z, Limo Q, Jiajun W, Li L (2002) Re- port of two cases of tinea infection with scutu- la-like lesions caused by Microsporum gypseum. International Journal of Dermatolo- gy 41(6):372–373 Sea boot foot ᭤ Immersion foot Sea lice ᭤ Seabather’s eruption Seabather’s eruption Synonym(s) Sea lice PART19.MIF Page 522 Friday, October 31, 2003 12:22 PM [...]... syndrome Shaving bumps Siemerling-Creutzfeldt syndrome ᭤ Pseudofolliculitis ᭤ Addison-Schilder barbae Sheep-pox disease Sign of Leser-Trelat ᭤ Orf Definition Abrupt appearance and growth of multiple seborrheic keratoses, caused by an underlying malignancy Shin spots References ᭤ Diabetic Schwartz RA ( 199 6) Sign of Leser-Trelat Journal of the American Academy of Dermatology 35(1):88 95 dermopathy Skeeter's syndrome... Varicose and telangiectatic leg veins 536 Spiegler-Fendt, pseudolymphoma of Spiegler-Fendt, pseudolymphoma of ᭤ Pseudolymphoma Spiegler-Fendt sarcoid ᭤ Pseudolymphoma Spiradenoma Therapy Surgical excisionଙ References Michal M ( 199 6) Spiradenoma associated with apocrine adenoma component Pathology, Research & Practice 192 (11):1135–11 39 Spirillary fever ᭤ Rat-bite fever Spironolactone Synonym(s) Eccrine... Streptococcus pyogenes; superantigen behavior of pyrogenic exotoxin-A (SPE-A); may also produce streptococcal pyrogenic exotoxin-B (SPEB), streptococcal pyrogenic exotoxin-C (SPE-C), streptococcal superantigen and mitogenic factor, as well as non–group-A streptococci aureus; release of tumor necrosis factor-α (TNF-α) and interleukin-1b (IL1b), which mediate signs and symptoms of disease; predisposing... seborrheic blepharitis Therapy Light electrodesiccation; liquid nitrogen cryotherapy; laser ablation; shave removal; isotretinoin for multiple lesions References de Berker DA, Taylor AE, Quinn AG ( 199 6) Sebaceous hyperplasia in organ transplant recipients: shared aspects of hyperplastic and dysplastic processes? Journal of the American Academy of Dermatology 35(5 Pt 1): 696 – 699 Sebocystomatosis ᭤ Steatocystoma... spilus ᭤ Nevus Therapy Destruction by electrodesiccation or laser vaporization spilus Spectacle frame granuloma ᭤ Acanthoma fissuratum Sphingomyelin-cholesterol lipidoses ᭤ Niemann-Pick References Requena L, Sangueza OP ( 199 7) Cutaneous vascular anomalies Part I Hamartomas, malformations, and dilation of preexisting vessels Journal of the American Academy of Dermatology 37(4):523–5 49 S Spider nevus... shock-like syndrome Strawberry patch ᭤ Nevus flammeus Streeter’s dysplasia ᭤ Amniotic band syndrome Streeter's spots ᭤ Aplasia cutis congenita Strep toxic shock syndrome ᭤ Streptococcal toxic shock-like syn- drome Strep toxic shocklike syndrome ᭤ Streptococcal toxic shock-like syn- drome Streptobacillary fever ᭤ Rat-bite fever Streptococcal toxic shocklike syndrome Synonym(s) Strep toxic shock-like... epidermal necrolysis; Henoch-Schönlein purpura; urticaria; viral exanthem; Kawasaki disease; figurate erythema; fixed drug eruption; lupus erythematosus; aphthous stomatitis S 542 Stewart-Bluefarb syndrome Therapy Prednisone Therapy Radical amputation of the limbଙ; radiation therapy References Prendiville J (2002) Stevens-Johnson syndrome and toxic epidermal necrolysis Advances in Dermatology 18:151–173... early positive Nikolsky sign; within 24–48 hours, rash progresses to generalized, superficial blistering eruption, with tissue paper-like sur- Veien NK ( 199 8) The clinician's choice of antibiotics in the treatment of bacterial skin infection British Journal of Dermatology 1 39 Suppl 53:30–36 Staphylococcal toxic shock syndrome ᭤ Toxic shock syndrome Stasis dermatitis Synonym(s) Venous eczema Definition... sequestration in microcirculation, with increased contact of leukocytes with the capillary endothelium and S 540 Steatoblepharon References Weingarten MS (2001) State-of-the-art treatment of chronic venous disease Clinical Infectious Diseases 32(6) :94 9 95 4 Steatoblepharon ᭤ Dermatochalasis Steatocystoma Stasis dermatitis Scaly, crusted, and eroded plaque on the lower extremity release of inflammatory mediators;... carcinoma; drug eruption; lymphoma; leukemia Therapy Trimethoprim and sulfamethoxazoleଙ; ketoconazole; itraconazole; for severe disease: amphotericin B – 0.7–1 mg per kg IV daily for 4–8 weeks, followed by trimethoprim and sulfamethoxazole for 2–3 years References Rivitti EA, Aoki V ( 199 9) Deep fungal infections in tropical countries Clinics in Dermatology 17(2):171– 190 South American pemphigus ᭤ Fogo selvagem . heparan sulfate sulfami- dase, N- acetyl-alpha-D-glucosaminidase, acetyl-CoA alpha-glucosamide N- acetyl- transferase, or N- acetyl-alpha-D-glu- cosamine-6-sulfatase Pathogenesis Autosomal. sulfate sulfamidase, or N- acetyl-alpha-D-glucosaminidase, or acetyl- CoA alpha-glucosamide N- acetyltrans- ferase, or N- acetyl-alpha-D-glucosamine- 6- sulfatase, resulting in accumulation. extensor sur- faces of extremities; round-to-oval, red- brown-to-purple, infiltrated plaques, the center of which may be atrophic; non- tender, firm, oval, flesh-colored or viola- ceous nodules

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