sign in sign up
<<
Home , Dermatitis herpetiformis, Edema, Livedo reticularis, Paresthesia, Xanthoma

4/10/2012

LUPUS

 Acute – “butterfly” malar , transient, follows sun exposure  Lesions on hands often spare knuckles DERMATOLOGIC  Evaluate for internal MANIFESTATIONS OF INTERNAL disease (ANA/ENA)  +/- oral ulcers DISEASE  Kim Sanders MPAS, PA-C Nephritis concerning OHSU Department of (usually dsDNA+)

PHOTO CREDITS:

 SCLE – photosensitive,  Lynne Morrison M.D. photodistributed  Bolognia et al. Dermtext.com  Lesions may appear  The internet annular, eczematous, psoriasiform.  Not scarring  15-50% progress to SLE  Med induced: HCTZ, NSAIDs, diltiazem, and terbenafine  Usually anti-Ro +

LUPUS ERYTHEMATOSUS LUPUS

 Multisystem,  Discoid Lupus – face, autoimmune disorder scalp, and ears  Female > male 6:1  Can occur on mucosa  SLE 4 fold  Can scar, also higher in AA females follicular plugging and  SLE criteria –rash scarring alopecia photosensitivity, oral ulcers, ,  Long standing lesions serositis, renal can dvp SCC disorder, neurologic  Only 5-10% dvp SLE disorders, hematologic disorder, +ANA.

1 4/10/2012

LUPUS  More rare types –  ¼ of adult pts have  Tumid – no epidermal change. Dermal assoc malignancy process. Can appear like urticarial plaques on  Females 2x> males face and trunk  can overlap with other  Lupus panniculitis – intense in collagen vascular the fat with indurated and depressed areas. diseases  Lupus pernio – red/dusky/purple on  Poikiloderma, fingers, , elbows, and lower legs. Brought violaceous color on by cold. (elbows, with fine scale)  Photodistribution  Periungal telangiectasia and dilated loops. Ragged cuticles

LUPUS DERMATOMYOSITIS

 Heliotrope sign -  Diagnosis – Clinical, biopsy and DIF violaceous and puffy  Eval for SLE – diffuse non-scarring alopecia, periungal telangiectasia, Raynauds, retic,  Gottrons sign – , , ANA (and ENA), violaceous UA, CBC with diff, platelets, CMP, ESR. poikiloderma over knuckles, elbows, and  Rheumatology referral knees  TX: topical/intralesional steroids, SUN  Gottrons papules – PROTECTION,hydroxychloroquine, , over knuckles methotrexate. With SLE - azathiaprine, cyclosporine, …  Erosions and bullae are a bad sign  Calcinosis cutis

DERMATOMYOSITIS DERMATOMYOSITIS

 Malaise and  Connective -  Muscle soreness/ vascular disease and decreased  Autoimmune strength  Symmetric proximal,  Can be assoc with extensor, interstitial dz inflammatory and cardiac dz myopathy and  Rarely drug-induced characteristic skin  DX: , eruption. ANA, ENA, muscle  Polymyositis – muscle enzymes, MRI, LFT’s, only, spares the skin LDH, 24 hour  Amyopathic – skin creatinine, MI2 only, no muscle  Malignancy eval

2 4/10/2012

DERMATOMYOSITIS  Skin findings:  Diffuse , can have leukoderma except  Characteristics of subtypes: perifollicular (“salt and  Juvenile – Calcinosis cutis is common, usually pepper”) no malignancy  Matted telangiectasias of  Adult – Common malignancies ovarian, breast, face, lips and palms colon, lung, and pancreas  Sclerodactyly  Amyopathic – no myopathy, skin only. Can have late onset myopathy after 15mo-6 years.  Calcinosis cutis – distal 14% have malignancy, 13% have ILD.  Raynaud’s and ulcers on fingers common  Treatment: sunscreen, topical steroids,  Dry skin hydroxycholorquine, methotrexate, azathioprine, IVIG  Pruritus

SYSTEMIC SCLEROSIS (SCLERODERMA) SCLERODERMA

 Collagen vascular dz  Labs: primarily affecting  ANA +, and Scl-70 (diffuse) or anti-centromere females (30-50yo) (limited)  Symmetric induration of the skin with  TX: CCB’s for Raynauds (nifedipine). Avoid cold involvement of distal and tobacco. areas  ACE inhibitors (for renal involvement?)  Systemic involvement  ?Prednisone, ?methotrexate, in trials – esophagus, , , and kidneys  May be fatal (lung involvement)

ERYTHEMA SYSTEMIC SCLEROSIS MULTIFORME

 Strongly assoc. with a preceding infection  Limited vs Diffuse Recurrent cases likely  CREST: Calcinosis HSV. cutis, Raynaud’s  Can also be drug phenomenon, induced, radiation esphogeal dysmotility, induced, and sclerodactyly, and idiopathic telangiectasia  Appears targetoid (matted). with dusky center. Can be bullous. Common on the palms and soles, +/- oral involvement, acral distribution.

3 4/10/2012

ERYTHEMA MULTIFORME URTICARIA

 Minor – self limited, recurrent, usually in  Vascular rxn young adults (spring characterized by the and fall) and lasts 1-4 appearance of wheals, weeks. generally surrounded  Major – more by a red halo or flare extensive w/ mucosal  Severe itching, involvement. stinging, or prickling Previously thought to sensation. be a precursor to SJS.  May be annular or  Treatment is polycyclic. supportive  Individual wheals (prednisone may rarely last >12 hours. provoke recurrences).

ERYTHEMA NODOSUM URTICARIA

 Bilateral, symmetric  Acute – resolves in panniculitis with days to weeks, with deep, tender, bruise- complete resolution of like nodules symptoms within 6  Pre-tibial weeks.  Can have malaise, leg  Chronic - >6 weeks. , arthralgias,  Females 2x more fever, HA, affected than males. conjuctivitis, GI More common in complaints adults

ERYTHEMA NODOSUM URTICARIA

 Causes: strep  Triggers: drugs are infection, TB, most common intestinal infection,  Acute >50% idiopathic fungal infection, followed by URI, , IBD, drugs, and foods drugs  Chronic 60% “ordinary”  Tx: identify trigger, (autoimmune, rest, elevation, anti- pseudoallergic, inflammatories infection related, and idiopathic) then physical and vasculitic causes

4 4/10/2012

URTICARIA ACANTHOSIS NIGRICANS

 Type II – familial, present at birth  Physicial urticarias: dermatographism, cold,  Type III – neck, axillae, heat, cholinergic, aquagenic, solar, vibratory, and groin. Occurs in obesity, exercise induced. acromegaly and  +/- allergic rhinitis, asthma, nasal polyps and gigantism, Cushing’s, food induced DM, ,  Food: acute. Addison’s, hyperandrogenic states.  Can do food diary  Drugs: OCP’s,  Infections: URI, strep, Hep B/C, mono , and  Emotional stress, menthol, , pollen, glucocorticoids. and alcohol

URTICARIA ACANTOSIS NIGRACANS

 D/DX: urticarial vasculitis, BP, EM, GA,  Tripe palms – Sarcoidosis, CTCL which would all last >24 hours thickened velvety  TX: antihistamines. Consider Singulair. Cold palms with baths, sarna, phototherapy, nifedipine, dapsone, pronounced gold, methotrexate. dermatoglyphics. 95% occur in pt’s with .  Lung CA most common if only palms affected. Tripe palms + AN = gastric cancer.

ACANTHOSIS NIGRICANS ACANTHOSIS NIGRACANS

 Grey, brown, or black  Treatment – remove malignancy, treat DM, hyperpigmentation weight loss, Oral retinoids, metformin, tretinoin, and velvety textured calcipotriol, urea, sal acid, CO2 laser and pulsed plaques. dye laser may be of benefit.  Symmetric distribution.  Type I – Rare. Assoc with malignancy. Increased extent of involvement.  Increased suspicion >40yo, not obese with rapid onset

5 4/10/2012

LIVEDO RETICULARIS ERUPTIVE

 Netlike mottled or  Small yellowish reticulated pink or orange to red-brown reddish blue papules that appear in discoloration. crops over entire body  Markedly elevated  Exposure to cold can triglycerides. accentuate.  Also seen with DM,  +/- coldness, obesity, pancreatits, numbness, chronic renal failure, . hypothyroidism, , corticosteriods, or systemic retinoids.

LIVEDO RETICULARIS “” PALPEBRARUM

 Most not assoc with systemic disease, but may be  Most common. Soft a manifestation of lupus, dermatomyositis, yellowish orange scleroderma, rheumatic fever, rheumatoid oblong plaques on arthritis, Hep C, parvovirus B19, , eyelids meningococcemia, pneumococcal , TB,  Assoc with other types , breast CA, and on and on…… of xanthoma, but are  emboli can cause. May be uni or typically present bilateral. Usu with , , nodules, without any other ulceration or gangrene. disease  >1/2 pt’s with normal lipid profile

XANTHOMATOSIS PEUTZ-JEGHER SYNDROME

 Cutaneous manifestation of lipidosis in which  AD. Mucocutaneous plasma lipoproteins and free fatty acids are pigmented macules changed quantitatively. and hamartomatous  Names based on clinical morphology polyps of the GI tract.  Can be associated with genetic diseases  Dark brown to black round to oval macules  Morphologies fairly specific for the associated elevated lipid on central face, lips and . Can be seen on hands, feet, tongue, and periumbilical.

6 4/10/2012

PEUTZ-JEGHERS SYNDROME THANKS FOR YOUR ATTENTION!

 Polyps in GI tract usually in jejunum and ileum.  Questions??? +/- , obstruction, hemorrhage, and .  Increased risk for malignancy  Tx: check stool for , colonoscopy, upper endoscopy

DERMATITIS HERPETIFORMIS

 Symmetric distribution favoring elbows, extensor forearms, upper back, buttocks and knees.  Urticarial papules, vesicles and .  INTENSE pruritis, burning or stinging. May only see crusting

DERMATITIS HERPETIFORMIS

 Cutaneous manifestation of celiac disease  Only about 20% have intestinal symptoms  Males > females 2:1  Uncommon in AA and Asian populations  Can be associated with Hashimoto’s thyroiditis  Dx: biopsy and DIF (perilesional), anti- endomysial antibioties, tissue  TX: free diet!!! Dapsone, topical steriods for itching. GI eval?? Probably

7