primary systemic amyloidosis presenting with classic cutaneous findings: a case report
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Prevalence of dermatoses and quality of life impairment in internalmedicine inpatients
(Poster reference number 5298)Yasemin Goeksu, MD, Department of Dermatology, University Hospital of Zurich,Zurich, Switzerland; Alexander A. Navarini, MD, PhD, Department ofDermatology, University Hospital of Zurich, Zurich, Switzerland; EdouardBattegay, MD, Division of Internal Medicine, University Hospital of Zurich,Zurich, Switzerland; Lars E. French, MD, Department of Dermatology, UniversityHospital of Zurich, Zurich, Switzerland; Lukas U. Zimmerli, MD, Division ofInternal Medicine, University Hospital of Zurich, Zurich, Switzerland; Ralph P.Braun, MD, Department of Dermatology, University Hospital of Zurich, Zurich,Switzerland; Richard Klaghofer, PhD, Department of Psychiatry andPsychotherapy, Zurich, Switzerland
Background: Inpatients in internal medicine typically experience severe impairmentin their quality of life. Because crucial, life endangering diseases must take priority inmedical management over secondary conditions such as skin care, the latter mightbe neglected to some extent.
Objective: Here we evaluated the prevalence of dermatologic diseases, and globaland skin-specific impairment in quality of life in patients hospitalized for internalmedical diseases and assessed whether the skin-specific pathology was viewed asrelevant problem by doctors and patients.
Methods: During a prospective 3-month single-center study, 200 patients hospital-ized at the division of internal medicine for any reason were examined clinically fordermatoses. The study participants were interviewed concerning impairment inquality of life of their illnesses by the SF-12 questionnaire for general quality of lifeand the Dermatology Life Quality Index to measure impairment of quality of lifecaused by dermatologic disease.
Results: Upon physical examination at admission by the internal medicine resident,no skin changes had been registered in 64% of patients. After the dermatologiccheck-up, inpatients in internal medicine had on average 13 (range, 3-25) derma-tologic diagnoses regardless of their main medical conditions. The skin-dependentquality of life impairment was mild with a mean Dermatology Life Quality Indexscore of 3. The general physical quality of life impairment was severe with a SF-12sum score of 33. Eighty-four percent of patients desired therapy for their skincondition during the hospitalization period, especially for dry skin (76%), warts(69%), seborrheic eczema (67%), and onychorrhexis (53%). For subjectively innoc-uous dermatoses, such as nevi (0%), poikiloderma civatte (0%), lentigo (2.6%),cherry hemangioma (1.5%), teleangiectasia (1.8%), lentigo (2.6%), and seborrheickeratosis (4.8%), less than 5% of all affected patients requested treatment.
Limitations: The study was limited to a single visit and skin examination and did notfollow-up to ascertain diagnosis and treatment.
Conclusion: Patients hospitalized in internal medicine for medical conditions areaffected with dermatologic conditions that are for the greater part left unrecognizedand untreated. Skin health is a part of well-being, and correspondingly, patientsoften desire dermatologic therapy, especially for irritating but easily treatableconditions such as dry skin.
APRIL 20
cial support: None identified.
CommerPrevalence of obstructive sleep apnea in obese patients with psoriasis
(Poster reference number 5479)Robert Bissonnette, MD, Innovaderm Research Inc, Montreal, Quebec, Canada;Catherine Maari, MD, Innovaderm Research Inc, Montreal, Quebec, Canada;Chantal Bolduc, MD, Innovaderm Research Inc, Montreal, Quebec, Canada;Simon Nigen, MD, Innovaderm Research Inc, Montreal, Quebec, Canada
Background: Obstructive sleep apnea (OSA) is a common disorder affecting up to 4%of the North American population. There are little data regarding the prevalence ofOSA in psoriasis patients. The objective of this trial was to study the safety andefficacy of adalimumab in patients with psoriasis and OSA.
Methods: A total of 20 patients with psoriasis and sleep apnea were enrolled in thisstudy. Eligibility requirements included a diagnosis of sleep apnea by polysomno-graphic analysis with at least 15 episodes of apnea/hypopnea per hour of total sleepand chronic plaque psoriasis with a minimum 5% of the body surface affected.
Results: At this time, only baseline data including results of polysomnography areavailable. A total of 22 patients were evaluated by polysomnography. Of thesepatients, 6 had been previously diagnosed with OSA. Of the remaining 14 patientsevaluated, a diagnosis of sleep apnea was made for 12 patients (85.7%) who had atleast 15 episodes of apnea/hypopnea per hour. Mean baseline measurements ofapneas/hypopneas per hour, sleep latency, sleep efficiency and total wake time forall 20 randomized patients were 73.0 6 29.1, 15.6 6 10.0 minutes, 69.2 6 21.7 %,and 119.16 78.0 minutes, respectively. A mean of 73 apnea/hypopnea episodes perhour is very high, as more than 30 per hour is considered severe OSA.
Conclusion: Obstructive sleep apnea is an underdiagnosed comorbidity in psoriaticpatients. Dermatologists should question their patients more often about sleepingproblems, especially those who suffer from obesity. Further studies are needed tobetter define the prevalence of sleep apnea in patients with psoriasis.
cial support: 100% funded by a grant from Abbott Laboratories
Commer .12
Primary systemic amyloidosis presenting with classic cutaneous findings:A case report
(Poster reference number 5465)Joshua Butler, MD, Mayo Clinic Jacksonville, Jacksonville, FL, United States;James Keeling, MD, Mayo Clinic Jacksonville, Jacksonville, FL, United States;Jason Sluzevich, MD, Mayo Clinic Florida, Jacksonville, FL, United States
Background: In primary systemic amyloidosis, deposition of insoluble monoclonalimmunoglobulin light (L) chains or L-chain fragments occurs in various tissuesincluding skin, smooth and striated muscles, connective tissues, blood vessel walls,and peripheral nerves. The amyloid of primary systemic amyloidosis is made byplasma cells in the bone marrow.
Case report: We report a case of 69-year-old white woman referred for anintertriginous rash. She noted easy bruising in skin folds, areas of skin breakdown,and ulceration. Otherwise, the eruption was asymptomatic. Review of systemsrevealed a 1-year history of weakness, numbness in hands and feet, dyspnea onexertion, and chronic diarrhea. Previous SPEP showed amonoclonal gammopathy ofunknown significance, IgA kappa restricted. She presentedwith purpuric patches ofthe eyelids, inframammary creases, and lower abdomen. Discreet purpuric maculesand patches were noted on a background yellow hue. Skin biopsy from left waistlineshowed Congo red positivity around vessels and within the dermis, consistent withamyloidosis. Amyloid subtyping performed by on peptides extracted from Congoredepositive microdissected areas of tissue showed AL kappa immunoglobulin lightchains. Repeat SPEP confirmed a monoclonal IgA kappa proteinemia. Bone marrowshowed an atypical plasmocytosis with 8% to 10% infiltration of bone marrow spaceby plasma cells. Echocardiogram was consistent with amyloid cardiomyopathy. Hermuscle weakness and atrophy indicate extensive neuromuscular involvement andher marked weight loss suggest malabsorption from GI tract involvement. She wasstarted on systemic chemotherapy by our hematology colleagues.
Discussion: Patients with primary systemic amyloidosis have cutaneous findings in30% to 40% of cases. Mucocutaneous involvement can be an early indicator of anunderlying plasma-cell dyscrasia. Petechiae, purpura, and ecchymoses that occurspontaneously or after minor trauma are the most common skin signs. The mostcharacteristic skin lesions consist of papules, nodules, and plaques that are waxy,smooth, and shiny. This case illustrates typical cutaneous manifestations of primarysystemic amyloidosis.
cial support: None identified.
CommerRelapsing polychondritis associated with skin vasculitis: Case report
(Poster reference number 5432)Maria Silvia Sousa, MD, Santa Casa de Belo Horizonte, Belo Horizonte, Brazil; LuizMauricio Costa Almeida, MD, Santa Casa de Belo Horizonte, Belo Horizonte,Brazil; Luiza Laborne Sousa Pinto, Santa Casa de Belo Horizonte, Belo Horizonte,Brazil; Michelle dos Santos Diniz, MD, Santa Casa de Belo Horizonte, BeloHorizonte, Brazil
Background: Relapsing polychondritis (RP) is a systemic disease of unknownetiology that seems to be related to an autoimmune inflammatory process. It ischaracterized by a recurring inflammation with consequent destruction of thecartilage of the nose, auricular pavilions, trachea, larynx, and joints.
Case report: A 33-year-old woman with recurrent episodes of bilateral auricularchondritis preserving lobes, neurosensorial bilateral hearing loss, nasal chondritiswith saddle nose deformity, and scleritis. She presented elevated inflammatorymarkers and leukocytosis. In the beginning she was treated with methylpredniso-lone and cyclophosphamide pulses, with improvement. The patient abandonedtreatment and 6 months ago there was reactivation of the bilateral auricularchondritis and the appearance of skin ulcers indicating vasculitis in the lower limbs.Shewas treated with prednisone andmethotrexate, with partial improvement of thelesions.
Conclusion: The involvement of the skin in RP occurs in 17% to 39% of the cases.Biopsy of lesions of cutaneous vasculitis shows leukocytoclastic vasculitis in 5% to14% of the cases. Corticotherapy is the basis of treatment and methotrexate canspare the corticoid. Most patients evolve with remissions and relapses. The maincauses of death are infection, systemic vasculitis, and malignancy. The worstprognostic factors are saddle-nose deformity, arthritis, laryngotracheal narrowing,vasculitis, and hematuria.
cial support: None identified.
CommerJ AM ACAD DERMATOL AB133