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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 23, Issue 4, 2017

Original

Palmoplantar pustulosis – a cross-sectional analysis in Germany

Background: Palmoplantar pustulosis (PPP) is arecalcitrant chronic inflammatory skin disease. Datarelevant for the medical care of patients with PPP arescarce. Thus, the aim of this work was to investigatethe disease burden, clinical characteristics, andcomorbidity of PPP patients in Germany. Patientsand Methods: PPP patients were examined in a crosssectionalstudy at seven specialized psoriasis centersin Germany. Results: Of the 172 included patients withPPP, 79.1% were female and 69.8% were smokers.In addition, 25.0% suffered from psoriasis vulgaris,28.2% had documented psoriatic arthritis, and 30.2%had a family history of psoriasis. In 77 patients themean Dermatology Life Quality Index (DLQI) was 12.2± 7.7 (mean ± SD). The mean Psoriasis PalmoplantarPustulosis Area and Severity Index (PPPASI) was 12.6 ±8.6. Mean body mass index was above average at 27.1± 5.5. The PPP patients had previously received anaverage of 2.6 ± 2.1 different anti-psoriatic systemicdrugs or UV-therapies. The systemic drugs that hadbeen used most frequently were corticosteroids in40.1% of patients, followed by acitretin (37.8%), andmethotrexate (27.9%). The PPPASI was 13.4 ± 8.9 inpatients without current systemic therapy and 10.4 ±7.9 in patients with systemic therapy. Conclusion: ManyPPP patients had a concomitant diagnosis of psoriasisvulgaris and/or psoriatic arthritis or had a familyhistory of psoriasis. Despite the fact that many of thepatients were using anti-psoriatic therapies, there wasstill a high burden of disease within this PPP cohort.This insufficient control of symptoms demonstratesthe urgent need for new PPP treatments.

The effect of comorbidities on overall mortality in Stevens- Johnson Syndrome: an analysis of the Nationwide Inpatient Sample

Background: Stevens Johnson Syndrome (SJS) is a lifethreateningskin condition with an overall mortalityrate of 5%. Although the causes and pathologyof the disease have been well studied, the factorsthat significantly contribute to mortality remainunclear. Objective: To determine relevant risk factorsthat increase the likelihood of inpatient mortalityafter diagnosis of SJS. Methods: A retrospectivecohort study of the 2010-2011 Healthcare Costsand Utilization Project (HCUP) Nationwide InpatientSample (NIS) database was conducted. This studyincluded 1,811 patients who encountered inpatienthospital stays with a discharge diagnosis of SJS.Results: The primary outcome of our study was inhospitalmortality. We analyzed the prevalence andassociated inpatient mortality of underlying criticalillness in patients with SJS. Three age ranges ofpatients in this study showed significantly increasedrates of inpatient mortality by odds-ratio with a 95%CI: 70-79 years (10.91% mortality, OR=4.57, p=0.001),80-89 years (10.67% mortality, OR=4.48, p=0.001), and90+ years (9.30% mortality, OR=4.22, p=0.028). Twocomorbid conditions showed significant associationwith increased inpatient mortality in SJS by odds-ratiowith a 95% CI: cirrhosis (14.58% mortality, OR=2.79,p=0.028) and metastatic disease (10.62% mortality,OR=1.87, p=0.031). Interpretation: Age (70+ years),cirrhosis, and metastatic disease were identified assignificantly associated with inpatient mortality afterdiagnosis with SJS. These findings enhance currentunderstanding of the pathology of this disease, aswell as help improve clinical management of high-riskpatients to reduce inpatient mortality.

Case Report

Chronic paracoccidioidmycosis in a woman with Crohn Disease

We report a rare case of chronic paracoccidioidomycosis(PCM) in a woman with Crohn disease in the setting oftreatment with azathioprine and mesalazine. Serumtests for antigens to Paracoccidioides brasiliensis,Histoplasma capsulatum, and Aspergillus fumigatuswere negative. An incisional biopsy of an oral lesionwith periodic acid-schiff and Grocott-methenaminesilverstains revealed chronic granulomatousinflammation with multinucleated giant cells withParacoccidioides brasiliensis within the cytoplasm,confirming the diagnosis of PCM.

An unusual presentation of seborrheic keratoses in a man with hereditary hemochromatosis

Hereditary hemochromatosis is an autosomalrecessive disorder that disturbs iron metabolismand results in iron deposition throughout the body.Iron accumulation in various organs may cause awide range of systemic symptoms and cutaneousmanifestations of the disease are particularlystriking. Classically, hereditary hemochromatosis hasbeen termed “bronze diabetes.” Although diffusehyperpigmentation is a well-described feature ofthis disease, other cutaneous symptoms may alsooccur, and a variety of anomalies may be observed.We present a case of long-standing hereditaryhemochromatosis associated with hypopigmentedplaques, which were found to be seborrheic keratoseson histologic examination. The cutaneous findingsin hereditary hemochromatosis are summarized andan unusual case of seborrheic keratosis manifestingas hypopigmented plaques in a man with hereditaryhemochromatosis is described. PubMed was usedto search the following terms: hemochromatosis,hereditary, hyperpigmentation, hypopigmentation,keratosis, melanin, seborrheic, siderosis. Despitethe generalized hyperpigmentation that is usuallyobserved in hereditary hemochromatosis, seborrheickeratosis may present rarely as hypopigmented lesionsin individuals affected by this disease. Therefore,seborrheic keratoses should be considered in thedifferential diagnosis in hemochromatosis patientswho present with uncharacteristic pigmentation

Case Presentation

Pasteurella multocida ecthyma complicated by necrotizing fasciitis

Necrotizing fasciitis is a serious infection of theskin and soft tissues. Pasteurella multocida israrely reported to cause necrotizing fasciitis and isassociated with high mortality. We describe a femalepatient with a past medical history of diabetesmellitus and myeloproliferative disorder presentingwith bullae and erythema of the right forearmsecondary to P. multocida infection after possiblecat bite. Despite adequate antibiotic coverage shedeveloped necrotizing fasciitis diagnosed clinicallyand on diagnostic imaging. Patient was taken to theoperating room emergently and underwent irrigationand debridement with subsequent split-skin graft. Sherecovered well after the surgeries and was dischargeon intravenous antibiotics. At clinic follow-up, herwounds were healing well without any significantnew symptoms.

Infantile myofibromatosis – a clinical and pathological diagnostic challenge

Infantile myofibromatosis is a rare disorder offibroblastic/myofibroblastic proliferation andrepresents the most frequent type of mesenchymaltumor in the neonatal period and primary infancy.Three clinical types have been described: solitary,multicentric, and generalized (with visceralinvolvement). A correct characterization of thehistopathology is essential to diagnose theseneoplasias in early infancy. We present a case ofmulticentric infantile myofibromatosis with regressionover time.

Malignancy-associated Sweet syndrome: acute febrile neutrophilic dermatosis associated with recurrence of metastatic cervical cancer

We present a rare case of acute febrile neutrophilicdermatosis, also known as Sweet syndrome,associated with recurrence of metastatic cervicalcancer. This report highlights similar reports andserves as an important reminder of the relationshipbetween Sweet syndrome and cervical cancer.Increasing awareness of Sweet syndrome assistsclinicians in recognizing characteristic findings andencourages evaluation of patients for new-onsetor recurrent neoplastic disease. Additionally, wediscuss the typical presentation of the syndrome, theproper workup and treatment, and a common pitfallencountered in the diagnosis of Sweet syndrome.

Erythromelalgia involving the face

Erythromelalgia is a rare disorder characterized byburning pain, erythema, and increased temperaturetypically involving the distal extremities. Althoughit can progress to involve the face, erythromelalgiapresenting only on the face is particularly rare. Thisdisorder is often misdiagnosed when it presentson the extremities and is even more likely to bemisdiagnosed when presenting only on the face,delaying appropriate treatment and causingconsiderable frustration for the patient. We report acase of a 26-year-old woman with erythromelalgiathat involved only the face for a number of years andwas treated unsuccessfully as rosacea, seborrheicdermatitis, and contact dermatitis. She subsequentlydeveloped involvement of the ears and hands inthe more typical distribution of erythromelalgia. Wediscuss the differential diagnosis of erythromelalgiainvolving the face and extremities, the proposedpathogenesis and management of the disorder, andthe psychological distress this condition can cause.Even when the correct diagnosis of erythromelalgiais made, treatment is difficult, with no single therapyconsistently effective.

Dermatosis papular acantolítica de la vulva con buena respuesta a tacrólimus tópico

Papular acantholytic dermatosis of the vulva is a rare,chronic disorder and is an entity that remains to be fullyunderstood. It shares clinical and histopathologicaloverlap with Darier disease and Hailey-Hailey disease.We describe a 30-year-old woman with papularacantholytic dermatosis of the vulva. The lesionsconsisted of whitish papules and erosions on the labiamajora. Histologically, there was hyperkeratosis andfocal parakeratosis with acantholytic and dyskeratoticcells. She did not respond completely to topicalsteroids but clinical improvement occurred after theuse topical tacrolimus.

Primary isolated osteoma cutis of the face

We report a healthy, 44-year-old woman presentingwith an at least a 20-year history of hardened papulesin the forehead region, extending to the scalp.The biopsy and histopathologic exam confirmed adiagnosis of osteoma cutis. We review the literaturereview and discuss the classification of the cutaneousossification process presented, along with the resultsof the surgical treatment.

Reconstruction of a large leg defect with a combined hatchet flap technique

The repair of lower leg defects, not amenable toprimary closure, may be challenging, since the skinat this level is scarcely elastic and difficult to mobilize.Local skin flaps are generally utilized only to restoremoderately sized losses, whereas grafting becomesa realistic choice in presence of larger wounds.We present the case of a 70-year-old man with awide defect on his right leg following the surgicalexcision of a squamous cell carcinoma. The woundwas successfully closed with a combination of threehatchet flaps. The various steps of the procedure andthe subsequent outcome are illustrated. Our choiceproved to be an easy, time-sparing, but effectivereconstructive solution for a large skin defectinvolving the leg.

Photo Vignette

Paget disease of the male breast

Paget disease of the breast is an uncommon tumor ofthe nipple-areola complex that usually presents as anerythematous and erosive lesion. We report the caseof a 61-year-old man that presented with a three-yearhistory of an erythematous lesion of the right areola,first treated with topical corticosteroids withoutbenefit. He was then referred to our dermatologydepartment and the clinical suspicion of Paget diseasewas considered. The diagnosis was later confirmed bybiopsy. This case report highlights the importanceof clinical recognition of this entity along with otherdiseases that mimic these skin changes in order toallow earlier diagnosis and proper follow-up.

Letter

Web app based patient education in psoriasis – a randomized controlled trial

Purpose: Patients report wanting more informationabout psoriasis and clear expectations from the onsetof therapy. Dermatologists do not think patientsreceive or internalize adequate information. There isa need for further explanation of treatment regimensto increase knowledge, compliance, and patientsatisfaction. Recent advancements in web technologyhave the potential to improve these psoriasisoutcomes.

Methods: A web based application was created toeducate psoriasis patients using video, graphics,and textual information. An investigator blinded,randomized, controlled study evaluated the website’sefficacy in 50 psoriasis patients at Wake Forest BaptistHealth Dermatology. Patients were randomized intotwo groups: Group 1 received a link to the educationalweb app and a survey following their visit; Group 2received a link to the survey with no educational webapp. The survey assessed patient knowledge, selfreportedadherence to medication, and adequacy ofaddressing concerns. Twenty two patients completedthe study.

Results: Patients in the web app group scored anaverage of 11/14 on the psoriasis knowledge quiz,whereas patients in the control group scored anaverage of 9/14 for an improvement of roughly 18%(p=0.008, n=22).

Conclusion: Web app based education viaDermPatientEd.Com is an efficient way toimprove knowledge, but we did not demonstrateimprovements in self-reported medication adherenceor the ability to address concerns of psoriasis patients.

UV tanning advertisements in national high school newspapers

Many young adults have utilized indoor ultraviolet(UV) tanning, putting them at higher risk fordevelopment of skin cancers. Prior to the increasedregulations on indoor tanning for minors, indoortanning businesses marketed to teens throughmodalities such as advertisements in high schoolnewspapers. The purpose of this study was toquantify tanning advertisements in high schoolnewspapers published across the United Statesbetween August 2014 and July 2015. Online versionsof the newspaper issues were available on issuu.com.Tanning advertisements appeared in 3 of 23 highschool newspapers with advertisements of any kind(13%). Among all newspapers with advertisements,10% were indoor tanning advertisements. Onenewspaper in Colorado contained advertisements ofany kind and had 0 tanning advertisements. A priorstudy of Colorado high schools showed 11 of 23schools (48%) to contain tanning advertisements. Thissuggests that there may be a decrease in indoor UVtanning advertisements owing to increased tanningregulation by state legislatures, national attentionto UV tanning, or a general decrease in high schooltanning advertisements.

An incomplete picture: challenges of partial biopsies in large diameter atypical melanocytic lesions

Large diameter atypical pigmented lesions (LDAPL)can be challenging to diagnose accurately usingpartial biopsies because of pathologic heterogeneity,while at the same time large excisions of these lesionsconfer significant morbidity to patients. Consequently,clinicians are often challenged by the managementof these lesions. In this case, we describe an elderlypatient with a history of multiple basal cell carcinomas,prior melanomas, and a family history of melanomawho presented with an irregularly pigmented brownand dark brown patch on his upper back. This lesionwas evaluated with multiple partial incisional biopsiesfrom the most atypical appearing areas of the lesionidentified on dermoscopy, each showing mild andmoderate atypical melanocytes. However, the patchcontinued to change clinically and eventually thepatient underwent a 5mm wide local excision, whichrevealed severely atypical melanocytic proliferationwith areas consistent with melanoma in situ. Thiscase highlights the need for clinicians to lower theirthreshold for excisional biopsy of LDAPL in high-riskpatients.

Serpiginous hypopigmentation secondary to intra-articular corticosteroid injection

Corticosteroids have been a mainstay of therapy forthe treatment of many inflammatory diseases forwell over 50 years. Cutaneous side effects of localcorticosteroid therapy include striae, thinning of theskin, hypopigmentation, and atrophy, which are wellknowncomplications of this treatment modality.Herein, we present an unusual cutaneous adverseside effect rarely seen in intra-articular corticosteroidinjections.

Bidet nail syndrome: report of a typical case

Worn-down nail syndrome is a nail disordercharacterized by thinning of the distal nail plateinduced by repetitive mechanical trauma. A Frenchvariant, entitled “bidet nail syndrome” was firstdescribed by Baran.

Patient perceptions of text-messages, email, and video in dermatologic surgery patients

Introduction: We developed dermatology patienteducation videos and a post-operative text messageservice that could be accessed universally via webbasedapplications. A secondary outcome of thestudy was to assess patient opinions of text-messages,email, and video in the health care setting which isreported here.

Methods: An investigator-blinded, randomized,controlled intervention was evaluated in 90 nonmelanomaMMS patients at Wake Forest BaptistDermatology. Patients were randomized 1:1:1:1 forexposure to: 1) videos with text messages, 2) videosonly,3) text messages-only, or 4) standard of care.Assessment measures were obtained by the use ofREDCap survey questions during the follow up visit.Results: 1) 67% would like to receive an email withinformation about the procedure beforehand2) 98% of patients reported they would like otherdoctors to use educational videos as a form of patienteducation3) 88% of our patients think it is appropriate forphysicians to communicate to patients via textmessage in certain situations.

Conclusion: Nearly all patients desired physicians touse text-messages and video in their practice and themajority of patients preferred to receive an email withinformation about their procedure beforehand.

Considerations for the use of medical devices in dermatology

This manuscript addresses the significantconsiderations concerning the development anduse of medical devices in dermatology. With therapidly growing demand and booming marketfor medical devices, especially lasers, it is crucialthat dermatologists become familiar with thenuances associated with supporting clinical studies,consumer-driven marketing strategies, and thecomplex relationships that exist between physicians,industry, and consumers. An examination of theserelationships includes an overview of the potentialbiases pertaining to advisory panels and treatingclinicians. The aim of this paper is to serve as anintroduction to the background of medical devicesand to offer dermatologists important informationon what should be considered before recommendingtreatment.