White Scale Sign for Xeroderma

Letters

Sophia Akhiyat, BS Table. Odds Ratios of Preference for a Quick Response Given Online Portal Experience, Prior Skin Biopsy, Misty G. Eleryan, MD Personal/Family History of Melanoma, and Sex Serena Durrani, BA Charles B. Mitchell, MD Characteristic Odds Ratio (95% CI) P Value Online portal experience 3.767 (1.846-7.687) <.001 Author Affiliations: The George Washington University School of Medicine and Prior skin biopsy 0.297 (0.115-0.768) .01 Health Sciences, Washington, DC (Akhiyat, Mitchell); The George Washington Personal/family history 1.043 (0.500-2.176) .91 Medical Faculty Associates, Washington, DC (Eleryan, Mitchell); Johns Hopkins of melanoma University, Baltimore, Maryland (Durrani). Female 1.011 (0.502-2.037) .98 Corresponding Author: Sophia Akhiyat, BS, The George Washington University School of Medicine and Health Sciences, 2300 I St NW, Washington, DC 20052 Results | The survey yielded an estimated 85% response rate, ([email protected]). with 204 patients out of an estimated 240 patients agreeing Accepted for Publication: September 22, 2016. to participate. The population was 56.37% male and 43.63% Published Online: December 7, 2016. doi:10.1001/jamadermatol.2016.4349 female with no statistically significant difference, and Author Contributions: Dr Mitchell and Ms Akhiyat had full access to all of the 62.25% of participants had a college degree or higher level data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. of education. Concept and design: Akhiyat, Mitchell. The highest ranked notification preferences were the on- Acquisition, analysis, or interpretation of data: All Authors. line portal (59.40%) and telephone call (48.95%). About 54% Drafting of the manuscript: Akhiyat, Eleryan, Mitchell. Critical revision of the manuscript for important intellectual content: All Authors. of patients reported their preference would change depend- Statistical analysis: Eleryan, Durrani. ing on whether biopsy results were normal or abnormal Administrative, technical, or material support: Eleryan, Mitchell. (P < .001). For a normal skin biopsy result, most patients Supervision: Akhiyat, Mitchell. (n = 143) reported a preference for an online portal (55.20%). Conflict of Interest Disclosures: None reported. For abnormal results, most patients (n = 145) indicated a pref- 1. Stewart MA. Effective physician-patient communication and health erence for a telephone call (69.2%). outcomes: a review. CMAJ. 1995;152(9):1423-1433. Among participants with previous online portal experi- 2. Meza JP, Webster DS. Patient preferences for laboratory test results notification. Am J Manag Care. 2000;6(12):1297-1300. ence, 52.05% indicated a preference for online portals. Of the participants who did not have experience using online por- 3. Choudhry A, Hong J, Chong K, et al. Patients’ preferences for biopsy result notification in an era of electronic messaging methods. JAMA Dermatol. 2015; 2 tals, 61.11% indicated a preference for a telephone call; χ tests 151(5):513-521. suggested a correlation between age and online portal experience, as well as age and online portal notification preference (P = .05). White Scale Sign for Xeroderma Most participants ranked depth of information received Xeroderma, also known as xerosis cutis, is a common condi- (44.78%) and amount of time to discuss results (35.82%) as their tion that has become ever more important to diagnose in light most important factors for selecting a notification modality of the number of aging patients. The condition is frequently when receiving abnormal results. Other demographic data did seen among the elderly, but it has also been observed in not significantly influence ranking of preferred factors for being younger patients affected by atopic dermatitis.1 The clinical fea- notified of abnormal results. tures of xeroderma are flaky, dry, and cracked skin areas. Po- Patients who indicated that they preferred a quicker tentially because of its high frequency, xeroderma lacks diag- method of skin biopsy result notification did not differ based nostic criteria and signs. Most clinicians tend to wait to make on history of skin biopsies or skin cancers. However, the odds the diagnosis of xeroderma until they see several skin areas of patients with online portal experience preferring a quicker are flaky, dry, and cracked, and this tendency to delay the di- method was statistically significant (Table). agnosis until the condition is full-blown is unnecessary. Clear- cut, microscopic, early signs are therefore required. We pro- Discussion | Our findings support that online portals are the most pose a dermoscopic sign that, in our experience, invariably preferred method of skin biopsy result disclosure among pa- appears in pathologically dry skin areas. tients, particularly when results are normal. Patients re- The brunt of the pathologic changes in xeroderma is in the ported that amount of information and time to discuss re- stratum corneum and epidermis. Single corneocytes shed from sults were their most important factors for choosing a modality the surface during the physiologic process of renewal are nor- for receiving abnormal results. In addition, the odds of pa- mally invisible. In xeroderma, however, because the normal tients with online portal experience preferring a quicker no- process of shedding and removal of intercellular adhesion is tification method was significant. disturbed,2 whitish scales form. This effect of scale produc- There were study limitations that warrant discussion. tion starts microscopically, usually on the shins, and later We had a small sample size. Our location in a metropolitan spreads to the thighs, proximal extremities, and trunk. The seb- area and large percentage of participants with college orrheic areas of the body are always spared. When the inten- degrees or higher education may not be representative of sity is reached that is clinically obvious, branlike scales are shed other communities in the United States. We also did not col- in large amounts that can form dusty clouds when patients re- lect demographic information regarding participant racial/ move their stockings. This clinically obvious scaling, to- ethnic group. gether with pruritus, is what finally prompts most clinicians

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to recognize xeroderma. Subsequently, cracks and fissures of Figure. White Scales in Xeroderma Detectable by Dry Dermoscopy the stratum corneum can develop, along with asteatotic eczema that causes dull, chronic inflammation. Histologic stud- A Patient 1, clinical photograph B Patient 1, dry dermoscopy ies reveal little alteration of stratum corneum, nor epidermis,3 early on. In later stages, eczematous changes (eczema cra- quelé) develop.

Methods | Overall, we included 11 patients (6 women aged 26 to 82 years and 5 men aged 67 to 87 years) in the study, which we conducted between March 1, 2015, and April 1, 2016. The study was approved by Kantonale Ethikkommission Zürich and written patient informed consent was obtained.

Results | In xerotic areas of any size, stage, and race, white scales

were always detectable in dermoscopy (Figure, A-D). We call C Patient 2, clinical photograph D Patient 2, dry dermoscopy this occurrence the white scale sign (WSS). These scales were large amounts of corneocytes that stuck together and were only visible by dermoscopy on dry skin (Figure, E). On moist skin, however, such as when ethanol was applied for dermoscopy (Figure, F), the white scales disappeared at once. When dry skin was treated with emollients at the locus of a positive WSS, the white scales disappeared 15 minutes later (Figure, G). Thus, the WSS was useful in detecting xeroderma on native, untreated skin. The histopathological features of a biopsy specimen taken in a spot of xeroderma with a positive WSS showed parakeratosis and features of eczema (Figure, H). E Patient 3, dermoscopy without fluid F Patient 3, dermoscopy with 90% ethanol Discussion | We have taken a positive WSS as a cue to discuss and usually prescribe emollients. Many patients are not aware that they are affected by xeroderma. In our experience, the WSS revealed the condition in many cases; clinically, xeroderma would have been missed because of the absence of wide- spread and clinically obvious scaling. Xeroderma occurs not only in old age4,5 but also among younger adults with eating disorders, HIV infection, essential fatty acid deficiency, atopic dermatitis, and many forms of ichthyosis. Scaling without xeroderma is usually temporary and can occur after inflamma- G H tory rashes. As yet, few to no clinical criteria for detecting and Patient 3, treated with an Patient 1, hematoxylin-eosin stain emollient before dermoscopy diagnosing xeroderma exist. We propose that clinicians look for the WSS in patients of all ages. The WSS can contribute to the detection and diagnosis of xeroderma and thus allow suit- able treatment before asteatotic eczema develops.

Amrei Klemmer, MagDr Florian Anzengruber, MD Dmitry Kazakov, MD Alexander A. Navarini, MD, PhD Xeroderma (XD) with features of beginning asteatotic eczema, Fitzpatrick skin type II: A, Clinical view. B, Dermoscopy (original magnification ×10). XD, Fitzpatrick skin type IV: C, Clinical view. D, Dermoscropy (original magnification Author Affiliations: Department of Dermatology, University Hospital of Zurich, ×10). E, Dermoscopy without fluid (original magnification ×10). F, Dermoscopy Zurich, Switzerland. with 90% ethanol. G, Dermoscopy with an emollient applied 15 minutes before. Accepted for Publication: September 22, 2016. H, Dermatopathology showing focal parakeratosis, spongiosis, lymphocytic Corresponding Author: Alexander A. Navarini, MD, PhD, Department of exocytosis, and scarce lymphohistocytic infiltrate in the upper dermis. Dermatology, University Hospital of Zurich, Gloriastrasse 31, 8091 Zurich, Switzerland ([email protected]. Twitter and Instagram: @AlexNavarini). Study concept and design: Anzengruber, Navarini. Published Online: December 14, 2016. doi:10.1001/jamadermatol.2016.4360 Acquisition, analysis, or interpretation of data: All authors. Author Contributions: Ms Klemmer and Dr Anzengruber had full access to all Drafting of the manuscript: Klemmer, Anzengruber, Navarini. the data in the study and take responsibility for the integrity of the data and the Critical revision of the manuscript for important intellectual content: accuracy of the data analysis. Ms Klemmer and Dr Anzengruber shared first Anzengruber, Kazakov, Navarini. authorship. Administrative, technical, or material support: Klemmer, Kazakov, Navarini.

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Conflict of Interest Disclosures: Dr Anzengruber reported receiving funding from the HSM2 (Hochspezialisierte Medizin) and payments from Abbott and Figure 1. Asymptomatic Undermined Oval Ulcer of the Shaft of the Penis Celgene, and serving on the advisory board of LEO Pharma. Dr Navarini reported receiving funding from the Promedica and Bruno-Bloch Foundation. No other disclosures were reported. Funding/Support: Funding for the study was provided by the Department of Dermatology, University Hospital of Zurich. Role of the Funder/Sponsor: The funding source had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. 1. Goeksu Y, Zimmerli LU, Braun RP, et al. Acutely ill patients in internal medicine departments want treatment for undiagnosed, symptomatic skin conditions. Dermatology. 2012;225(2):115-120. 2. Humbert P, Dréno B, Krutmann J, et al. Recommendations for managing cutaneous disorders associated with advancing age. Clin Interv Aging. 2016;11: 141-148. Figure 2. Lesional Histologic Specimen 3. Tezuka T, Qing J, Saheki M, Kusuda S, Takahashi M. Terminal differentiation of facial epidermis of the aged: immunohistochemical studies. Dermatology. 1994; 188(1):21-24. 4. Ghadially R, Brown BE, Sequeira-Martin SM, Feingold KR, Elias PM. The aged epidermal permeability barrier. Structural, functional, and lipid biochemical abnormalities in humans and a senescent murine model. J Clin Invest. 1995;95 (5):2281-2290. 5. Elias PM, Ghadially R. The aged epidermal permeability barrier: basis for functional abnormalities. Clin Geriatr Med. 2002;18(1):103-120, vii.

OBSERVATION

Unusual Presentation of Poppers Dermatitis Cutaneous adverse effects of alkyl nitrites (“poppers”) are under- estimated because the drug is generally used as an inhalant. Pop- pers can cause irritant or allergic contact dermatitis, especially on the face, with exceptional cases in other areas of the body.1-3 We report a unique case of an ulcer of the penis secondary to pop- Full-thickness necrosis of the epidermis is seen with a collection of eosinophils pers toxic effects in a homosexual man, highlighting the impor- and neutrophils associated with a diffuse inflammatory infiltrate of the dermis tance of including exogenous chemical agents in the differential (hematoxylin-eosin, original magnification ×100). diagnosis of genital ulcers in men who have sex with men (MSM). dermatitis. He was asked about his sexual behaviors and about Report of a Case | A homosexual man presented with an ulcer- use of sex toys or drugs. The patient reported using a popper ative lesion on his penis of 2 months’ duration. He had con- called “Jungle Juice” during his sexual encounters, and he could sulted several dermatologists and received different local and not rule out accidental contact between his contaminated systemic treatments including with antibiotics, steroids, and hands and his genitals. Results of patch testing using an In- antimycotic drugs, with no improvement. The man had a stable ternational Contact Dermatitis Research Group standard se- partner and denied both casual and unprotected sex as well ries and a single patch test with alkyl nitrite preparation at 2% as the use of creams or balms on the penis. in water were negative at 48 and 72 hours. A final diagnosis of On examination, the shaft of the penis had an asymptom- irritant (popper) contact dermatitis was made. atic, oval ulcer of more than 3 cm on its long axis with undermined borders and a sanious reddish bottom (Figure 1). The Discussion | Poppers is a generic term for volatile substances be- patient underwent serological antibody screening for syphi- longing to the alkyl nitrite chemical family. The chemical has lis and human immunodeficiency virus (HIV); microbiologi- vasodilatory properties and relaxes smooth muscles. In the cal screening for the presence of common bacteria and yeasts; past, it was used in cardiology practice, but its use was dis- and polymerase chain reaction testing for the presence of continued owing to its toxic effects. Its use is illegal, but to by- Treponema and Chlamydia species, all with negative results. pass legal restrictions, alkyl nitrites are sold as liquid incense, Histologic analysis of the ulcer border showed full epidermal room odorizer, or even leather cleaner. Poppers are widely used necrosis with infiltration of neutrophils and eosinophils. A dif- in the MSM community for their ability to relax the anal sphinc- fuse dermal inflammatory infiltrate rich in polymorphonu- ter and for their psychoactive effects. Hematological, neuro- cleocytes, and focal fibrinoid necrosis of superficial vessels logical, and particularly ocular adverse effects of poppers have were also seen (Figure 2). been increasingly reported.4,5 In the skin, they induce irri- The patient underwent counseling with a psychologist to tant or allergic contact reactions, mainly reported on the face. investigate his personality and a possible diagnosis of artifact Poppers dermatitis in sites other than the face is rare and may

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