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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 12, 2017

Original

Association of pemphigus and systemic corticosteroid use with comorbid health disorders: A case-control study

Background: Pemphigus is a group of debilitating autoimmune blistering disorders associated with painful blisters of the skin and/or mucous membranes. Identification and management of the comorbiditiesof pemphigus is critically important to minimize morbidity and decrease mortality.

Objective: To identify the comorbid health conditions of pemphigus vulgaris.

Methods: This was a case-control study of 130 cases of pemphigus verified by a clinical and laboratory diagnosis and 390 age and sex-matched controls with complete follow-up at a large metropolitanquaternary care medical center.

Results: Pemphigus vulgaris and its treatments were significantly associated with type 2 diabetes mellitus (adjusted odds ratio [95% confidence interval]: 5.68 [2.93-11.02]), hypertension (2.15 [1.25-

3.71]), osteopenia (10.07 [3.72-27.25]), osteoporosis (4.19 [1.50-11.73]), cataracts (7.00 [1.81-27.07]), insomnia (15.00 [1.75-128.39]), and benign prostatic hyperplasia (6.84 [1.79-26.18]). A history of taking systemic corticosteroids was found in 76% of pemphigus vulgaris patients. There were significant statistical interactions between pemphigus vulgaris and a history of using systemic corticosteroids as predictors of diabetes mellitus type 2, hypertension, osteoporosis, and insomnia.

Conclusions: Safer and more effective systemic treatment options are needed for pemphigus to minimize iatrogenic complications of disease.

Dermatology Medical Education: A Multicenter Survey Study of the Undergraduate Perspective of the Dermatology Clinical Clerkship

Background/Aims: Limited data are available regarding the undergraduate dermatology clinical clerkship curriculum in the United States. Our primaryaim is to assess medical students’ perspectives of the dermatology clinical clerkship.

Methods: A multicenter survey study was conducted, which included four California dermatology academic programs. A 17-item questionnaire was designed to investigate medical student perception with regard tothe overall educational value of the various teaching aspects of the dermatology clinical clerkship.

Results: A total of 152 medical student surveys were completed. Over half of the medical students felt proficient in diagnosing the most commondermatologic conditions. Eighty-seven percent of medical students were very satisfied with the dermatology clerkship. Ninety-one percent of students felt the length of the clerkship was appropriate.

Conclusions: The vast majority of medical students reported a high level of proficiency in the treatment and diagnosis of common skin disorders. In contrast, our findings suggest that medical students may not begaining sufficient hands-on experience in conducting certain dermatologic procedures following the dermatology clerkship. Overall, medical studentperception of the dermatology clinical clerkship was mostly positive.

Commentary

A national cross-sectional analysis of dermatology away rotations using the Visiting Student Application Service database

The highly competitive nature of the dermatology match requires applicants to undertake a variety of measures in the hopes of securing a residency position. Among the opportunities available to applicants is the chance to participate in away or “audition” rotations during their final year of undergraduate medical education. Away rotations are now performed by a majority of medical students applying into dermatology, but littleresearch has been done to describe the nature of this opportunity for interested applicants. An analysis of all dermatology electives offered in the Visiting Student Application Service (VSAS) database wasperformed. Results indicate that students have the option to pursue electives in a variety of subjects offered by 100 sponsoring institutions spread across a wide geographic distribution. Although manyopportunities exist, this analysis sheds light on several areas for improving the quality of this experience for interested applicants, including providing more electives in advanced subject matter, permitting more flexibility in scheduling, and promoting wider participation in VSAS.

Characteristics of research tracks in dermatology residency programs: a national survey

Pursuing research is encouraged in dermatology residency programs. Some programs offer specific research or investigative tracks. Currently, there is little data on the structure or scope of research tracks in dermatology residency programs. An anonymous online survey was distributed to the Association of Professors of Dermatology listserve in 2016. Program directors of dermatology residency programs in the United States were asked to participate and 38 of the 95 program directors responded. The survey results confirmed that a 2+2 research track, which is two years of clinical training followed by two years of research, was the most common investigator trackmodel and may promote an academic career at the resident’s home institution. Further studies will help determine the most effective research track models to promote long-term outcomes.

Elective time during dermatology residency: A survey of residents and program directors

Elective time during residency training provides residents with exposure to different subspecialties. This opportunity gives residents the chance tonurture growth in particular areas of interest and broaden their knowledge base in certain topics in dermatology by having the chance to work withexperts in the field. The purpose of this study was to assess the views of residency program directors and dermatology residents on the value of elective time through a cross sectional survey. An eight-questionIRB exempt survey was sent out to 113 residency program directors via email through the American Professors of Dermatology (APD) program director listserv. Program directors were asked to forward a separate set of 9 questions to their residents. The majority of programs that responded allowed for some elective time within their schedule, often duringthe PGY 4 (3rd year of dermatology training), but the amount of time allowed widely varied among many residency programs. Overall, residents and program directors agree that elective is important in residencytraining, but no standardization is established across programs.

Case Report

Pyoderma gangrenosum, acne, and suppurative hidradenitis syndrome in end-stage renal disease successfully treated with adalimumab

PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH syndrome in a patient with end-stagerenal disease (ESRD) who was successfully treated with the tumor necrosis factor inhibitor, adalimumab. The case underscores the challenges associatedwith the treatment of PASH syndrome as well as the ongoing search to establish a genetic basis for the syndrome. Renal impairment has been reported in association with pyoderma gangrenosum but has notbeen described in PASH syndrome. We believe this to be the first reported case of a patient who developed PASH syndrome in the setting of ESRD.

Rituximab and intravenous immunoglobulin as alternatives to long-term systemic corticosteroids in the treatment of pemphigus: a single center case series of 63 patients

Rituximab and intravenous immunoglobulin [IVIg] have recently emerged as effective treatments for pemphigus refractory to corticosteroids [CS]. This case series sought to compare the clinical, serologic,and adverse effects of CS, IVIg, and rituximab in patients with pemphigus. A retrospective review of 63 patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), or paraneoplastic pemphigus (PNP)was performed. Clinical remission (CR), serologic remission (SR), and adverse effects were evaluated. Three study groups were compared: patients treated with systemic CS, refractory patients treated withIVIg, and refractory patients treated with rituximab. The overall number of adverse effects was not significantly different between the groups but those observed in patients treated with systemic CS weremore severe. CR was less likely in the patients treated with systemic CS than in patients treated with IVIg or rituximab, P-value = 0.000467. SR was more likely in patients treated with systemic CS or rituximab thanin patients treated with IVIg, P-value = 0.002118. These results suggest that the clinical efficacy of IVIg is not correlated with an expected concomitant SR. Frequently reserved for refractory pemphigus,IVIg and rituximab are significantly more likely to produce clinical remission than systemic CS therapy, suggesting their utility as first-line treatments.

NYU Grand Rounds

Papular mucinosis, or localized lichen myxedematosis (LM) (discrete papular type)

Lichen myxedematosus is condition characterized by localized areas of dermal deposition of mucin, presenting with firm papules localized to few areas of the body. The condition needs to be excluded from scleromyxedema, which, in addition to the firm papular eruption, has areas of induration and is usually associated with a monoclonal gammopathyand systemic symptoms. We present a 62-year-old woman with a several-year history of asymptomatic, firm papules over the face and arms with no evidence of thyroid disease or a monoclonal gammopathy,which is consistent with a diagnosis of localized lichen myxedematosus, the discrete papular variant. The patient is being treated with a topical calcineurininhibitor.

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita (EBA) is a rare, acquired subepidermal blistering disease. EBA is characterized by autoantibodies to collagen VII,which serves to link the epidermis to the dermis. The two most common presentations of EBA are classical noninflammatory EBA and bullous pemphigoid-like EBA. Diagnosis of EBA can be challenging as it sharesclinical and histopathologic features with other blistering diseases. Treatment is often recalcitrant and will often necessitate multiple therapies. We presenta case of a thirty-six-year-old Chinese man with EBA and review the literature.

Local heat urticaria

We present a 38-year-old woman with local heat urticaria confirmed by heat provocation testing. Heat urticaria is a rare form of physical urticaria that istriggered by exposure to a heat source, such as hot water or sunlight. Although it is commonly localized and immediate, generalized and delayed onset forms exist. Treatment options include antihistamines and heat desensitization. A brisk, mechanical stroke elicited a linear wheal. Five minutes after exposure to hot water, she developed well-demarcated,erythematous blanching wheals that covered the distal forearm and entire hand.

Generalized hyperhidrosis secondary to presumed eccrine gland dysfunction with possible apocrine metaplasia.

We present a 57 year-old man presented with generalized hyperhidrosis and widespread, smooth, flesh colored papules on the torso and extremities.Histological examination from multiple biopsies demonstrated morphologic alteration of the eccrine glands with an apocrine phenotype, suggesting eitherapocrine metaplasia or the presence of “apoeccrine glands.” The morphologic similarities between eccrine, apocrine, and apoeccrine as they relate to ourpatient’s histologic findings are discussed. We consider secondary causes of generalized hyperhidrosis, which may also play a role in this patient’s presentation. Treatment and further workup are discussed, whilemanagement of this patient remains in progress.

Primary anetoderma with undifferentiated connective tissue disease

Anetoderma is a rare benign elastolytic disorder that is characterized by focal loss of elastin fibers on histopathology and is often recalcitrant to treatment. We present a case of a patient with a 20-year history of pruritic and painful hyperpigmented atrophic papules clustered on the neck, axillae, inframammary folds, and right medial thigh. Although the histopathologyof her axillary lesions was consistent with anetoderma, her clinical presentation is unusual given the extent of involvement, reported pain and pruritus, and sharp demarcation of the distribution. The diagnosticuncertainty of this case led to added difficulty in management of a disease that is already notoriously difficult to treat and may significantly impact patient’s quality of life.

Paraneoplastic Pemphigus in a 34-year-old

Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Diagnosis of PNP requires direct immunofluorescence of perilesional skin and indirect immunofluorescence. Treatment of PNP is difficult and largely limited to glucocorticoids, steroid-sparing immunomodulators, rituximab and intravenous immunoglobulin (IVIG). Despite therapies, prognosis is poor. We report a case of paraneoplastic pemphigus in a 34-year old male with severe stomatitis and lichenplanus-like cutaneous lesions.