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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 24, Issue 9, 2018

Review

Infantile hemangiomas: our current understanding and treatment options

Infantile hemangioma (IH) is the most common vascular tumor of infancy, affecting up to 10% of all infants. Our understanding of IH and its management has greatly evolved. The etiology of IH is unclear but hypoxia is thought to play a key role. Furthermore, GLUT1, IGF2, and HIF-1-? are thought to be important mediators. Current management options include active observation, medical treatment, and surgical intervention. The goals of treatment are preventing cosmetic disfiguration, psychosocial distress, and life-threatening complications. Infantile hemangioma should be managed with an individual, patient-centered approach. Generally, uncomplicated IH can be observed up to 18 months. However, IH should be treated in the setting of bleeding, ulceration, functional compromise, or eventual failure to regress.

Commentary

Psoriasis therapy and aortic inflammation — translating statistical to clinical significance

Psoriasis patients are known to have comorbid aortic vascular inflammation, which is one of the leading causes of cardiovascular morbidity and mortality in this population. Many studies report statistically significant improvements in aortic vascular inflammation after use of tumor necrosis factor inhibitors or interleukin-12/23 antagonists. However, the clinical significance in reduction of adverse cardiovascular events in psoriatic patients owing to biologic therapy has not been examined. Regardless of clinically significant cardiovascular benefits, dermatologists should continue to treat psoriasis patients optimally to mitigate the unfavorable effect this disease has on quality of life.

Genital Rejuvenation: The Next Frontier in Medical and Cosmetic Dermatology

Genital rejuvenation encompasses not only the nonsurgical interventions but also the surgical procedures that are utilized to improve the functional aspects and/or enhance the aesthetic presentation of the genitalia of women (vaginal rejuvenation) and men (scrotal rejuvenation). Vaginal rejuvenation was introduced into the medical literature in 2007; yet, within the last decade, physician and patient interest in this field has markedly increased. In contrast, the term scrotal rejuvenation was only coined in 2018. Rejuvenation of the genitalia may be considered for hair-associated (alopecia and hypertrichosis), morphology-associated (vulvovaginal atrophy, excess clitoral or labial tissue, scrotal wrinkling, and vaginal or scrotal laxity), and vascular-associated (angiokeratomas) changes of the vagina and scrotum. As women and men gain insight into the conditions that are amendable to genital rejuvenation, the demand for vaginal rejuvenation and scrotal rejuvenation will likely increase. Genital rejuvenation may become the next frontier in medical and cosmetic dermatology and dermatologists have the opportunity to provide rejuvenation of the vagina and scrotum for their patients.

Dermatologic features in good film characters who turn evil: the transformation

Dermatologic depictions in film are often used to symbolize evil. To ascertain whether certain dermatologic characteristics are more representative of evil in movies, skin findings in film characters before and after evil transformation were compared using the Good Movie Characters Turned Bad poll courtesy of The Internet Movie Database (IMDb). The poll ranked the 35 most remarkable moral transformations in cinema. Data was analyzed from 27 characters who met study criteria and there was a statistically significant, greater number of dermatologic features seen in characters after an evil transformation. Periorbital hyperpigmentation, infraorbital edema, alopecia, pallor, and nonspecific hyperpigmented skin abnormalities (telangiectasias, ecchymosis) were dermatologic traits that were found at a statistically significantly higher frequency post-evil transformation. This work suggests an effort should be made to educate audiences and film makers that skin conditions in reality are not a marker of evil intent.

Tuberculosis and interleukin blocking monoclonal antibodies: Is there risk?

Several new monoclonal antibodies that interfere with interleukin (IL) cascades have come to market in recent years. They follow a generation of drugs that block tumor necrosis factor (TNF). It has been well established that TNF is important in the containment of Mycobacterium tuberculosis (Mtb) and that blocking this cytokine increases the risk of tuberculosis (TB) infection. Thus, judicious screening for Mtb of patients taking TNF blocking drugs has been the standard of care. It remains unclear if the newer monoclonal, interleukin blocking drugs, which affect IL-12, IL-23, and IL-17 pathways are associated with risk of Mtb reactivation. Herein we discuss what is known about the immunologic response to Mtb and discuss the data that is currently available for the new interleukin monoclonal antibody blocking medications regarding the risk of latent TB reactivation or active TB infection.

Original

The use of notable protagonists in dermatology clinical cases: A quasi-randomized controlled trial

Background: Educators have attempted several methods to create more entertaining problem-based learning (PBL) experiences and more engaging PBL patients. To this end, our study compared the use of unique, memorable PBL characters with generic, unmemorable characters. Methods: This prospective quasi-randomized controlled study utilized 476 university students. All subjects read ten medical cases that focused on dermatological illnesses. Cases were identical for everyone except subjects were allocated to have notable protagonists (NP) (i.e. cartoon characters or celebrities) or generic protagonists (GP) as patients in their cases. Surveys and tests were completed immediately, 7-10 days later, and 28-31 days later. Results: There were no significant differences in post-test scores at any point between the groups. The only significant difference with regard to the subjective learning experience was for the entertainment level of the cases. The NP mean was 64.1±24.2, whereas the GP mean was 56.0±24.6 (t[444]=3.52, P=0.0005). The NP group also had a significantly higher proportion of subjects who researched dermatology/medicine topics after reading the cases (10.6% versus 2.7%, χ²(1,N=215)=5.47, P=0.02). Conclusions: The current study found that cases utilizing NPs, while still preserving the same educational value as cases using GPs, can provide a more entertaining learning experience and stimulate outside learning.

Case Report

The development of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder at the site of a melanoma excision scar

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell lymphoma (CTCL), representing 2% of all primary cutaneous lymphomas. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear. We present the first case of PCSM-LPD, to our knowledge, arising at a past melanoma excision site. A 72-year-old woman with a past medical history significant for melanoma-in-situ excised 36 years ago presented to our clinic for evaluation of a single, erythematous plaque of the posterior arm within a melanoma excision scar. A biopsy was performed, revealing PCSM-LPD. Reports of the development of other T-cell lymphoproliferative disorders after prior skin trauma such as chemical burns, thermal injury, and mechanical trauma exist in the literature. Physicians should be aware of the possibility of the appearance of T-cell lymphoproliferative disorders at the site of scars or prior trauma with a time lag of months to years.

Scabies presenting as cutaneous nodules or malar erythema: reports of patients with scabies surrepticius masquerading as prurigo nodularis or systemic lupus erythematosus

Scabies surrepticius is a unifying term that represents non-classical presentations of scabies mite infestation. A patient with scabies surrepticius is described: a man with scabies masquerading as prurigo nodularis. The 91-year-old man had metastatic prostate cancer and presented with diffuse pruritic nodules. Prurigo nodularis was suspected; however, the biopsy revealed scabies mites in the stratum corneum. He was successfully treated with topical permethrin 5% cream and oral ivermectin. In addition, the features of a woman with scabies mimicking systemic lupus erythematosus are summarized. The 47-year-old woman had idiopathic thrombocytopenic purpura and presented with malar erythema and a positive antinuclear antibody (titer 1:320). A diagnosis of systemic lupus erythematous was entertained until skin scraping and mineral oil preparation revealed scabies mites; she was successfully treated with oral ivermectin. In conclusion, Sarcoptes scabiei infestation can present with atypical clinical morphology and an absence of classical lesions such as burrows conventionally distributed in the interdigital web spaces, volar wrists, periumbilical area, or genitalia. Scabies surrepticius is a term that has been designated to describe these unusual presentations. Prurigo nodularis and systemic lupus erythematosus can be added to the litany of conditions masquerading as scabies and are included amongst the guises of scabies surrepticius.

Case Presentation

Subcutaneous panniculitis-like T-cell lymphoma responsive to combination therapy with methotrexate and corticosteroids

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare condition that falls underneath the umbrella of primary cutaneous T-cell lymphomas (CTCLs). SPTCL can be very difficult to diagnose as it may mimic other subtypes of CTCL, such as γ/δ T-cell lymphoma (TCL), or other forms of panniculitis. Confirmation of diagnosis often requires immunohistochemical analysis and is essential for proper prognosis and therapeutic management. Herein, we present a case of SPTCL that mimicked lupus panniculitis and was successfully treated with prednisone taper and methotrexate.

Nodular amyloidosis in a patient with systemic scleroderma

Primary cutaneous amyloidosis may be characterized as macular amyloidosis, lichenoid amyloidosis, or nodular amyloidosis. Nodular amyloidosis results from the deposition of immunoglobulin light chains and may rarely be associated with systemic amyloidosis. We report an unusual case of a patient with systemic scleroderma who developed primary cutaneous nodular amyloidosis on the left lower leg. The diagnosis was confirmed with a skin biopsy with Congo red staining and a novel technique using a laser microdissection and mass spectrometry-based proteomic analysis method for amyloid protein characterization. A work-up for systemic amyloidosis was negative and the patient improved symptomatically with wound care. Patients with primary cutaneous nodular amyloidosis should be followed clinically over time for the possible development of systemic amyloidosis, although the risk of disease progression is likely low.

A trichogenic tumor with aggressive features initially diagnosed as basal cell carcinoma

Trichoblastic carcinoma is a rare carcinoma often arising in a pre-existing trichoblastoma. It may resemble basal cell carcinoma, posing a diagnostic challenge. Trichoblastic carcinoma is divided into low-grade and high-grade tumors. Low-grade tumors resemble basal cell carcinomas and are therefore synonymous in some classifications. High-grade tumors, which commonly present on the scalp in older individuals or in patients with Brooke-Spiegler syndrome, have been associated with a higher potential for distant metastasis and death. We present a case in which a 73-year-old female had a long-standing scalp nodule for over 30 years that rapidly increased in size. The patient's lesion was initially diagnosed as basal cell carcinoma on shave biopsy, but upon excision, revealed features concerning for trichoblastic carcinoma such as brisk mitotic activity and comedo-like necrosis. Sudden change in an atypical scalp lesion that has been present for many years should increase suspicion for an atypical trichogenic tumor, such as trichoblastic carcinoma.

Seven-year itch: a perplexing case of lichen planus-lupus erythematosus overlap syndrome

Lichen planus-lupus erythematosus overlap syndrome is a rare disorder characterized by clinical and histopathological features of both lichen planus (LP) and lupus erythematosus (LE). Cutaneous lesions commonly affect the distal arms, legs, face, and trunk and these plaques are often large, scaly, painful, and atrophic, often exhibiting hypopigmentation or a red to blue-violet color. We report a case of LP-LE overlap syndrome diagnosed in a man previously believed to have atypical lichen planus who presented with an exacerbation of exuberant pruritic erythematous scaly plaques. The patient had six separate skin biopsies all of which displayed features of LP. Because the clinical symptoms did not correlate to the histopathological picture, a seventh skin biopsy with direct immunofluorescence (DIF) was performed and immunologic markers measured. The DIF demonstrated early lupus bands; serologic testing exhibited elevated ANA and anti-SSA. These findings established the diagnosis of LP-LE overlap syndrome. The patient was started on hydroxychloroquine with short-term trials of oral prednisone during disease flares, which took place in the first three months of treatment.

Photo Vignette

Aplasia cutis congénita con defecto óseo subyacente: evolución favorable tras manejo conservador

La aplasia cutis congénita es una rara alteración caracterizada por la ausencia de áreas localizadas de piel en el momento del nacimiento. Suele manifestarse como una lesión solitaria localizada principalmente en el cuero cabelludo, que puede estar asociada con otras malformaciones congénitas. Las complicaciones pueden ser fatales, por lo que es necesario un tratamiento individualizado que vendrá determinado por el tamaño, localización y grado de afectación de estructuras subyacentes. Presentamos un caso de aplasia cutis congénita del cuero cabelludo con múltiples lesiones y defecto óseo subyacente de 3 × 1.5 cm de tamaño, pero sin otras anomalías asociadas. El manejo conservador permitió una adecuada y completa epitelización cutánea con cierre del defecto óseo subyacente sin necesidad de procedimientos invasivos.

Letter

The successful treatment with ixekizumab in a multi-failure psoriasis patient

We report a patient with severe psoriasis who failed to respond to phototherapy, conventional systemic treatment and four biologic agents (etanercept, ustekinumab, adalimumab and secukinumab). Combination of a higher-dose secukinumab regimen with phototherapy had no success. Remarkably, ixekizumab, an IL-17A inhibitor, provided almost complete psoriasis clearance after 24 weeks of treatment. The reason for the success of ixekizumab after the failure to respond to a biologic with same mechanism of action is still unknown. Interestingly, failure of secukinumab does not preclude future therapeutic success with a second IL-17A-inhibitor.

Secukinumab for psoriasis in a patient with hepatitis B

The case reported describes a 48-year-old man with congenital hepatitis B receiving secukinumab for treatment of psoriasis. Some biologic therapies have been associated with an increased risk of reactivation of hepatitis B. In the case of this patient, secukinumab has successfully managed his psoriasis without evidence of hepatitis B virus reactivation.

Effective treatment of nail psoriasis with apremilast: report of two cases and review of the literature

Nail psoriasis can cause great morbidity and a negative impact on the personal and work-related life of the patients. However, it responds more poorly to most drug therapies. Classically, the first line of treatment for nail psoriasis has been topical medication, but the new biological drugs seem to be the most effective treatment. Apremilast is another systemic oral drug that has shown a significant reduction of the severity in moderate-severe plaque psoriasis, as well as nail and scalp psoriasis. We present two cases of patients who exhibited a rapid response to treatment with apremilast.

The ginseng pimple: an inflammatory papule following ginseng consumption

Ginseng is a popular herbal remedy derived from the plant roots of the Panax genus and has been used in traditional Asian medicine for thousands of years. In the United States, it has become increasingly popular and is taken for many conditions, including as an immune enhancer. Cutaneous adverse effects have been reported to occur following ginseng consumption, although detailed clinical descriptions are limited. A 60-year-old woman who repeatedly developed inflammatory papules following ginseng consumption is described and the characteristics of ginseng use in healthcare are reviewed.

Corrigendum

Corrigendum: Editorial boards of dermatology journals and their potential financial conflict of interest

The original article was published on August 15, 2018 and corrected on September 15, 2018.

The revised version of the article removes a co-author, unintentionally retained during the editorial proofing process. This change appears in the revised online PDF copy of this article.

Retraction

RETRACTED: Ehlers-Danlos syndrome caused by the c.934C>T, p. Arg312Cys mutation in COL1A1 gene: an Italian family without cardiovascular events

The article entitled “Ehlers-Danlos syndrome caused by the c.934C>T, p. Arg312Cys mutation in COL1A1 gene: an Italian family without cardiovascular events” has been retracted because the description and characterization of the disease in a family may have been previously published. Upon publication of this article we were notified by an author of a study appearing in 2016 in another journal claiming that characteristics and symptoms of the family described closely matched their study, and that the two studies describe the same family. Whereas constituent family members appearing in both articles were not identical (differing by one member), symptoms and diagnoses of each family proband appeared to be consistent in both studies, leading to the editors’ conclusion that it is likely that the same family was being described in two separate articles.The corresponding author of the article in Dermatology Online Journal was informed of this incident, and responded with the assertion that they were unaware of the study published in 2016, and provided no additional information. They further requested that their article be retracted. In light of the available information and author’s request, the editors of Dermatology Online Journal have retracted this article.The original article was published on July15, 2018 and corrected on September 15, 2018.The original article was published on July15, 2018 and corrected on September 15, 2018.