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 27, Issue 2, 2021
Basal cell carcinoma associated with non-neoplastic cutaneous conditions: a comprehensive review
Basal cell carcinoma (BCC) can be a component of a collision tumor in which the skin cancer is present at the same cutaneous site as either a benign tumor or a malignant neoplasm. However, BCC can also concurrently occur at the same skin location as a non-neoplastic cutaneous condition. These include autoimmune diseases (vitiligo), cutaneous disorders (Darier disease), dermal conditions (granuloma faciale), dermal depositions (amyloid, calcinosis cutis, cutaneous focal mucinosis, osteoma cutis, and tattoo), dermatitis, miscellaneous conditions (rhinophyma, sarcoidal reaction, and varicose veins), scars, surgical sites, systemic diseases (sarcoidosis), systemic infections (leischmaniasis, leprosy and lupus vulgaris), and ulcers. The relationship between the BCC and the coexisting non-neoplastic condition may be coincidental or possibly related to the development of the BCC; alternatively, the development of the BCC may be unrelated to the coexisting non-neoplastic conditions and secondary to either a Koebner isomorphic response or a Wolf isotopic response in an immunocompromised district of skin. This paper reviews several of the case reports and studies that describe the association of BCC with these non-neoplastic cutaneous conditions.
Free open access data sources for dermatology researchers
Free open access data sources are a valuable tool for dermatology researchers and may uncover crucial information about dermatological diseases and delivery of dermatological care. This short review discusses six open access data sources including: National Health and Nutrition Examination Survey (NHANES), National Health Interview Survey (NHIS), National Survey of Children's Health (NSCH), National Ambulatory Medical Care Survey (NAMCS)/National Hospital Ambulatory Medical Care Survey (NHAMCS), Centers for Medicare and Medicaid Services (CMS), and Gene Expression Omnibus (GEO). We explain the role of each data source in dermatology and provide examples of past studies, which have used these data repositories.
Rosacea videos on social media: A comparison of accuracy, quality, and viewer engagement
Background: Little is known regarding the accuracy, quality, and viewer engagement of video-based online education. Objective: To assess the accuracy, quality, viewer engagement, and viewer experience of rosacea videos on social media. Methods: Reviewers searched "rosacea" and examined videos on YouTube in September 2018. Videos were categorized by source: 1) Healthcare sources: university/professional organizations, industry, and individuals that were healthcare professionals, and 2) Non-healthcare sources: lay media and individuals that were not healthcare professionals. Video accuracy was measured using the Dy et al. Accuracy Scale (DAS) and Accuracy in Digital-health Instrument (ANDI). Video quality was measured using the Global Quality Scale (GQS). Viewer engagement was measured by the engagement ratio (total likes+dislikes+comments/total views). Results: Of the videos analyzed, 71.7% of videos were from non-healthcare sources. Videos produced by healthcare sources (28.3%) were significantly more accurate than those produced by non-healthcare sources, as measured by ANDI (3.57±0.83 versus 2.54±1.07, P=0.001). Videos created by non-healthcare sources received significantly greater engagement than those by healthcare professionals (viewer engagement ratio 0.031±0.044 versus 0.014±0.013, P=0.0159). Conclusion: Rosacea videos on social medi produced by non-healthcare sources were less accurate and of lower quality but received greater viewer engagement than those produced by healthcare sources.
Assessing the utility of the decoy effect on biologic treatment preference
Background: Many patients struggle with choosing and adhering to biologics. Psychological approaches (e.g. decoy effect) may impact patients' choices when selecting a biologic. Objective: Assess whether decoy options influence choice between injectable treatment options. Methods: Following IRB approval, 750 subjects >18 years were recruited through MTurk. Subjects were randomized in a 1:1:1 ratio into the following groups: 1) baseline comparison between a more effective, weekly injection and a less effective, every-three-month injection; 2) baseline with a decoy inferior to the weekly injection; 3) baseline with a decoy inferior to the every-three-month injection. Treatment preference was self-reported and compared using chi-square tests. Results: Sixty-six percent of subjects preferred the weekly injection versus 34% for the every-three-month injection (group 1). There was a 4% increase in the number of subjects who preferred the weekly injection (70%; group 2; P=0.34) and a 3% increase for the every-three-month injection (37%) when a decoy inferior to them was included (group 3; P=0.56). Conclusion: Psychological approaches can be used to enhance treatment initiation and adherence. However, the decoy effect did not appear to have a significant impact in this study. Patients' preferences for efficacy versus frequency of injection may be rather fixed.
Psychodermatology fellowship: is it time?
The fields of dermatology and psychiatry have overlap in the patient populations and diseases they treat. However, there are not currently fellowship opportunities for dermatologists or psychiatrists specifically interested in gaining expertise in psychodermatology. We discuss the logistics of a one-year psychodermatology fellowship, open to residents after completing either dermatology or psychiatry residencies, and the potential benefits that it could bring to both dermatology and psychiatry training programs across the county.
Progressive nodular histiocytosis: an unusual disorder
Progressive nodular histiocytosis (PNH) is a rare type of non-Langerhans cell histiocytosis of the xanthogranuloma group. Less than 20 cases have been reported. We report here a novel case of PNH with dermoscopic description and post-surgical outcome. Our patient presented with pruritic papules and nodules with progression over two years. Dermoscopic examination of large lesions showed multiple telangiectases without ulceration. There was no mucosal involvement. Histopathological analysis of a papule showed a dermal infiltrate composed of histiocytes and many Touton giant cells; a nodule revealed a dermis occupied by spindle cells arranged in a storiform pattern. The nodules were resected and the largest surgical defect, on the forehead, was corrected with A-T advancement flap. The papules were treated with cryosurgery achieving adequate cosmetic outcome without recurrence.
Erosive pustular dermatosis of the scalp induced by gefitinib: case and review of the literature
Gefitinib is a selective tyrosine kinase inhibitor of epidermal growth factor receptor (EGFR) used for the treatment of malignant neoplasms. The most frequent skin complication during gefitinib therapy is an acneiform papulopustular eruption, usually distributed in the seborrheic areas but occasionally widespread. We report a patient with erosive pustular dermatosis of the scalp, a neutrophil-mediated skin disease presenting with sterile pustules evolving into erosions and crusts on the scalp, during treatment with the EGFR inhibitor gefitinib for lung cancer. A literature review of the drug-induced cases of this rare entity is provided.
Loose anagen syndrome in one identical twin girl
Loose anagen syndrome (LAS) is a hair disorder involving insufficient anchoring of the hair follicle to the scalp owing to an autosomal dominant or sporadic mutation in the gene encoding keratin 6. There are three phenotypes of LAS, including type B, which presents in young, light-haired girls as unruly, uncombable hair with diminished growth. We present a 2-year-old girl with LAS type B whose identical twin sister was unaffected. The diagnosis was confirmed with a painless hair pull test proven to contain anagen hairs with ruffled cuticles on trichoscopy, preventing the need for unnecessary referrals and diagnostic tests.
The use of Goeckerman therapy in managing erythrodermic psoriasis resistant to multiple medications
Erythrodermic psoriasis is a relatively rare, more dangerous inflammatory variant of psoriasis associated with high morbidity and mortality. It can be exceptionally challenging to manage, defeating even the most experienced dermatologist's arsenal of treatment strategies. Goeckerman therapy, a regimen of ultraviolet B phototherapy and crude coal tar, has demonstrable efficacy in severe and recalcitrant plaque-type psoriasis. However, its utility in erythrodermic psoriasis has not been explored within the dermatology literature. Herein, we present a patient with a long-standing history of erythrodermic psoriasis refractory to eleven treatment modalities including four biologic agents, who had his erythroderma 'turned around' following Goeckerman therapy. 'Turned around' is used to describe dramatically reducing a patient's cutaneous inflammation so that previously recalcitrant disease can now respond to maintenance therapy. The importance of a one to three week 'cool down' period of topical corticosteroid therapy prior to phototherapy or crude coal tar use is highlighted in this case as well. Although Goeckerman therapy is no longer regularly used, it remains one of the most efficacious treatments available for intractable psoriasis, attracting patients from all over the country desperate for symptom relief. This case suggests it may be useful in 'turning around' extremely difficult-to-treat erythrodermic psoriasis as well.
Epidermodysplasia verruciformis: report of two patients with autosomal dominant inheritance
Epidermodysplasia verruciformis is a rare genodermatosis associated with mutations in the EVER1/TMC6 and EVER2/TMC8 genes. The inheritance is considered to be autosomal recessive, but reports suggesting an autosomal dominant inheritance indicate disease genetic heterogeneity. Its onset occurs in early childhood and presents as a combination of pityriasis versicolor-like, flat wart-like and seborrheic keratosis-like lesions, with a potential for malignant transformation, mainly squamous cell carcinoma.
Response of pruritic scrotal epidermolytic acanthomas to pimecrolimus 1% cream
Epidermolytic acanthomas (EA) are rare benign tumors of unclear etiology that present as flat, sometimes slightly keratotic, pale or whitish papules that are usually asymptomatic. Not uncommonly, their clinical appearance in the anogenital area might lead to misdiagnosis as other lesions that commonly develop at this site, such as condylomata acuminata. Though mainly asymptomatic, there are also reports of EA presenting with persistent genital pruritus. We describe the first reported case of pruritic scrotal EA successfully treated with topical pimecrolimus.
Targetoid lesions in a patient with systemic lupus erythematosus
Rowell Syndrome is a rare presentation of lupus erythematosus manifesting as an eruption of erythema multiforme-like papules and plaques with immunological findings of positive rheumatoid factor, speckled antinuclear antibodies, and/or anti-Ro/La antibodies. This case highlights the unusual and highly debated presentation of Rowell Syndrome in a 66-year-old woman with newly-appearing erythematous, targetoid plaques in the setting of previously diagnosed systemic lupus erythematosus. Skin biopsy revealed histological features of full-thickness epidermal necrosis with focal sub-epidermal separation and a superficial perivascular lymphocytic infiltrate interpreted to favor Rowell Syndrome given her clinical history and presentation. Although no standard treatment exists, a prednisone taper and topical corticosteroids proved effective initially, with complete resolution at six months on mycophenolate mofetil and belimumab.
Vulvar edema: diagnostic clue to Crohn disease
Vulvar lesions are clinically challenging for physicians because the differential diagnosis may include many entities. Vulvar edema is one of the most frequent symptoms and is normally attributed to a local cause. Herein, we present a case report of vulvar Crohn disease (VCD) in a 9-year-old girl, in which skin lesions preceded the systemic gastrointestinal symptoms. Both clinical features and histopathological findings guided us to an early Crohn disease (CD) diagnosis. Dermatologists often have the opportunity to detect systemic diseases at early stages. A good knowledge of the CD skin manifestations could lead to an early CD diagnosis, especially in children. The suspicion of CD in those cases in which cutaneous involvement precedes digestive symptoms is crucial to prevent future psychological and physical consequences.
Generalized epidermolytic ichthyosis with palmoplantar hyperkeratosis
Epidermolytic ichthyosis (EI, OMIM 113800) is a rare autosomal dominant keratinization disorder that is caused by keratin 1 or 10 gene mutation. It can be classified clinically based on the presence of palmoplantar hyperkeratosis involvement and extent of skin involvement. The diagnosis is made by clinical and histopathological examinations that can be confirmed by genetic testing. We present a 2-year-old girl who presented with erythematous and thick scaling skin. Her condition began at birth as multiple flaccid blisters that would easily break into erosions. There was no history of similar condition nor consanguinity within her family. Skin examination revealed diffuse erythematous skin covered with thick scales and erosion, predominantly on her face, extremities, palms, and soles. The skin histopathology examination showed diffuse parakeratosis with vacuolar and granular degeneration within granular and spinous layers along the epidermis. She was diagnosed with generalized EI with palmoplantar hyperkeratosis based on the clinical and histopathological examinations. Clinical improvement was observed after a one-month treatment with mupirocin cream, sodium bicarbonate bath, and moisturizer after bathing.