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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 26, Issue 12, 2020

Review

Recent advances in hidradenitis suppurativa in pediatrics

Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition that can cause significant physical, mental, and socioeconomic burden. There remains a paucity of literature on HS in the pediatric population. This systematic review highlights recent advances in pediatric HS in epidemiology, presentation, comorbidities, and management. PubMed, Embase, Google Scholar, and Clinicaltrials.gov databases were used to identify trials and articles published on HS in pediatric patients between January 2015 and October 2019. A total of 39 articles were included. Current evidence suggests that pediatric onset HS may be associated with genetic factors along with endocrine and metabolic abnormalities. Delayed diagnosis in children with HS contributes to poor outcomes. Overall, children and adults with HS share similar lesion types and involved areas. Pediatric HS is associated with a number of comorbid conditions including acne, obesity, inflammatory joint disease, Down syndrome, inflammatory bowel disease, and diabetes. There are currently no pediatric treatment guidelines. Adalimumab is approved for the treatment of moderate-to-severe HS in children 12 and older. Other targeted immunomodulators and hormonal modulators are under investigation. Although the number of studies concerning HS are increasing, further investigation is warranted to better characterize HS, facilitate early diagnosis, and determine the best management for children.

Cutaneous manifestations of COVID-19: a systematic review and analysis of individual patient-level data

Distinctive patterns in the cutaneous manifestations of COVID-19 have been recently reported. We conducted a systematic review to identify case reports and case series characterizing cutaneous manifestations of confirmed COVID-19. Key demographic and clinical data from each case were extracted and analyzed. The primary outcome measure was risk factor analysis of skin related outcomes for severe COVID-19 disease. Seventy-one case reports and series comprising 144 cases of cutaneous involvement in COVID-19 were included. The most frequently occurring morphologies were: morbilliform (30.6%), varicelliform (18.8%), urticarial (13.2%), chilblains-like (12.5%), and acro-ischemic (9%). The median age of patients was 51 years (mean: 45.9, range: 0 to 91). Patients with chilblains-like eruptions had lower frequencies of extracutaneous COVID-19 symptoms (5/18, 27.8%, P<0.05) and were less likely to have severe COVID-19 disease (2/18, 11%, 95% CI 1.4% to 34.7%, P=0.02). Patients with livedoid and acro-ischemic morphologies had severe COVID-19 more frequently than those with other morphologies (17/21, 81%, 95% CI 58.0% to 94.5%, P<0.0001). The most frequently observed cutaneous manifestations of COVID-19 (morbilliform, varicelliform, and urticarial) are well-described patterns of viral exanthems. However, chilblains-like, livedoid, and acro-ischemic morphologies are not traditionally associated with viral infections and were significantly associated with severity of COVID-19 disease.

Original

Clinical evaluation of infantile hemangiomas treated with atenolol

Currently, propranolol, is the first line treatment for problematic infantile hemangioma (IH) management. However, serious side effects have been reported. For that reason, atenolol, a hydrophilic selective beta-1 blocker with the potential for fewer side effects, has been explored. A descriptive, observational case series study of 30 patients between the ages one to 5 months with superficial, deep, or mixed IH was conducted between January 2016 and December 2017. Oral atenolol was administered using a single once daily dose of 1mg/kg, which was adjusted for weight gain each month. The IH was assessed using the Hemangioma Activity Score (HAS) at initiation of treatment, four months, and 9 months of age and improvement percentage was calculated at four and nine months of age. A total of 25 patients completed three evaluations. The baseline, four-month, and 9-month HAS were 4.6, 2.39, and 0.65, respectively. Mean improvement percentage at four months of age was 46.76% and at 9 months of age was 85.65%. No side effects were reported. This study suggests atenolol as an effective treatment for IH in almost all cases, especially in patients who initiated treatment before three months of age. It was well tolerated in all our cases.

Commentary

Representation of Fitzpatrick skin type in dermatology textbooks compared with national percentiles

As time progresses, more patients with skin of color will be seen by dermatologists. To meet the needs of the ever-changing population, medical education needs to analyze how residents are trained in recognizing dermatological disorders in patients with skin of color. The aim of this study was to analyze dermatology textbooks to evaluate how well skin of color patients are represented compared to the current national distributions. The most common skin types depicted in the textbooks were Fitzpatrick skin types II and III, whereas the least common skin types depicted were skin types V and VI. There was a significant difference between a national distribution of skin types when compared to photographs in each of the textbooks (P<0.001). These findings emphasize the need to better represent patients with skin of color in medical textbooks.

Teledermatology application use in the COVID-19 era

Owing to the COVID-19 outbreak, the use of telemedicine applications has increased throughout the United States. Using an algorithm to analyze mobile application rankings, we were able to examine how applications with telemedicine services have increased in prevalence and rank pre- and post- COVID-19. Telemedicine apps saw an increase of 210.92 ranked positions on average. Within US telehealth, skin conditions have become the fifth most common diagnosis. Widespread use of teledermatology has been well-accepted. Dermatologists and patients report high satisfaction using teledermatology during COVID-19 and intend to continue using these services in the future. COVID-19 has assisted in reducing physician concerns previously preventing some dermatologists from utilizing teledermatology in their services. Additionally, the geographical and socioeconomic barriers preventing some patients from receiving dermatologic care have been minimized through the use of teledermatology. Addressing these obstacles for dermatologic care improves healthcare equity.

Drug costs of Medicaid-covered therapies for pemphigus vulgaris treatment

Pemphigus vulgaris is the most common form of pemphigus affecting an estimated 30,000-40,000 people in the United States. Costs of systemic and immunoglobulin therapies for pemphigus vulgaris have remained persistently high. Herein, we address the current costs and changes in costs of immunosuppressive treatments, anti-inflammatory treatments, and immunoglobulin treatments covered by Medicaid for pemphigus vulgaris from 2013-2020.

Correlation of well-being during dermatology residency with future career plans

Burnout is increasingly common in the medical field. In dermatology, burnout is attributed to high patient volume and excessive time spent on electronic medical record system activities. The shortage of the dermatology workforce in academic medicine is well-known. Studies have yet to examine the relationship between well-being during dermatology residency and the pursuit of academia. Objective: To assess the well-being of dermatology residents in the United States, identify barriers/enablers to well-being, and determine the implications of these measures. Methods: A cross-sectional survey was distributed to program coordinators of 136 accredited dermatology programs with instructions to forward to their current dermatology residents. Residents provided self-reported ratings on validated scales measuring burnout, depression, anxiety, fatigue, and quality of life. Descriptive statistics and correlations were examined. Results: Residents with higher levels of burnout reported they were significantly less likely to pursue academia, full-time work, clinical research, and fellowships after residency. The results showed opposite effects for residents with higher qualities of life. Conclusions: This study showed that resident well-being can have a significant impact on residents' future career plans, including pursuing academic dermatology, clinical research, and fellowship. Addressing burnout in the field of dermatology offers an opportunity to increase the academic dermatology workforce.

Self-reported tattoo prevalence and motivations in transgender adults: a cross-sectional survey

Purpose: To assess the prevalence and motivations for obtaining tattoos among transgender persons. Methods: A survey of 696 transgender persons recruited from the Study of Transition, Outcomes, and Gender (STRONG) cohort evaluated the prevalence of tattoos and motivations for acquiring tattoos. Results: Transmasculine persons were more likely than transfeminine persons to have tattoos (66.5% versus 24.0%, P<0.05). Most commonly reported motivators were personal preference, aesthetics, and/or symbolism (61.8%). Scar coverage and replacement of anatomic features accounted for 10.2% of responses. Conclusion: Future studies should look into the relationship between tattoos and health status in the transgender population.

The dermatology residency application process

The dermatology application process is grueling, that is tough to navigate without the proper guidance. This commentary is meant to shed light on the factors that can help applicants stand out in order to be successful in the match. It includes observations from successful applicants from the most recent match process.

Case Report

Childhood granulomatous periorificial dermatitis: case report and review of the literature

Childhood granulomatous periorificial dermatitis (CGPD), considered a clinical variant of perioral dermatitis, typically affects prepubertal children of African descent. It is a condition of unknown etiology characterized by the presence of a monomorphic yellow-brown papular eruption limited to the perioral, perinasal, and periocular regions that histopathologically shows a granulomatous pattern. This disorder should be differentiated from other conditions as granulomatous rosacea, sarcoidosis, and lupus miliaris disseminatus faciei. We report a case of a 9-year-old boy who presented with flesh-colored perorificial papules on the face, evolving for two months. Upon treatment with topical tacrolimus for follicular eczema, an aggravation of the condition was observed. A skin biopsy confirmed the diagnosis of CGPD. Our patient was successfully treated with a combination of topical metronidazole and topical erythromycin.

PD1 inhibitor induced inverse lichenoid eruption: a case series

The increased use of monoclonal antibodies that target the immune checkpoint T cell receptor programmed death-1 (PD1) to treat numerous solid tumors has led to several reports describing associated cutaneous adverse events. Although lichenoid reactions have been well described, we propose that PD1 inhibitor-induced inverse lichenoid eruption (PILE) is a distinct variant. We describe two patients who presented with nearly identical deeply erythematous, malodorous, eroded anogenital plaques with focal crusting. Diagnosis of PILE was established given the biopsy findings and temporal association with PD1 inhibitor therapy. Treatment with clobetasol ointment was successful without necessitating discontinuation of immunotherapy. The findings were consistent with the only other previously published case of inverse lichenoid eruption in the groin secondary to PD1 inhibitors.

Acne fulminans induced by a low dose isotretinoin: case report and review of the literature

Acne fulminans is a rare complication of classic acne. Less than 200 cases have been reported. It usually affects adolescent males with pre-existing acne vulgaris. It is characterized by an acute eruption of numerous and large inflammatory nodules, plaques, erosions, and ulcers covered by hemorrhagic crusts. The disorder may occur spontaneously or may be triggered by isotretinoin. We report a young boy who developed acne fulminans after isotretinoin therapy at a dose of 0.1mg/kg/day. A systematic literature review gathering previously reported cases on PubMed revealed that one similar case has been reported. Regarding therapeutic strategies, there are no randomized clinical trials to identify the best treatment for acne fulminans. Recommendations are based on case series and case reports. We share this case to raise awareness of the induction of acne fulminans by a very low dose of isotretinoin.

Solitary circumscribed neuroma of the glans penis. An unusual finding

Palisaded encapsulated neuroma is a rare, benign neural tumor. The involvement of the glans penis is rare; few cases have been reported. We present a 52-year-old man with a five-month course of a solitary painless lesion of the glans penis. Full excision of the nodule was performed. Histopathological and immunohistochemical analyses and examination was consistent with a palisaded encapsulated neuroma. We describe one of a few existing cases of this kind of tumor in the glans penis.

Case Presentation

Lichenoid sialadenitis in chronic graft versus host disease

Chronic graft versus host disease (cGVHD) remains the principal long-term life-threatening complication in hematopoietic stem cell transplant recipients. We present a case of lichenoid sialadenitis in a 23-year-old-man with systemic cGVHD. The histological examination showed a lymphocytic inflammatory infiltrate adjacent to the salivary gland duct, similar to the histological aspects described in the typical manifestations of oral lichen planus and lichen planopilaris. This consists of a band-like inflammatory infiltrate not only targeting the cutaneous epithelium but also adnexal structures, such as hair follicles and salivary gland ducts. It is well described that the oral lesions in cGVHD share most of morphological and clinical manifestations with those described in oral lichen planus. The mechanisms of lichenoid salivary gland ducts destruction might be similar, although xerostomy appears to be specific to cGVHD, which may represent a clinical sign of massive salivary gland impairment related to ductal lichenoid destruction in patients with cGVHD.

Disseminated cryptococcosis presenting with cutaneous involvement in an immunocompromised patient

Cryptococcosis is a fungal infection that typically affects immunocompromised patients. It most commonly affects the lungs and may then disseminate to the central nervous system, bone, skin, and adrenal glands. Herein, we describe a 69-year-old man who presented with skin lesions as the initial manifestation of disseminated cryptococcosis. Initial workup led to an assumption that the patient was immunocompetent. Later in the clinical course, idiopathic depletion of CD4 T cells was discovered. This case highlights that disseminated cryptococcosis may present with cutaneous symptoms even when there is no evidence of pulmonary or central nervous system involvement and may be the first sign of an underlying cellular immune dysfunction.

Necrotizing sialometaplasia: a case report of a non-ulcerated histopathological presentation

A 27-year-old woman presented with the chief complaint of severe pain in the palate region, which had been present for two months. Upon examination, she was found to have a firm, non-ulcerated nodule measuring about 2.5cm at the palatal junction. Incisional biopsy was recommended because the clinical differential diagnosis was mucoepidermoid carcinoma or squamous cell carcinoma. Anatomopathological examination revealed squamous metaplasia of the salivary gland ducts with preservation of the lobular architecture. Immunohistochemistry showed metaplastic ducts with low reactivity for p53 and Ki67, as well as positivity for CK AE1/AE3, CK7, p63, S-100, and SMA. The final diagnosis was necrotizing sialometaplasia. No treatment is required for this disease. Thirty-nine days after biopsy, total remission was observed with no signs of relapse after two years.

Acrokeratoelastoidosis: is there an association between asthma and sporadic cases in children?

Acrokeratoelastoidosis (AKE) is a rare, benign papular keratoderma that presents as keratotic papules on the lateral margins of the palms and soles. It is most commonly inherited in an autosomal dominant fashion, although sporadic cases are also described. We present a sporadic case of AKE in an 11-year-old girl with a past medical history significant for asthma. On literature review, we found three other cases presenting in children with a past medical history of asthma. We suggest a possible association between asthma and sporadic cases of AKE in children. Current understanding of the pathophysiology of AKE and its associated risk factors is limited and no effective treatment exists. Awareness of a possible association with asthma and atopy, careful history recording in young patients presenting with sporadic cases of AKE, and further research may help to delineate the likelihood of an association between AKE and asthma or atopy. Developing a better understanding of the associated factors that may contribute to the disease process may help guide more effective, targeted treatments in the future.

Clinical resolution of generalized lichen amyloidosis with dupilumab: a new alternative therapy

Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis characterized by deposition of amyloid protein in the skin without visceral involvement. Although it is usually limited to localized areas of the body, it rarely can present in a generalized fashion and is severely pruritic. The limited form is treated with skin directed therapies such as topical or intralesional corticosteroids or topical tacrolimus but the generalized type is more difficult to treat. We present a patient with generalized primary cutaneous lichen amyloidosis successfully treated with dupilumab.

Photo Vignette

Acquired lymphangiectasia: a rare mimic of genital warts

Acquired lymphangiectasia of the vulva is very uncommon. Owing to the non-specific papillomatous manifestation and the vast array of possible differential diagnoses, lymphangioma circumscriptum (LC) still presents a diagnostic challenge. In this report, we present a very rare form of acquired vulvar LC in a 71-year-old patient with a longstanding history of asymptomatic lesions over the labia majora that had been previously treated as genital warts. On examination, the patient had multiple clustered translucent papules up to 15mm in diameter, morphologically reminiscent of vesicles, that oozed clear fluid throughout her groin and swollen labia majora. The patient also suffered concomitant bilateral lower-extremity lymphedema. A skin biopsy showed multiple, irregular-shaped lumina containing eosinophilic material in the upper dermis. Dilated lymphatic channels were lined by a single layer of flattened endothelial cells and the overlying epidermis showed acanthosis, hyperkeratosis, focal mild pseudoepitheliomatous hyperplasia. There is still no consensus on the optimal management of LC. Our patient was referred to a plastic surgeon for further evaluation and treatment. Although there are a variety of therapeutic modalities for LC, positive results are few and relapses are observed.

Lichenoid drug eruption after treatment with ixekizumab for plaque psoriasis

Lichen Planus (LP), the prototype of lichenoid dermatoses, is an idiopathic, T cell-mediated, autoimmune, inflammatory disease. It may affect the skin, hair, nails, and mucous membranes. Many clinical variants of LP have been described, including lichenoid drug eruption or drug induced LP, associated with a myriad of culprit medications. We describe a 63-year-old woman with longstanding psoriasis effectively controlled with ixekizumab, who developed lichenoid drug eruption . Her lichen planus lesions improved after treatment discontinuation and the patient was started on an IL23 inhibitor to treat her psoriasis through an alternative mechanism of action. Our report adds to the literature and provides insight into the complex pathophysiology of lichen planus.