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Open Access Publications from the University of California

About

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 25, Issue 11, 2019

Review

Systemic 5-fluorouracil induced lupus erythematosus: a review of the literature

Drug-induced subacute cutaneous lupus erythematosus (SCLE) is the most common subtype of drug-induced systemic lupus erythematosus and has been associated with more than 100 drugs. It presents weeks to months after initiation of the culprit medication. The eruption is typically in a photodistribution and it is marked by positive serology to anti-Ro (SSA) antibody. Systemic 5-fluorouracil (5-FU) is a less-common culprit of drug-induced SCLE and its occurrence is likely dependent on exposure to ultraviolet light. Herein, we present a review of drug-induced lupus induced by the pyrimidine analog, 5-FU, and its prodrugs, capecitabine and uracil-tegafur. The search was carried out using the following terms: (PubMed: keywords included drug-induced lupus, 5-fluorouracil, subacute cutaneous lupus erythematosus, capecitabine, uracil-tegafur, discoid lupus, systemic lupus erythematosus).

Original

Melanoma in individuals with neurofibromatosis type 1: a retrospective study

Background: Neurofibromatosis type 1 (NF1) is a cancer syndrome associated with many different cancer types. There are limited studies examining melanoma risk in this population. Objective: To identify melanoma cases in NF1 patients and compare melanoma incidence rates relative to a general population sample. Methods: A retrospective single institution case review of 857 NF1 patients (seen from 7/1997 to 7/2017) was conducted. We calculated age- and calendar period-adjusted standardized incidence ratios (SIRs) for white patients >20 years old overall (N=282) and for females (N=156) at their last visit date. We obtained general population melanoma reference rates from the Surveillance, Epidemiology, and End Results (SEER) 9 database. Results: Among 857 patients, 52.2% were female, 54% were <20 (mean±sd=10.9±4.6) years old, and 46% were >20 (40.4±14.9) years old at their last visit date. One white female patient had a malignant melanoma diagnosed at 47 years old. The adjusted SIR was 0.97 (95% CI 0.05-4.78) overall (N=282) and 1.62 (95% CI 0.08-7.98) for females (N=156). Conclusions: We did not find statistical evidence for an increased melanoma risk in adults with NF1. However, additional large studies are warranted to clarify whether melanoma risk is increased in NF1 patients.

Commentary

Implementation of a patient-assisted teledermatology model in the Veteran Health Administration

Owing to the inherently visual nature of the field of dermatology, advances in imaging and communication technology have resulted in wide-spread application of teledermatology since its introduction in the mid-1990s. In the last 20 years, studies have repetitively shown that teledermatology provides effective and efficient quality care for patients. It also increases access to underserved patients and reduces traveling costs, wait times, and unnecessary referrals. In this letter the authors seek to analyze implementation of a direct patient to dermatologist model in a Veteran Health Administration (VHA) patient population, referred to as patient-assisted teledermatology. This population is largely over the age of 65 and a significant portion are either without internet or have the minimum technology necessary to participate in the studied model. Owing to these observations and personal experiences, the authors found the implementation process of a patient-assisted model to be challenging in this population.

Diagnosis of cutaneous T-cell lymphoma by insurance type before and after the Affordable Care Act: a national database study

The Affordable Care Act (ACT) was implemented to increase health care access and reduce the uninsured in the age group between pediatric and Medicare populations (18-64). The association of the ACA with insurance type upon diagnosis (uninsured, Medicaid, non-Medicaid) has been investigated for otolaryngologic, gynecologic, and the top five non-skin malignancies. Such studies for cutaneous malignancies are lacking. We conducted a retrospective analysis of the prospective National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) cancer database to assess the impact of the ACA on new diagnoses of cutaneous T-cell lymphoma (CTCL) by insurance type. Unlike prior studies of other malignancies, we did not observe significant differences between rate of diagnosis of CTCL by insurance type before and after full implementation of the ACA in all states, expansion states, and non-expansion states. Skin cancers do not have screening guidelines and CTCL is an uncommon malignancy, both of which may contribute to these findings. However, Medicaid-expansion states were much closer to reducing the percentage of newly diagnosed uninsured patients with CTCL than non-expansion states. As such, it may be prudent to investigate intrinsic socioeconomic barriers to care in Medicaid patients to improve their access to care to decrease the uninsured population and improve outcomes.

Case Report

Pleomorphic dermal sarcoma in a man with HIV: report with next-generation sequencing analysis and review of the atypical fibroxanthoma/pleomorphic dermal sarcoma spectrum

Atypical fibroxanthoma (AFX) is a rare cutaneous fibrohistiocytic tumor that typically arises on chronically sun-damaged skin, such as the head and neck, as a nondescript ulcerated papule, nodule, or tumor. The clinical prognosis is usually favorable and metastasis is rare. Pleomorphic dermal sarcoma (PDS), or undifferentiated pleomorphic sarcoma, is a recently introduced diagnostic moniker for AFX-like tumors with more aggressive clinical and histologic features such as necrosis and vascular invasion. The exact relationship between AFX and PDS has been debated. Diagnosis of these tumors is generally based on immunohistochemical staining to exclude other mimics. A wholly specific marker for this tumor does not exist, leading to diagnostic ambiguity in certain cases. Herein, we present a case of pleomorphic dermal sarcoma in a 53-year-old man with human immunodeficiency virus that displayed patchy S100 staining concerning for melanoma upon hospital pathology review. Next-generation sequencing analysis confirmed a mutation pattern consistent with published molecular signatures of AFX/PDS. In discussing this case, we review the current understanding of AFX/PDS and discuss diagnostic pitfalls, as well as emphasize on how next-generation sequencing techniques might improve accuracy in the diagnosis of tumors in the spectrum of AFX/PDS.

Case Presentation

Radiation induced lichen planus - an uncommon side effect

Cutaneous lichen planus is classically characterized by violaceous, pruritic, planar papules and plaques, most commonly affecting the extremities. Lichen planus following radiation therapy is extremely rare and lichen planus following radiation therapy for prostate carcinoma has not been previously reported in the literature. We report a 66-year-old man who presented to the dermatology clinic with a symmetric pruritic eruption affecting the pelvic and gluteal region within two months of radiation therapy targeting the prostate and pelvic lymph nodes for prostate adenocarcinoma. The patient did not have a prior history of lichen planus. Physical examination demonstrated well demarcated, violaceous papules and plaques in a circumferential band-like distribution on the bilateral gluteal, lumbosacral, and pelvic region. In addition, he had a few discrete lesions on the calves and dorsal feet. Punch biopsy revealed an acanthotic epidermis with "saw-tooth" rete ridges and a lichenoid inflammatory infiltrate. A diagnosis of hypertrophic lichen planus was made, reinforcing the importance for clinicians to recognize radiation therapy as a risk factor for developing lichen planus despite no prior history of lichen planus.

Molluscum contagiosum infection with features of primary cutaneous anaplastic large cell lymphoma

CD30+ T cell pseudolymphomas (CD30+ PSL) are a group of benign inflammatory cutaneous disorders that can develop in settings of viral infections or drug reactions. Owing to their histological similarities to malignant lymphomas, these benign infiltrates are occasionally misdiagnosed as malignant, causing significant concerns for patients and physicians. Herein, we report a patient with CD30+ PSL associated with molluscum contagiosum whose initial biopsy revealed atypical large CD30-expressing cells, leading to a misdiagnosis of primary cutaneous anaplastic large cell lymphoma and referral to our cutaneous lymphoma clinic. We report this case to demonstrate that reactive CD30+ infiltrate associated with molluscum contagiosum can be mistaken for T-cell lymphomas and patients should be reassured in these cases.

Gorlin syndrome in a patient with skin type VI

Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant disorder that is characterized by multiple basal cell carcinomas developing at a young age, keratocystic odontogenic tumors of the jaw, palmar or plantar pits, calcification of the falx cerebri, and skeletal abnormalities. Nevoid basal cell carcinoma syndrome is caused by mutations in the PTCH1 or SUFU genes. Our patient with Fitzpatrick skin type VI was diagnosed with Gorlin syndrome based on the presentation of multiple major diagnostic characteristics. Although he is 33 years old, he has not developed any multiple basal cell carcinomas to date.

Photo Vignette

Unilateral hyperhidrosis secondary to brainstem meningioma producing mass effect

Unilateral hyperhidrosis of neurological origin has been associated with head trauma, cerebral palsy, spinal cord injury, peripheral neuropathy, lesions of the hypothalamus, and cerebral or brainstem strokes. In this report, we describe a 61-year-old man with isolated sweating on the left side of his entire body. A right-sided brainstem meningioma producing mass effect is suspected as the underlying etiology.

Neonatal sucking blister

Neonatal sucking blisters result from vigorous sucking on hand or forearm in utero. Clinically, one observes a tense, fluid-filled blister, which when ruptured forms an erosion. We report a female neonate with a sucking blister on the distal dorsal aspect of her left forearm. These benign bullae should be differentiated from other diseases of the newborn through their presentation, characteristic morphology, and self-limiting course.

Eccrine poromatosis following chemotherapy and radiation therapy

Eccrine poroma presents as a single, symptomless erythematous papule in areas with a high density of eccrine sweat glands. Although rare, eccrine poromas can present as multiple lesions, otherwise known as eccrine poromatosis. The etiology of eccrine poromatosis is unclear. We present two cases of eccrine poromatosis in patients who had undergone chemotherapy, radiation therapy, and stem cell transplant. This case report serves to raise awareness of this condition and highlight its association with malignancies and their treatment.

Letter

Dupilumab for bullous pemphigoid with intractable pruritus

Bullous pemphigoid (BP) is an autoimmune blistering disorder that predominantly affects the elderly. Treatment regimens typically include topical and systemic immunosuppressive medications. Although effective, systemic corticosteroids are sometimes poorly tolerated in the elderly patient, contributing to the overall morbidity and mortality of BP. Dupilumab is a monoclonal antibody targeting interleukin 4 receptor alpha (IL4R?), approved for the treatment of atopic dermatitis, as well as moderate to severe asthma and chronic rhinosinusitis with nasal polyposis. In recent reports, dupilumab has been successfully used off-label to treat a variety of pruritic disorders, including chronic spontaneous urticaria [1], anal and genital itch [2], allergic contact dermatitis [3], and prurigo nodularis [4, 5]. We report here a case of an elderly patient with refractory BP whose symptoms of pruritus and blistering became well-controlled with the addition of dupilumab to the treatment regimen.

Topical timolol for treatment of persistent granulation tissue in the setting of severe hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a potentially debilitating dermatological disease that negatively impacts patients' quality of life. Severe cases can be further complicated by persistent granulation tissue at the ostia of sinus tracts, which may prove recalcitrant to standard interventions. Herein we report such a case in which a patient experienced significant improvement from severe HS but was left with persistent granulation tissue that complicated his course of recovery. When standard interventions failed, we elected to begin treatment with topical timolol. After three months, the majority of the granulation tissue had regressed and has remained quiescent after 12 months of follow up. The patient has tolerated the treatment well and continues to use topical timolol daily as needed for flares. We believe that topical timolol can provide a practical and painless alternative to current invasive and expensive therapies for persistent granulation tissue associated with severe HS.

Bilateral keratoprostheses in a patient with ocular mucous membrane pemphigoid.

Mucous membrane pemphigoid (MMP) with ocular involvement, also referred to as ocular cicatricial pemphigoid, is a rare, autoimmune blistering disease that can have devastating effects for patients. The resulting corneal neovascularization, recurrent corneal abrasions, and ulceration can ultimately result in severe vision loss. We present a 64-year-old man with recalcitrant ocular MMP and consequently advanced corneal involvement that received bilateral keratoprostheses. This case highlights an aggressive but successful placement of bilateral keratoprostheses in recalcitrant ocular MMP, which restored his vision and ultimately his quality of life.

Multidermatomal herpes zoster: a pain in the neck?

Herpes zoster classically presents as a vesicular eruption along a single dermatome that correlates with the dorsal root ganglion in which varicella zoster virus (VZV) reactivates. Such cases most commonly involve a single thoracic dermatome, but other rare presentations of herpes zoster have been reported including multidermatomal herpes zoster. This letter reports a case of multidermatomal herpes zoster affecting cervical dermatomes C2-C5 and presents all previously published cases of multidermatomal herpes zoster in which involved dermatomes were reported to determine if this condition has a predilection for cervical dermatomes. A total of eight other cases were reviewed and involvement of cervical dermatomes was observed in 6 of 9 cases (66.7%). This suggests a propensity for multidermatomal involvement to affect cervical dermatomes beyond that encountered in classic herpes zoster. Clinicians should be aware of this presentation of herpes zoster especially in the head and neck region where the classic vesicular eruption may not be confined to a single dermatome.

Presence of arthralgia exacerbates decreased quality of life in hidradenitis suppurativa patients

Hidradenitis suppurativa is characterized by chronic follicular occlusion that presents with recurrent nodules, inflamed abscesses, and scarring. Research has shown that these patients have a decreased quality of life. In addition to its psychosocial effects, hidradenitis suppurativa has recently been associated with joint pathology. In this study, we distributed a survey consisting of the Short Form 12 Health Survey, used for assessing health outcomes, along with additional questions about joint pain to an online hidradenitis suppurativa support group in order to understand the effect of comorbid arthralgia on quality of life in this disease. The respondents in this study had significantly reduced physical health composite scores-12 (PCS-12), (35.8 versus 50, P<0.001) and mental health composite scores-12 (MCS-12), (33.7 versus 50, P<0.001) scores compared to the general population. Additionally, patients reporting severe arthralgia had significantly lower PCS-12 (32.3 versus 36.5; P<0.05) and MCS-12 (33.3 versus 40.5; P<0.001) scores compared to those with mild arthralgia. Despite the effect of comorbid arthralgia on quality of life, only 11% reported having been asked about joint pain by their dermatologist. Routine screening questions concerning associated arthralgia and diminished quality of life may be helpful during clinician assessment and treatment of hidradenitis suppurativa patients.

Corrigendum

Corrigendum: Systemic contact dermatitis related to alcoholic beverage consumption

The original article was published on September 15, 2019 and corrected on November 15, 2019.The revised version of the article adds a missing author. This change appears in the revised online PDF copy of this article.