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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 6, 2020

Original

Impact factor of major dermatology journals and the increasing influence of dermatology in the house of medicine.

Background: Journal impact factor (JIF) is a bibliometric proxy of relative journal importance. Mean dermatology JIF has nearly doubled since 1997. The reasons behind the increase have not been previously explored. Objective: To assess factors contributing to rising dermatology JIF. Methods: This bibliometric study utilized publicly-available citation and JIF data from the Thomson-Reuters InCites Journal Citation Reports "Dermatology and Venereology" category, from 1997-2017. Results: From 1997-2017, aggregate dermatology JIF increased by 70%, associated with a 64% increase in JIF numerator (total journal citations) and a 3% decrease in JIF denominator (total journal articles and reviews). In the four highest-JIF journals (JAAD, JAMA Dermatology/Archives of Dermatology, JID, and BJD), there was an increase in citations coming from non-dermatology specialty journals, including oncology, rheumatology, and multidisciplinary sciences. Journal impact factor was positively correlated with five JIF alternatives. Immediacy Index, a reflection of how fast dermatology journals are cited, increased four-fold (P<0.001). Limitations: Impact factor numerator/denominator data were not available before 1999. Conclusions: The nearly two-fold rise in dermatology JIF from 1997-2017 was associated with increased citations, an increasing proportion of which came from non-dermatology journals. This may reflect growing influence of dermatology research within both dermatology and other fields of medicine.

Commentary

Is radiation therapy as a primary treatment modality for squamous cell carcinoma of the hand the best choice? Case series and review of the literature

Background: Squamous cell carcinoma (SCC) is the most common malignancy of the upper limb, and can pose a significant therapeutic challenge. Early treatment needs to be radical whilst maintaining function. Methods: We describe two cases of upper limb squamous cell carcinoma treated with radiotherapy, review the literature on radiotherapy as a primary treatment modality, and discuss the specific difficulties treating SCC in the hand. Results: Radiation therapy was inadequate in tumor clearance in both cases, with recurrence both extensive and distal to the initial focus. Moreover, both patients developed progressive functional loss related to pain, swelling, and stiffness. Conclusion: The evidence basis for radiation therapy as a primary modality is limited, although clearance rates are comparable to surgery. Both radiotherapy and surgery can be utilized to treat SCC. However, we make the case for the hand being especially susceptible to the unwanted side effects of radiotherapy.

A case of vancomycin-induced drug rash with eosinophilia and systemic symptoms (DRESS) syndrome with failure to respond to cyclosporine treatment

Drug rash with eosinophilia and systemic symptoms (DRESS) is a rare delayed drug reaction that often occurs 2-6 weeks after initiation of therapy and may develop into a life-threatening systemic reaction. Besides immediate discontinuation of the suspected inciting drug, initiation of high dose systemic corticosteroids has long been the mainstay of treatment for severe cases. Nevertheless, significant drawbacks associated with systemic corticosteroid therapy, such as the requirement of a long tapering period post resolution and extensive adverse side effects profile, have motivated clinicians to seek alternative treatment options. Over the past decade, an undisputed increasing number of favorable case reports has highlighted cyclosporine as an emerging, safe, and effective alternative despite inconsistent dosing regimens reported. Herein, we report a severe case of vancomycin-induced DRESS syndrome in which the patient failed initial intervention with cyclosporine and needed rescue with methylprednisolone. To the best of our knowledge, this constitutes the first unsuccessful report of cyclosporine treatment for DRESS syndrome.

Case Report

Cutaneous Legionella infections in allogeneic hematopoietic cell transplantation recipients

To date, only twenty cases of cutaneous legionellosis have been reported. Cutaneous legionellosis has heterogeneous manifestations including abscesses, nodules, and cellulitis. The detection of most cutaneous Legionella species requires specific diagnostic cultures and assays. Herein, we report a case of cutaneous legionella in a hematopoietic cell transplantation recipient with culture-negative nodules unresponsive to empiric antibiotics. We also discuss the varied morphology of cutaneous legionellosis and important diagnostic considerations.

Calcinosis cutis arising in morphea: a case series

Calcinosis cutis, although common in systemic sclerosis, has been rarely reported in patients with morphea. We describe four patients with calcinosis cutis arising within morphea plaques, discuss their treatments and outcomes, and review previously published cases. Current management recommendations for concomitant morphea and dystrophic calcinosis cutis are based on limited data and expert opinion, which has primarily focused on reduction of active inflammation and reduction of symptoms related to calcinosis or ulceration. In most cases, no improvement of calcinosis was noted. The use of intralesional corticosteroids to active lesions in conjunction with systemic treatment, including methotrexate when indicated, appear promising treatments to halt progression of the disease. Surgical excision seems to be the most definitive treatment for calcinosis affecting morphea plaques, but the current literature lacks details regarding disease recurrence following operative management.

Morbihan disease: a case report and differentiation from Melkersson-Rosenthal syndrome

We present a 32-year old woman with a 9-year history of upper facial swelling. A workup by the ophthalmology department led to the diagnosis of Melkersson-Rosenthal syndrome. Re-evaluation in our dermatology clinic confirmed a diagnosis of Morbihan disease. Herein, we review case reports and case series of upper facial swelling in the dermatologic and ophthalmologic literature. Although the two entities share histopathological changes, they tend to have different clinical presentations. Melkersson-Rosenthal syndrome appears to be more likely diagnosed in the ophthalmologic literature when the clinical presentation and histopathology may be more consistent with Morbihan disease. In a patient with upper facial swelling, an absence of orolabial swelling, and lack of facial neuropathy, we argue for a diagnosis of Morbihan disease over Melkersson-Rosenthal syndrome, especially if the patient has a history of rosacea.

Case Presentation

Eruption of squamous cell carcinomas after beginning nilotinib therapy

Chronic myelogenous leukemia (CML) is characterized by a reciprocal translocation between the long arms of chromosomes 9 and 22 leading to the formation of a constitutively active tyrosine kinase. Tyrosine kinase inhibitors (TKIs) are the treatment of choice for patients diagnosed with CML and have many associated side effects including the rarely-reported eruption of squamous cell carcinomas (SCCs). Herein, we report a patient with CML who presented with sudden onset of multiple scaly lesions on his legs and trunk after beginning treatment with nilotinib, a novel TKI. Six biopsies were performed at his initial presentation and four of these lesions were confirmed to be keratoacanthoma-type SCCs. One month later, the patient reported the development of multiple new similar lesions on his legs, arms, and face. Four more biopsies were performed revealing keratoacanthoma-type and well-differentiated SCCs. Certain tyrosine kinase inhibitors such as sorafenib and quizartinib have been reported to cause eruptive keratoacanthoma (KA)-type SCCs as seen in our patient. However, there is only one other report in the literature of nilotinib promoting the development of SCCs or KAs. Physicians should be aware of this potential adverse effect and patients taking nilotinib should be closely monitored by a dermatologist.

Nevoid melanoma and eruptive nevi from erlotinib

Cutaneous side effects such as acneiform eruption, xerosis, and paronychia are frequently observed in patients undergoing treatment with epidermal growth factor receptor (EGFR) inhibitors for non-small cell lung cancer and other solid tumors. Interestingly, these side effects appear to positively correlate with length of remission, indicating that disruption of homeostatic EGFR signaling in the skin may serve as a marker of therapeutic EGFR inhibition in tumors. We report the case of a woman with metastatic lung cancer in remission being treated with the EGFR inhibitor, erlotinib, who experienced numerous commonly occurring adverse cutaneous reactions early in her treatment, and after two years of treatment developed eruptive nevi as well as a nevoid melanoma. Changes in pigmented lesions and the development of melanoma have been described during treatment with the BRAF inhibitor, vemurafenib, and are believed to relate to paradoxical activation of BRAF and the MAPK pathway. We speculate that a similar mechanism may occur during treatment with EGFR inhibitors. Therefore, thorough skin examinations are essential for patients undergoing long term treatment with erlotinib.

Red puffy hand syndrome mistaken for inflammatory arthritis

Red puffy hand syndrome is an uncommon clinical manifestation of intravenous drug abuse, which presents with bilateral, painless and non-pitting erythema and edema of the dorsal hands. The pathophysiology is believed to primarily be the result of lymphatic blockage from either direct toxicity of the injected drug, drainage of impurities, or infection complications. A woman in her 40's with remote intravenous drug use presented with over a decade of fixed, painless erythema and swelling of bilateral dorsal hands. Owing to an elevated rheumatoid factor, which would later be attributed to patient's untreated hepatitis C, these findings were mistaken for rheumatoid arthritis and unnecessarily treated with methotrexate and prednisone. Upon proper recognition of her underlying Red puffy hand syndrome, systemic medications were discontinued and appropriate care was initiated with lymphedema decongestion and occupational therapy. Red puffy hand syndrome, albeit rare, is an important manifestation of intravenous drug abuse; its recognition will spare patients from unnecessary systemic treatments.

A perplexing case of superficial granulomatous pyoderma with sporotrichoid-like distribution

Superficial granulomatous pyoderma (SGP) is a rare pyoderma gangrenosum (PG) variant that differs from classic PG in that the ulcers tend to be more superficial, lack a rapidly advancing border, and are not typically associated with an underlying systemic disease. The ulcers are most commonly painless and located on the trunk, with a clean granulating base. They generally do not show undermining but may have a vegetative border. Lesions usually respond well to either topical or intralesional corticosteroids with complete healing. The classic histopathologic finding is a "three-layer granuloma" in the superficial dermis consisting of central neutrophilic inflammation and necrosis, a surrounding layer of histiocytes and multinucleated giant cells, and an outer most layer of plasma cells and eosinophils. Herein, we present a unique case of SGP with sporotrichoid-like distribution on the lower extremity.

An unusual presentation of localized bullous morphea

Bullous morphea is a rare variant of localized scleroderma characterized by occasional intermittent blisters. Lichen sclerosus is a chronic inflammatory disease. The coexistence of morphea and lichen sclerosus has been reported in different sites in the same patient and more rarely in the same lesion. We report the case of a 54-year-old woman with an atypical presentation of bullous morphea and some histological features of lichen sclerosus. She presented with a 5-year history of an ulcerated plaque, with a sclerotic and atrophic center and indurated budding margins, localized on the lumbar back. Initially the diagnosis of a squamous cell carcinoma was suggested. A skin biopsy confirmed the diagnosis of bullous morphea and showed some histological features of lichen sclerosus. Topical betamethasone and silicone gel ointment were prescribed leading to complete healing of the ulceration within five months. Our case is unusual because of the atypical clinical presentation, the histological aspect combining signs of bullous morphea and lichen sclerosus, and the favorable results with the use of local corticotherapy and silicone gel.

Pemphigus foliaceus in a patient with gastrointestinal stromal tumor treated with adjuvant imatinib mesylate

Pemphigus is an autoimmune bullous disease with a number of described associations, including medications, which have been grouped into three structural categories - thiol drugs, phenol drugs, and drugs with neither functional group [1]. Discontinuation of the offending medication is considered a mainstay of therapy. We report a patient in whom the onset of pemphigus foliaceus was associated with initiation of imatinib mesylate adjuvant therapy in a patient with resected gastrointestinal stromal tumor (GIST). Imatinib was continued because of the survival benefit to the patient with a resected, high risk GIST. Treatment with rituximab resulted in near resolution of his blistering rash and follow up enzyme-linked immunosorbent assay (ELISA) demonstrated reference range immunoreactivity for both desmoglein 1 and desmoglein 3. After dose increase of imatinib therapy owing to tumor growth, the patient subsequently again developed a similar eruption. Re-biopsy and ELISA were consistent with recurrence of pemphigus. In conclusion, although the patient's pemphigus was cleared with a single cycle of rituximab infusions while continuing imatinib therapy, the disease returned after imatinib dose was increased a year later, suggesting a dose-response relationship.

Photo Vignette

Umbilicated papules on the bilateral upper and lower extremities

Cryptococcosis is a rare opportunistic infection with morphologically diverse cutaneous presentations. Primary infection typically occurs in the lungs with subsequent hematogenous dissemination to other organ systems, especially in immunocompromised patients. Herein, we report a woman in her 70's who presented with pruritic, umbilicated papulonodules of the bilateral upper and lower extremities present for many weeks. She was diagnosed with disseminated Cryptococcus and subsequently evaluated for potential pulmonary and meningeal disease involvement. She died as a result of multiple medical comorbidities.

Cutis marmorata telangiectasia congenita with painful ulcerations

Cutis marmorata telangiectatica congenita (CMTC) is a rare, congenital, vascular disorder that may sometimes be associated with ulcerations of the involved skin. We present a case of CMTC, asymptomatic since birth, that began developing painful ulcerations during adolescence. Although laser therapy may benefit the superficial aspect of this vascular anomaly, the presence of deeper involvement in lesions with ulcerations may not respond favorably to laser therapy and the best approach needs to be further evaluated.

Porokeratosis ptychotropica: a rare variant that is commonly misdiagnosed

Porokeratosis ptychotropica (PP) is a rare variant of porokeratosis characterized by pruritic, scaly papules and plaques localized to the perianal and gluteal cleft regions. Clinically, PP resembles other common disorders, resulting in frequent misdiagnosis. The diagnosis of PP often takes several years to make, therefore many reported cases in the literature described the late stages of PP. We report a case of PP diagnosed at an early stage. By presenting our patient, we aim to raise further awareness of PP to avoid a delay in diagnosis, thus preventing long term complications of this rare entity.

Letter

What's in a name? a new nomenclature has been proposed For eosinophilic dermatosis of hematologic malignancy (EDHM): hematologic-related malignancy-induced eosinophilic dermatosis (He Remained)

Hematologic-related malignancy-induced eosinophilic dermatosis (He Remained) has recently been introduced as a new nomenclature to describe the eosinophilic dermatosis that has previously been observed in patients with hematologic malignancies. The condition has been reported in 208 patients; the ratio of men to women is 1.3:1. It is most commonly observed in chronic lymphocytic leukemia patients (77%, 160/208 patients). The chronic and relapsing eosinophilic dermatosis typically presents with pruritic lesions that are pleomorphic in morphology and mimic other conditions. The definitive pathogenesis of He Remained is still being established. However, neoplastic leukemia B cells - directly or indirectly (by stimulating a reactive polyclonal T cell response) - likely have an etiologic role in the pathogenesis of this condition in chronic lymphocytic leukemia patients. In addition, recruitment of eosinophils to the skin may occur secondary to an immune shift toward a T helper 2 type response, possibly caused by the neoplastic cells, that results in these T cells producing interleukin 4. Clinical observations, currently based on the prompt (within four weeks) and sustained (at least 12 weeks to 6 months) resolution of He Remained in two elderly men with He Remained, suggests that dupilumab may be the treatment of choice in chronic lymphocytic leukemia patients with this condition.

Social media and dissemination: impressions of fish pedicures following a case of onychomadesis

Although the popularity of fish pedicures as cosmetic or routine services and/or tourist attractions has peaked in the past decade, the practice is still trending today and accompanying risks are less well-known by the public. More recently, a case of onychomadesis following fish pedicure has gained interest in both public and dermatologic populations, highlighting the importance of patient education. In this study, we aimed to demonstrate the wide reach of the manuscript through social media and news outlets. The majority of social media correspondences have come from Thai accounts whereas news outlets were mostly from the United States and various European or Asian countries. Analyzed by Altmetric score, the report was within the top 5% of all research outputs ever scored by Altmetric. Finally, Google Trend data indicates that fish pedicure interest decreased thereafter to the lowest levels in the past decade after the case report was published, signifying its likely effect on diminishing public interest. This case report and analysis of its news/social media reach emphasize the importance of literature/media outlets in informing patients and the public of adverse effects. As fish pedicures continue to be popular both in the US and overseas, appropriate education of the public remains important in preventing complications.

Corrigendum

Corrigendum: Successful non-operative treatment of eruptive keratoacanthomas refractory to excision

The original article was published on March 15, 2020 and corrected on June 15, 2020. The revised version of the article corrects an Author's name. The changes appear in the revised online PDF copy of this article.