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Open Access Publications from the University of California

Dermatology Online Journal

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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 27, Issue 8, 2021


Morgellons disease etiology and therapeutic approach: a systematic review

Morgellons disease is characterized by patient reports of fibers embedded in and protruding from the skin. Etiologies from infection to delusion have been endorsed, and treatment guidelines are not well-defined. The objective of this manuscript is to evaluate the existing evidence regarding the etiology and treatment of Morgellons disease in an effort to better inform clinical management. A PubMed search including key words "Morgellons," "delusional parasitosis and fibers," "delusions of parasitosis and fibers," or "delusional infestation and fibers" was completed. Original publications directly assessing etiology or treatment methods of Morgellons disease published between January, 2010 and the time of manuscript preparation were reviewed and evaluated. Sixteen articles regarding etiology were reviewed. All studies were correlative in nature with various limitations. Support for a psychiatric etiology was more widespread than support for an infectious etiology. Eleven articles regarding treatment efficacy were reviewed. Antipsychotic regimens have the most evidence of efficacy. Existing data regarding Morgellons disease suggests a psychiatric etiology and supports treatment with a low-dose antipsychotic agent once non-psychiatric causes have been excluded.

Clinical recommendations to address dermatologic healthcare disparities in sexual and gender minority patients: a review

Background: In the United States, an estimated 4.5% of the population identifies as a sexual or gender minority (SGM). Efforts are underway to address this population's healthcare disparities. [A1] Objective: This review aims to highlight dermatologist's role in treating SGM patients, raise awareness about SGM-related stigma, and identify clinical interventions to improve SGM care. Methods: Articles were selected by review of literature from PubMed's database from 2000-2020. Results: The first intervention outlines methods to educate the healthcare team on the terminology used by the SGM community and how HIV epidemiology is a distinct topic through separate trainings. The second intervention emphasizes better communication with SGM patients in routine discussions, including the proper elicitation of a sexual history by avoiding heteronormative questioning. The last intervention discusses enhancing this population's clinical experience by updating clinical intake forms to include a fill-in-the-blank for patients' pronouns, refraining from gender-specific bathrooms, and advertising commitment to SGM care online. Conclusion: Our review article highlights a dermatologist's integral role in SGM care. The review emphasizes three distinct intervention areas that aim to destigmatize sexual/gender identity in the workplace, promote cultural humility, and improve the therapeutic alliance between SGM patients with dermatologists.


Competing risks analysis of Merkel cell carcinoma with concurrent chronic lymphocytic leukemia and non-Hodgkin lymphoma

Background: Although hematogenous malignancy is a risk factor for poorer prognosis in Merkel cell carcinoma (MCC), current guidelines make no specific recommendations for surveillance. Objective: We aim to characterize MCC-specific mortality compared to other causes of death for patients with hematologic malignancy in MCC, which will guide workup and surveillance strategies. Methods: The Surveillance, Epidemiology, and End Results-18 registry was queried for MCC patients with chronic lymphocytic leukemia (CLL) or non-Hodgkin lymphoma (NHL). Results: Of 8519 patients with MCC, 146 (1.7%) had CLL and 234 (2.8%) had NHL. Chronic lymphocytic leukemia patients had 5-year cumulative incidence of MCC-specific mortality of 38.4% versus 28.4% in patients without CLL/NHL. For both cohorts, oncologic risk was highest within the first three years of diagnosis with competing risks favored thereafter. On competing risk regression, a history of CLL trended toward statistical significance with poorer MCC-specific mortality (subdistribution hazard ratio: 1.33, 95% CI: 0.963-1.834, P=0.084), while NHL was not prognostic. Conclusions: Merkel cell carcinoma patients with CLL may benefit from more aggressive initial management. Surveillance for similar length in CLL patients with MCC may be appropriate; this co-morbidity did not affect the timeframe by which the risk of competing causes of death exceeded oncologic risks.

The continued inappropriate use and overuse of combination topical clotrimazole-betamethasone

Objective: Combination topical clotrimazole/ betamethasone dipropionate (C-BM) contains a high-potency topical corticosteroid and is not infrequently prescribed for inappropriate patient groups and body sites. Use of C-BM can lead to inadequate clearance or exacerbation of fungal infections as well as cutaneous atrophy, striae, and other skin maladies. Methods: We performed a retrospective chart review of 1,978 clinical visits where C-BM was prescribed within the University of Utah Health system between 2014 and 2018 to better understand current prescribing patterns. Results: 1,974 prescriptions were written for C-BM. 91.6% of patients were at least the recommended age of 17 years. C-BM was most commonly prescribed for rashes of an inflammatory (42.2%) or fungal nature (38.1%). Clotrimazole/betamethasone dipropionate was prescribed for sensitive areas (face, axillae, groin or diaper region) in 48.9% of patients. Family medicine clinicians prescribed 58.3% of C-BM prescriptions, whereas dermatology clinicians accounted for 3.4%. Conclusion: We strongly recommend clinicians use alternative treatments for rashes or refer to dermatologists.

Incidence estimates for lichen planopilaris and frontal fibrosing alopecia in a New York City health care system

Lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are scarring alopecias that cause significant distress and psychological morbidity. Limited studies have been performed examining the epidemiology of FFA and LPP. We performed a retrospective case cohort analysis by querying for patients with the ICD 10 code L66.1 (LPP, FFA) between 2015 and 2018 using the Clinical Data Warehouse (CDW) at NewYork-Presbyterian Hospital and Columbia Doctors. We calculated the one-year incidence of LPP/FFA between January 1, 2018 to December 31, 2018 by identifying all patients without a previously recorded ICD code for L66.1 who presented as a new hair loss patient based on chart review. A total of 170 patients were identified with a new diagnosis of LPP or FFA in 2018 among 1,187,583 patients. The standardized incidence per 100,000 was 12.75 for LPP and FFA combined, 7.35 for LPP alone, and 5.41 for FFA alone. The incidence peaked in the 51 to 60 age range (3.36). The incidence was highest in non-Hispanic White patients (17.27), White patients of unknown ethnicity (26.26), and non-Hispanic Asian patients (17.27). In New York City, LPP and FFA are uncommon diseases that are most common in middle-aged females and non-Hispanic White patients.

Evaluating the association of central centrifugal cicatricial alopecia (CCCA) and fibroproliferative disorders

Background: In central centrifugal cicatricial alopecia (CCCA), a lymphocytic scarring alopecia that primarily affects black women, it has been postulated that there is a "pro-fibrotic" tendency and increased risk for systemic fibroproliferative disorders. Objective: To determine whether women with biopsy-proven CCCA have a greater likelihood of systemic fibroproliferative disorders (FPDs) of the lungs (interstitial lung disease), arteries (atherosclerosis of the aorta), liver (non-alcoholic steatohepatitis), kidney (end stage renal disease), or uterus (uterine leiomyoma). Methods: We conducted a retrospective matched cohort study evaluating 427 cases with biopsy-proven CCCA and 1281 age- and sex-matched controls. Results: Black women with biopsy-proven CCCA, were not more likely to have interstitial lung disease (ILD), atherosclerosis of the aorta, non-alcoholic steatohepatitis (NASH), end stage renal disease (ESRD), or uterine leiomyoma. Central centrifugal cicatricial alopecia was associated with a history of never smoking and higher body mass index. Conclusion: In this large cohort of biopsy-proven women with CCCA, there was no association with specific fibroproliferative disorders when compared with age and sex matched controls. Future longitudinal studies may help confirm these results.


Characteristics and comorbidities of Medicare beneficiaries receiving care from Mohs micrographic surgeons based on fellowship training and practice setting

The characteristics and medical conditions of patients being managed by Mohs micrographic surgeons (MMS) have not been extensively established. In this cross-sectional review of 2017 Medicare Public Use data, we compared patient demographics and medical comorbidities among dermatologists billing for MMS based on surgeon fellowship training and practice settings. Overall patient complexity, as measured through Medicare's Hierarchical Condition Category (HCC) score, did not significantly differ by fellowship training status. However, among fellowship-trained surgeons, those in academic centers managed a higher proportion of dual Medicare-Medicaid beneficiaries (9.4% versus 5.4%, P<0.0001) with higher mean HCC scores (1.33 versus 1.13, P<0.0001). Depression and chronic kidney disease were notably more common among academic beneficiaries. These findings help to establish the patient complexity distribution among dermatologic surgeons, which may have important implications for perioperative management and monitoring given the growing prevalence of skin cancer and other medical comorbidities.

Case Report

Response of eosinophilic fasciitis associated with Waldenström macroglobulinemia to rituximab

Eosinophilic fasciitis (EF) and generalized morphea (GM) are rare and difficult-to-treat sclerosing skin diseases which may occur in association with hematologic disorders. We present a 66-year-old man with EF and associated Waldenström macroglobulinemia who received combination therapy with rituximab (375mg/m2 every other week, gradually extended to every eight weeks), prednisolone (1.25-30mg/d), and methotrexate (7.5-15mg/w). Three months after rituximab initiation, his skin condition improved steadily accompanied by a significant improvement in joint mobility with only mild and transitory flares (observation period: 59 months under treatment with rituximab). To date, there are five case reports on rituximab treatment of EF/GM with an association to hypergammaglobulinemia in three of those cases. Therapy effected significant improvement in four patients. Our case adds to the hitherto limited evidence that rituximab may be a promising therapeutic strategy for EF/GM in association with hypergammaglobulinemia.

Milia-like calcinosis cutis in Down syndrome: a new case with a review of the literature

We report an 11-year-old girl who presented with white papules on the dorsal and palmar region of the hands bilaterally. The parents reported that the lesions had appeared four months before and some had resolved spontaneously. The girl was suffering from celiac disease, Down syndrome, and alopecia areata treated with topical corticosteroids. At the first visit, the girl presented with alopecia areata, corticosteroid acne, and a dozen white papules located on the hands. On dermoscopy, a whitish structureless area was seen. Histological examination showed the presence of calcium deposits without tissue damage, thus confirming the diagnosis of milia-like idiopathic calcinosis cutis. At 6-month follow up, the lesions had completely disappeared. Milia-like idiopathic calcinosis cutis is a benign cutaneous disorder consisting of calcium deposits in an apparently undamaged dermis and is typically associated with Down syndrome. Up to a quarter of patients have coexisting syringomas. The milia-like papules tend to self-resolve as patients reach adulthood, so a wait-and-see approach is recommended.

Case Presentation

Darier disease, radiation therapy, and herpesvirus -- an unfortunate triad

Darier disease (DD) is a rare autosomal dominant keratinizing disorder often characterized by brown scaly pruritic papules over the face, neck, and trunk. Herein is reported a patient who developed secondary cutaneous herpes simplex virus (HSV) following exacerbation of his DD as a result of radiation therapy. In November 2020, a 78-year-old man presented to clinic for a pruritic rash on his back consistent with DD. He had developed the rash after the conclusion of chemoradiation therapy for recently diagnosed urothelial carcinoma of the bladder with squamous differentiation. However, he returned two weeks later complaining of a marked worsening of the rash associated with a pain and burning sensations. Histopathology was non-conclusive, but the lesions were found to be positive for HSV-1 by PCR. The patient recovered without complication over a period of two weeks following a course of valacyclovir. There is precedent in the literature for ionizing radiation inducing flares of DD lesions in overlying skin. In addition, DD has been shown to put a patient at increased risk for secondary infections such as HSV. This case report demonstrates that HSV could pose a significant risk to those with DD receiving radiation therapy and thus could warrant prophylactic treatment.

Angiokeratoma-like purpuric palmar nodules following chemotherapy

We describe a patient with leukemia undergoing chemotherapy who developed painful purpuric nodules of the digits. These findings were concerning for endocarditis (clinically) and angiokeratomas on gross histology. After extensive evaluation, we report the development of painful purpuric nodules as a likely side effect of the patient's therapeutic regimen (hydroxyurea, danorubicin, cytarabine, and methotrexate).

Cutaneous Leishmania aethiopica diagnosed in the United States

Cutaneous leishmaniasis is a parasitic infection caused by certain Leishmania spp and is endemic in the New world (Central and South America) and Old World (Africa and the Middle East) where it is transmitted via sandflies of the Phlebotomus and Lutzomyia species. We describe a case of a 61-year-old woman who presented with an asymptomatic red-brown papule on her lower back approximately one year after returning to the United States from a trip to Ethiopia and Cameroon. Polymerase chain reaction was performed on the biopsy material and identified Leishmania aethiopica. This case highlights an atypical location and demonstrates how to accurately diagnose and treat this parasitic infection.

Dupilumab in HIV-positive patients with atopic dermatitis: a long-term follow-up patient and a literature review

Dupilumab is an IgG4 human monoclonal antibody licensed for the treatment of moderate-to-severe atopic dermatitis. Despite evidence suggesting that T helper type two cytokines can modulate HIV-1 replication and anti-HIV-specific immune responses, impacting on viral reservoirs, HIV-positive patients under immunomodulating therapy have been excluded from clinical trials. We report a 47-year-old HIV-positive man with late-onset severe atopic dermatitis, treated with dupilumab and followed up for 27 months. Improvements in skin lesions and quality of life were observed after four months. Blood tests showed normalization of IgE levels, with the clinical condition remaining stable at a 27- month follow-up. We gathered 16 other cases reported in the literature of HIV-positive patients treated with dupilumab, with no, or few adverse reactions, for which it is unclear if dupilumab should be held accountable. With our case and literature review, we aim to shed light on dupilumab efficacy, safety, and tolerability among HIV-positive patients suffering from atopic dermatitis. In this regard, future research should focus on the effective role, underlying mechanisms, and efficacy of dupilumab in HIV-positive patients and HIV-positivity could be questioned as a valid exclusion criterion for clinical trials.

Adenodermatofibroma: a rare variant

Adenodermatofibroma is a newly recognized variant of fibrous histiocytoma (dermatofibroma), a benign lesion frequently encountered in dermatologic practice. There are many established variants of fibrous histiocytoma but there are only eight reported cases of this specific variant in the literature. This report reviews a case of an adenodermatofibroma presenting as a large, firm, atrophic plaque on the thigh. Histopathologic findings showed dilated glandular structures with apocrine features within a fibrohistiocytic cellular infiltrate, consistent with the diagnosis. We review the characteristic findings of adenodermatofibroma, discuss the differential diagnosis, and examine current theories speculating the origin of apocrine glands present within these lesions.

Pink nodule of the chin: an unusual presentation of metastatic carcinoma

Renal cell carcinoma (RCC) is the most lethal urological tumor, often because it is widely metastasized at the time of diagnosis. There are reports of cutaneous metastases, most commonly to the head and neck, presenting late after RCC is diagnosed. This case presentation explores a 45-year old female patient with a growing skin lesion on her chin, previously treated as an epidermoid cyst before presenting to dermatology clinic. We present a case of cutaneous metastatic clear cell renal cell carcinoma presenting 7 years after initial diagnosis.

Recurrent fungating tumor and a chronic rash in an immunosuppressed transgender patient: a case of Buschke-Lowenstein condyloma and epidermodysplasia verruciformis

A transgender female in her 40s with history of HIV and testicular cancer status post-genital X-irradiation presented with a perianal mass and pruritic rash across her chest. Physical examination revealed a bulky, verrucous tumor protruding outward from the anus involving the medial buttocks. Examination of the chest and arms showed numerous guttate, pink, flat-topped papules coalescing into plaques. Clinically and histologically the lesions were consistent with Buschke-Löwenstein condyloma (BLC) and acquired epidermodysplasia verruciformis (AEDV). Buschke-Löwenstein condyloma incisional biopsy tested negative for common low- and high-risk human papillomavirus (HPV) subtypes, including 6, 11, 16, and 18, possibly implicating beta HPV subtype or a less common pathogenic subtype. The patient underwent abdominoperineal resection of the BLC, which tested positive for low-risk HPV subtypes, suggesting the possibility of multiple implicated HPV subtypes in the same tumor. This case demonstrates a possible role of beta HPV or rarer HPV subtypes in the pathogenesis of verrucous carcinoma, particularly in the setting of immunosuppression.

Photo Vignette

Concomitant endogenous and exogenous etiology for gingival pigmentation

Oral pigmented lesions can be physiological or pathological, exogenous or endogenous, as well as focal, multifocal, or diffuse. Among them, the oral melanotic macule (OMM) is a small, well-delimited brown-to-black macule, often affecting the lip and gingiva. Amalgam tattoo (AT) is a grey or black area of discoloration on the oral mucosa as a result of entry of dental amalgam into the soft tissues, commonly gingiva and alveolar ridge. Herein, we present a patient with gingival pigmentation with features of both OMM and AT in the same location.

Bleomycin-induced skin toxicity: a case of flagellate dermatitis.

Bleomycin, an antineoplastic, glycopeptide antibiotic is commonly used to treat several malignancies, in particular, lymphomas, testicular carcinoma, and squamous cell carcinoma. As bleomycin degradation by enzyme hydrolase is less in the skin and lungs, a higher likelihood of cutaneous toxicity exists. We present a case of bleomycin-induced flagellate dermatitis, a characteristic cutaneous eruption that occurred as a result of bleomycin administration. A 58-year-old man with Stage 4 diffuse large B-cell lymphoma presented with sudden onset of skin lesions that appeared five weeks after initiation of third-line bleomycin-containing palliative chemotherapy. The whip-like, linear, hyperpigmented plaques were indicative of flagellate dermatitis. We aim to present the natural course of bleomycin flagellate dermatitis and its natural course.