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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 24, Issue 5, 2018

Review

Progestins and acne vulgaris: a review

The role of exogenous progestin in the development of acne is unclear. Progestins are known for their androgenic potential, but newer generations of progestins have low or anti-androgenic activity. This review will evaluate the association between progestins found in hormonal long-acting reversible contraceptives (intrauterine devices and subdermal implants) and acne, as well as the role of oral contraceptives in acne management. Our review demonstrates that the cause and effect relationship between progestins and acne is difficult to establish and future studies that seek to understand how progestins modulate acne are necessary.

Commentary

A glimpse into the underground market of melanotan

Melanotan-I and melanotan-II are alpha-melanocyte stimulating hormone (a-MSH) analogues that can be purchased illicitly online with relative ease and are injected subcutaneously to stimulate a tan. Little is known about the use of these unregulated substances. An observational survey was posted to an online forum in which participants share their experiences using melanotan-I or melanotan-II. Users were asked to complete this voluntary, anonymous survey, which had questions focusing on motivation and hesitation for and against using melanotan, difficulty in acquiring it, and plans for continuing to use melanotan in the future.

Chinese institutional payments for publishing dermatology journal articles

Cash prizes for academic publication were introduced by the Department of Physics at Nanjing University in the 1990s. Most Chinese universities and research institutions have established cash rewards for first authors of publications. Potential payments ranged from ~$14,000 for an original research article in JAAD to ~$2000 for a case report in JAMA Dermatology. We examined rewards for publication of academic dermatological articles in China by searching for the cash-reward policies of general and dermatology Chinese hospitals. Specific cash-rewards for publication in the top three highest impact dermatological journals were recorded and compared between two dermatological hospitals, four general hospitals, and Chinese national core journals. Rewards were based upon the Science Citation Index (SCI), impact factor (IF) and publication type. Payment policies were compared between dermatological hospital and general hospitals using the t-test. There was no statistically significant difference between the cash reward payments allotted by general versus dermatological hospitals in China (P=0.32). Chinese authors may receive monetary rewards for a publication in a top dermatology journal based upon journal impact factor and publication type. These policies motivate academic publications and provide an alternative means to reward researchers for their scientific achievements than currently practiced in the West.

Tracking Mohs micrographic surgery referrals at the VA

Large dermatology centers such as the Veterans Affairs health care system carry the challenge of providing adequate care for patients within an appropriate timeline. Herein we begin a discussion about the tracking systems in place at busy hospitals, such as the VA, where numerous biopsies are referred to outside providers for further treatment. The complex psychosocial aspect of providing care specifically to veterans is also addressed. Finally, we describe our system, which monitors malignant skin biopsies that are referred to outside clinics for Mohs Micrographic Surgery (MMS).

Case Report

HIV-associated erythema elevatum diutinum: a case report and review of a clinically distinct variant

A 24-year-old man with untreated human immunodeficiency virus (HIV) infection consulted our outreach clinic owing to the development of numerous asymptomatic nodules on his palms and fingers. Histopathologic evaluation revealed leukocytoclastic vasculitis and prominent fibrosis with a neutrophilic infiltrate consistent with erythema elevatum diutinum (EED). We referred the patient for initiation of antiretroviral therapy and started him on dapsone. The pathogenesis of EED is not completely understood, but it has been associated with numerous systemic conditions that may be infectious, inflammatory, or neoplastic. Only recently has EED been recognized as a defined reactive dermatosis of HIV. We present an exemplary case of HIV-associated EED and review the differential diagnosis, highlighting clinical features of EED that appear to be more frequently encountered in the HIV-infected population.

Lymphomatoid papulosis in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma: case report and literature review

Background: Chronic lymphocytic leukemia (CLL) is a B cell lymphoproliferative disorder that characteristically presents in older individuals. Small lymphocytic lymphoma (SLL) occurs when CLL cells infiltrate lymph nodes and other tissues but spare peripheral blood and bone marrow. Lymphomatoid papulosis (LyP) is an indolent cutaneous CD30+ lymphoproliferative disorder characterized by papules and nodules that develop and spontaneously regress over weeks to months. Methods: An 84-year-old man with CLL who developed LyP is described. The features of other patients who concurrently had both of these conditions are reviewed. Results: A man was diagnosed with CLL at age 50 years. At 84 years of age, he presented with red papules on his buttocks, which demonstrated a CD30+ lymphoproliferative disorder on biopsy. Correlation of the lesion history, morphology, and histopathology established the diagnosis of LyP. LyP and CLL/SLL, including in this patient, has only been reported in 11 individuals, to our knowledge. Conclusion: The concurrent expression of LyP and CLL/SLL is rare. Since the conditions derive from different lymphocyte subsets, the concurrent expression may be merely coincidental. However, the development of both conditions in the same individual may provide additional insight into the pathogenesis of these disorders.

Perforating disorder secondary to leflunomide and review of the literature of medications associated with perforating disorder

The perforating dermatoses are a group of disorders characterized by transepidermal elimination of a material from the upper dermis. The most widely accepted classification consists of four primary perforating disorders that are defined by the type of material eliminated and the type of epidermal disruption. Pathogenesis of the perforating dermatoses is poorly understood, but some appear to have a genetic component. There are also acquired forms, which have been associated with underlying systemic diseases and the use of certain drugs. In this report, we describe a perforating disorder that occurred secondary to leflunomide therapy. To our knowledge, this is the first case in which this has been reported. We also review the recent literature on medications associated with perforating disorders.

Leukemia cutis as the presenting symptom of acute myeloid leukemia: report of three cases

Leukemia cutis (LC), a rare cutaneous manifestation of leukemia, can precede, follow, occur concurrently with, or present in the absence of (aleukemic) systemic leukemia. Leukemia cutis is especially rare as the presenting symptom of leukemia and is associated with a poor prognosis. Although more commonly seen in acute leukemias of myeloid and monocytic lineage, lymphocytic/lymphoblastic leukemias can also involve the skin. Three cases of LC presented with diverse skin lesions ranging from an erythematous rash to violaceous macules and papules to subcutaneous nodules. One case clinically mimicked fixed drug eruption. All the patients had acute myeloid leukemia (AML). Lesions showed two overarching histologic patterns: atypical perivascular infiltrate or nodular dermal histiocytoid infiltrate. Our cases expressed myeloperoxidase (MPO), a helpful marker to distinguish myeloid from non-myeloid cells, and CD68, a monocytic marker frequently expressed in cutaneous AML. CD14, a marker of monocyte maturity, was negative. In the absence of systemic leukemia, common diagnostic tools for hematologic malignancies such as bone marrow biopsy and flow cytometry are non-contributory, making morphologic and immunohistochemical analysis of the skin lesions key to diagnosis.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus: report of a case and review of the literature

Severe bullous eruptions in systemic lupus erythematosus (SLE) patients include bullous SLE, Rowell syndrome, toxic epidermal necrolysis (TEN), and TEN-like eruption of acute cutaneous lupus (TEN-like ACLE). TEN-like ACLE, a rare manifestation of SLE that closely mimics TEN, can be distinguished by characteristic clinical and laboratory findings. A 27-year-old man with SLE who developed TEN-like ACLE after initiating mycophenolate mofetil for active SLE is reported. The reports of 37 women and six men  including our patient with TEN-like ACLE were also reviewed. The diagnosis of SLE or subacute cutaneous lupus erythematosus was either previously confirmed or established at the time of diagnosis of TEN-like ACLE in 41 patients. Fever was present in 59% of patients. The onset of TEN-like ACLE was either subacute (73%) or acute (27%). Thirteen cases did not clarify the nature of disease onset. The skin lesions often presented initially on sun-exposed sites (29 patients) and involved one or more mucous membranes (21 patients). A new medication may have caused the TEN-like ACLE in 67% of the patients. Systemic corticosteroids either alone or combined with hydroxychloroquine, intravenous immunoglobulin, or mycophenolate mofetil were the most commonly used treatment. Patients with TEN-like ACLE patients had an 89% survival.

Case Presentation

Generalized retiform purpura as a presenting sign of diffuse dermal angiomatosis

A 49-year-old man presented with progressive, painful, ulcerative, retiform purpuric patches on the torso and extremities. Multiple skin biopsies revealed a prominent pan-dermal vascular proliferation but no occlusive vasculopathy or cutaneous vasculitis. Diffuse dermal angiomatosis should be considered in the differential diagnosis of retiform purpura, especially in patients with atherosclerotic disease or underlying hypercoagulable states.

Diffuse vesiculobullous eruption with systemic findings

Drug induced linear IgA bullous dermatosis (LABD) is a rare blistering disease that has been shown to be associated with the use of various medications. Although rarely seen together, some of the medications associated with LABD can lead to the syndrome drug reaction with eosinophilia and systemic symptoms (DRESS), which presents with fever, cutaneous eruption, and multi-organ involvement. We present a patient who developed fever and a generalized vesiculobullous eruption after recently starting amlodipine and meloxicam. Initial laboratory tests demonstrated elevated liver function tests, leukocystosis, and eosinophilia. Histopathologic examination of the punch biopsy revealed a bulla with sub-epidermal split and numerous neutrophils. Direct immunofluorescence demonstrated broad deposition of IgA along the dermal-epidermal junction. These findings were consistent with an overlap between LABD and DRESS. Drug induced LABD and DRESS are independently both rare diseases. It is even more uncommon to see the two concurrently in the same patient. In this patient, these two conditions were thought to be triggered by either amlodipine or meloxicam. Given the high mortality rate associated with DRESS, it is important to recognize the presentation and initiate the appropriate treatment plan as soon as possible.

Diffuse dermal angiomatosis of the breast with adjacent fat necrosis: a case report and review of the literature

Diffuse dermal angiomatosis (DDA) is a type of reactive skin angioproliferation. Clinically, this rare disorder presents as red-violet purpuric papules and/or plaques (some with a greater tendency towards necrosis and ulceration), which can be localized in any body area, but is most often seen in the upper and lower extremities. Localization in the breast commonly presents with severe intractable breast pain and characteristic reticular violaceous erythematous plaques with central ulcerations. Histological examination is fundamental for the diagnosis and is characterized by varied patterns of lobular or diffuse hyperplasia of endothelial cells at the extravascular level. The condition is associated with various underlying conditions, many of which result in local tissue ischemia. In this report, we present a patient with DDA with an underlying mass lesion of the breast, which proved to be an adjacent fat necrosis. Various treatments have proven beneficial, including revascularization, oral corticosteroids, smoking cessation, and isotretinoin. In this case, our patient benefited from secondary excision of the affected area.

Cutaneous collagenous vasculopathy: development after coronary artery bypass surgery

Cutaneous collagenous vasculopathy (CCV) is a rare benign microangiopathy of the superficial dermal vessels. Clinically, it is characterized by widespread, asymptomatic development of cutaneous telangiectasia in the absence of systemic symptoms. Morphologically, it most resembles generalized essential telangiectasia and other telangiectatic syndromes such as telangiectasia macularis eruptiva perstans (TMEP), ataxia telangiectasia, and hereditary hemorrhagic telangiectasia. It is distinctive in its histology, showing characteristic dilated thick-walled blood vessels in the superficial dermis. The thickened walls of these superficial dermal blood vessels demonstrate reduplication of the basement membrane on PAS staining. We report a 63-year-old man with CCV with this condition for 20 years, starting in 1996. He was diagnosed in the past as having essential telangiectasia. The development of the telangectasias occurred after coronary artery bypass grafting, also performed in 1996. This case not only demonstrates the characteristic clinical and histologic findings, but also suggests a possible mechanism. Moreover, it illustrates that cases of generalized essential telangiectasia may in fact be CCV that are misclassified.

Photo Vignette

Firm, hyperpigmented subcutaneous nodule in the inguinal fold of an infant

Subcutaneous juvenile xanthogranuloma (JXG) of the inguinal fold, an unusual location, was diagnosed in an infant. Subcutaneous JXG should be included in the differential diagnosis of subcutaneous nodules of the lower body, despite the absence of the characteristic yellowish hue usually associated with JXG.

Rapidly growing subcutaneous mass in an infant

Fibrous hamartoma of infancy (FHI) is a benign mesenchymal tumor of young children. It has a broad clinical differential diagnosis and is often clinically confused for vascular and malignant soft tissue neoplasms. Recognition of the unique histologic features of FHI, a triphasic population of mature adipose tissue, mature fibrous tissue, and immature mesenchymal tissue, will ensure the correct diagnosis. In this report we present a case of this rare entity, including the associated clinical, radiologic, and histologic findings.

Letter

Clostridium difficile arising in a patient with hidradenitis suppurativa on clindamycin and rifampin

We report a case of Clostridium difficile infection in a patient with hidradenitis suppurativa who was taking clindamycin and rifampin. Clostridium difficile infection treatment prompted discontinuation of the medication. Clostridium difficile infection is known to develop after antibiotic treatment, such as clindamycin, but has rarely been associated with anti-tuberculosis agents, such as rifampin. Clinicians should be aware of the risk of Clostridium difficile infection in patients with hidradenitis suppurativa, even in those receiving rifampin.

Bullous pemphigoid and neurological disorders among a Jewish population

A retrospective case control study included data regarding 65 Jewish bullous pemphigoid (BP) patients diagnosed in a tertiary medical center and a control group consisting of 182 matched Jewish patients. The study indicates that Jewish patients with BP suffer from a higher prevalence of neurological diseases compared to patients with no BP, similarly to previous reports showing high prevalence of neurological diseases among BP patients from different ethnic groups.

Squamous cell carcinoma of the cervix arising in a patient on adalimumab � a need for cervical screenings in patients on tumor necrosis factor inhibitors

Adalimumab, a tumor necrosis factor (TNF) inhibitor, has been approved for treatment of hidradenitis suppurativa. We report a case of cervical cancer in a patient with hidradenitis suppurativa taking adalimumab, which prompted discontinuation of the medication. Physicians should obtain a detailed cervical medical history before putting a female patient on a TNF inhibitor. Patients on TNF inhibitors who have pre-existing cervical issues such as human papillomavirus (HPV), dysplasia, or high grade intraepithelial lesions should be counseled about an increased risk of developing squamous cell carcinoma (SCC) of the cervix while on a TNF inhibitor. Furthermore, patients on TNF inhibitors should comply with the national screening guidelines for cervical cancer and be tested for human papillomavirus.

Scalp-to-toes application of permethrin for patients with scabies

Scabies is an infestation of Sarcoptes scabiei resulting in intensely pruritic erythematous papules tipped with hemorrhagic crusts. Current guidelines and medication labels instruct application of topical permethrin 5% cream from neck down for treatment of adult patients with scabies. Although scalp and head involvement are observed in infants, it is rare in older children and adults. Herein, we present a case of an 11-month-old patient who had scalp involvement, and required a scalp-to-toes application of permethrin. We also present a 77-year-old patient with scabies involving the head, who failed initial treatment when permethrin was applied neck down, but had subsequent resolution when it was applied scalp-to-toes. These cases provide impetus for review of current guidelines to reflect application of topical permethrin 5% cream to all external surfaces of the body including the scalp and head.

Corrigendum

Acquired bullous acrodermatitis enteropathica as a histologic mimic of pemphigus foliaceus in a patient on parenteral nutrition

The original article was published on July19, 2017 and corrected on May 15, 2018. The revised version of the article includes a funding source for Dr. Maija Kiuru’s participation in this case report, awarded by the National Cancer Institute, National Institutes of Health grant K12CA138464. This change appears in the revised online PDF copy of this article.