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 1, 2018
A large proportion of patients seen in dermatology practices have underlying psychological issues associated with their skin diseases. One of the most flagrant examples of this are patients with delusions of parasitosis. These patients have false fixed beliefs that they are infested by parasites and experience cutaneous sensations of crawling, biting, and stinging associated with their delusions. There is no organic skin disorder and all cutaneous manifestations are self-induced. Rather than a psychiatrist, the dermatologist is often designated by the patient to handle the chief complaint, even though the main disorder is psychogenic. In spite of their limited evidence, antipsychotic medications have become the mainstay of therapy for delusions of parasitosis. The dermatologist must therefore be familiar with the approach to diagnosis and the use of antipsychotic or neuroleptic medications, which usually reside in the domain. There are few clinical trials and no substantial randomized controlled trials examining the efficacy of the psychiatrist antipsychotic medication used to treat delusions of parasitosis. This review article synthesizes the current available research and distils it down to analyzes 17 case reports, comprising 37 cases, examining the use of risperidone and olanzapine in the treatment of delusions of parasitosis. These findings are synthesized into a clinical pathway designed to assist dermatologists in effectively managing patients with delusions of parasitosis.
A pilot program for community dermatologists working with primary care residents to provide dermatology consults to a regional hospital
With the high demand and limited supply of dermatologists, the majority dedicate their time primarily to outpatient practice. A number ofobstacles to inpatient dermatology consults have been described, as well as the essential benefits for some of the sickest patients. Dermatology residency programs partially relieve this need, however with the transition to a single Graduate Medical Education accreditation system and many previously American Osteopathic Association-accredited dermatologyprograms ceasing to train residents after 2020, it is prudent to recruit additional possible consultants. One possible solution is to involve non-dermatology residents as part of the consult service team. We report on our experience of piloting such a program, for community dermatologists providing consults to a regional hospital utilizing family medicine andinternal medicine residents.
Background: Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are cutaneous hypersensitivityreactions that develop in response to specific triggers such as medications and certain infections. Vaccines, which undergo rigorous safety testing prior to use in humans, are a rare cause of SJS/TEN and little is known about the frequency of such events and corresponding pathogenesis.
Objective: Herein, we discuss a case of suspected TEN in a 19-year-old woman who received the meningococcal B vaccine (the first report of such an association) and conduct a systematic review of the associated literature. We also discuss management of this patient with a single dose of etanercept.
Methods: Relevant literature was searched using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method.Results: A total of 29 articles reporting EM, SJS, or TEN following vaccination were included from >5 countries. Of the 29, 22 articles reported EM, 6/29 reported SJS, and 4/29 reported TEN (3 articlesreported cases of both EM and SJS/TEN).
Conclusions: We suggest consideration of vaccines as an etiology for cases of SJS or TEN that begin with an EM-like presentation, and provide further evidence for the use of etanercept as a viable treatment for TEN.
The use of cyclosporine for Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum at the University of Louisville: A case series and literature review
Introduction: Cyclosporine therapy for Stevens-Johnson syndrome-toxic epidermal necrolysis (SJSTEN) was first reported in the literature by Renfro et al. in 1989. Herein we report an additional 4 cases of SJS-TEN treated with cyclosporine.
Methods: Case information was collected retroactively at the University of Louisville Hospital in Louisville, KY. All cases had a diagnosis of SJS or TEN by a dermatologist. All patients were ≥18 years of age and treated with cyclosporine during their admission.
Results: Three of four patients re-epithelialized within an average of 3.67 days of starting 3-4 mg/kg/day of cyclosporine. One patient passed away, likely due to advanced endometrial cancer.
Discussion: We provide a review of the literature on cyclosporine use for SJS/TEN, including various outcome measures — stabilization (cessation of new lesions), time to re-epithelialization, mortality rate, and hospital length of stay and, where available, comparison to other systemic agents.
Conclusion: The outcomes appear to be consistent with rapid re-epithelialization and low mortality as seen in many previous reports. Treating SJS-TEN with systemic agents including cyclosporine will remaincontroversial because the vast majority of data comes from case reports, case series, or small open prospective trials.
Schnitzler syndrome is a rare disease characterized by chronic urticaria and a monoclonal gammopathy, most commonly IgM with light chains of the kappa type. There are currently no known risk factorsassociated with development of the disease. We report a case of Schnitzler syndrome in a 48-year-old man with a family history of monoclonal gammopathies. The patient’s disease has been well controlled with anakinra therapy. Our case may contribute to a better understanding of the etiology of Schnitzler syndrome as his history could suggest a hereditarypredisposition for the disease. Further studies are necessary to determine whether a genetic component of Schnitzler syndrome exists, as first-degree relatives of patients with monoclonal gammopathies may be at risk for the development of the disease.
Anaplastic large cell lymphoma localized to the left breast years after radiotherapy for breast cancer
Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma that can involve the skin primarily or secondarily. Our case describes an unusual presentation of eruptive tumors localized to the leftbreast region several years following breast cancer surgery and radiation for carcinoma of the breast. This report highlights the challenges in reachingthe diagnosis of an aggressive systemic lymphoma presenting on the skin.
Senear-Usher Syndrome, or pemphigus erythematosus, is an autoimmune skin blistering disorder with an overlapping clinical presentation of pemphigus foliaceus and lupus erythematosus. Lesions typically involve the scalp, face, and upper chest or back. This case study focuses on a patient who presentedwith progressive scalp ulcers, hyperpigmentation, and eroded plaques with overlying hemorrhagic crust. Pemphigus erythematosus was diagnosedwith direct immunofluorescence, demonstrating immunoglobulin G and complement deposition both intercellularly and at the dermoepidermal junction. The patient is continuing treatment with systemicsteroids and steroid-sparing immunosuppressants.
Focal linear elastosis (FLE) is a benign skin findingcharacterized by hypertrophic linear plaques withabnormal elastic fibers on histology. We present aunique case in which focal linear elastosis occurredin the setting of joint hypermobility syndrome(JHS). Our patient, a 20-year-old man with a medicalhistory significant for symptoms consistent with JHS,had been followed by the rheumatology clinic formany months. He was referred to the dermatologydepartment for further evaluation of asymptomaticlongitudinal bands on his back that had been presentfor many years. He denied trauma but endorsed ahistory of ‘stretch marks.’ On examination there werenumerous horizontally oriented, firm, linear, yellowto flesh colored bands, all non-tender to palpation.Punch biopsies were performed of involved anduninvolved skin. Histopathology of normal skinrevealed no significant abnormalities whereasinvolved skin demonstrated broadened collagenbundles in the deep dermis. The elastic fiber stain,Verhoeff-Van Gieson, revealed a gross increase in thenumber of elastic fibers, fragmented fibers, fiberswith “paintbrush” or widened-ends, fibers of varyingthickness, and clumped fibers. This combination ofhistopathologic and clinical features was consistent with FLE.
Nivolumab reactivation of hypertrophic lichen planus, a case report and review of published literature.
We report a case of nivolumab-induced lichen planus (LP) reactivation that was previously in remission following chemotherapy for non-smallcelllung cancer (NSCLC). Chemotherapy-induced immunosuppression allowed for complete resolution of the patient’s pre-existing LP, a T-cell mediatedautoimmune process. When the patient was switched to nivolumab immunotherapy owing to progression of NSCLC, PD-1 inhibition led to an overwhelming T-cell response that seemed to have provoked a severe LPreactivation. Although lichenoid reactions have been reported with nivolumab, to our knowledge, this is the first reported case of nivolumab monotherapycausing LP reactivation in a patient with a strong personal and family history of the disease that was previously in remission after chemotherapy.
Tofacitinib is a targeted inhibitor of janus kinase (JAK), currently approved for the treatment of rheumatoid arthritis. We present a patient on treatment withtofacitinib who had an episode of classic dermatomal herpes zoster followed months later by atypical chronic cutaneous ulcers also caused by herpes zoster.
Eosinophilic infiltrate resembling eosinophilic cellulitis (Wells syndrome) in a patient with mycosis fungoides
Mycosis fungoides (MF) is a T-cell, non-Hodgkin lymphoma that primarily involves the skin. Extracutaneous involvement, such as in the parotidgland, is characteristic of end-stage disease. Eosinophilic cellulitis, or Wells syndrome, is a rare inflammatory dermatitis that involves a dermal infiltrate of eosinophils. We report a case of an 80-year-old man with a long-standing diagnosis of stage IIB MF who acutely developed parotid gland involvement and marked hypereosinophilia that most likely represented eosinophilic cellulitis. Activated T cells from his MF were likely a trigger factor for the development of his eosinophilic cellulitis. To our knowledge, this is the first reported case of an MF patient with atypical parotid gland involvement andeosinophilic cellulitis.
A novel association of pseudoainhum and epidermolytic ichthyosis, successfully treated with full thickness skin graft after failed z-plasty repair
Pseudoainhum is a rare constriction band variant thatmay progress to spontaneous digital strangulationand auto-amputation. Although its association withpalmoplantar keratodermas is well established, ithas not been reported in conjunction with classicepidermolytic ichthyosis. We describe the first suchcase in a 25-year-old woman who presented witha painful constricting band of the fifth toe. We alsodescribe her treatment course, which consisted ofa failed z-plasty, the traditional therapeutic optionfor acute pseudoainhum, and report the success ofsubsequent full thickness skin graft, suggesting thebenefit of this procedure as a therapeutic alternativefor patients with pseudoainhum.
La práctica de tatuajes tienen más de 8000 años de antigüedad, existiendo un incremento continuo en la sociedad occidental en las últimos 3 décadas La introducción de una sustancia exógena en la pielpuede provocar una respuesta inmunológica en su contra, estando descritas reacciones cutáneas por hipersensibilidad a una variedad de pigmentos, las que se limitan al área de un determinado color,atribuyéndose a los materiales inyectados. El color más reportado en la literatura es el rojo, que tradicionalmente se confeccionaban a base dederivados del mercurio (cinabrio). Diversos patrones histológicos de reacción están descritos, siendo el más frecuente el liquenoide. Presentamos una serie de 10 pacientes con reacción de hipersensibilidad a tatuaje rojo, con patrón histológico predominante dereacción granulomatosa y con moderada respuesta a tratamiento. Es importante cuando se está ante un patrón granulomatoso de reacción descartar sarcoidosis sistémica e infecciones por micobacterias.
Interstitial granulomatous dermatitis in a patient with chronic hepatitis C and mixed cryoglobulinemia
A 55-year-old man presented with a history of asymptomatic, bilateral, figurate dermatosis consisting of erythematous, annular, linear, andarciform, indurated nodules and plaques on the lateral walls of thorax, flanks, and hypogastrium. His medical history was positive for chronic hepatitis C. Skin biopsy was compatible with the diagnosisof interstitial granulomatous dermatitis. Additionalinvestigation revealed positive rheumatoid factor, antinuclear antibodies, and cryoglobulins. Thepatient started treatment with high-potency topical corticosteroid. After 12 weeks of treatment there was a partial clinical improvement of thedermatosis. Interstitial granulomatous dermatitis exhibits a distinct histopathological pattern with a diverse clinical appearance, which can be associated with autoimmune systemic diseases, neoplasms, several drugs, and infections. Optimal therapy for this condition is yet to be established, but topical corticosteroids have been a mainstay of treatment.
A 12-month-old boy presented with three months of asymptomatic hypopigmented flat-topped papules on the suprapubic skin and lower abdomen. Emollients and topical steroids offered no improvementand the patient was referred to the dermatology department. Shave biopsy revealed a papillated epidermis with scattered solitary mononuclearclear cells at all levels of the epidermis and an overlying basket weave orthokeratosis. The cells were epithelioid with increased amphophilic cytoplasm. Immunohistochemical staining was positive for CK7,CEA, and CAM5.2 and negative for S100, CD1a, and Mart-1. These findings were consistent with clear cell papulosis. No treatment was recommended as these lesions were asymptomatic. However, yearlyfollow up was recommended given the resemblance of these cells to those of Paget disease. Review of the literature demonstrates a total of 31 biopsy confirmed cases with AE1, CEA, and EMA positivity and S100negativity as the most consistent staining properties. A recent retrospective review of 19 cases documents long term follow-up of at least six years and up to 21 years. The results suggested a tendency toward selfresolution and an absence of malignant progression, supporting the benign nature of these lesions.
Precalcaneal congenital fibrolipomatous hamartoma is a benign condition of infancy that is sometimes misdiagnosed due to lack of reports in the literature. Lesions usually present with painless, non-pruritic, skin colored bilateral, solitary, symmetric nodules located in the middle of the heels. The lesions gradually increase in size and then regress by the age of 2 to3 years old.
Adult blaschkolinear acquired inflammatory skin eruption (BLAISE) with simultaneous features of lichen striatus and blaschkitis
Blaschkitis and lichen striatus are generally distinguished in the literature by the age of onset, lesion distribution, and histopathology. However, there is currently no clear consensus among authors about whether to consider blaschkitis and lichen striatus different clinical entities or a spectrum ofthe same disease. We present a case of adult BLAISE with features of both lichen striatus and blaschkitis, which seems to support the theory that these clinical entities may in fact represent a spectrum of the same pathological process.
Rubinstein-Taybi syndrome (RTS) is an autosomaldominant hereditary disease, which contains many skeletal and organ anomalies as well as mental retardation. Although high incidence of keloids in RTS is known, it is difficult to find a detailed report on the clinical features of keloids. In the following letter, we report an RTS patient fulfilling diagnostic criteria whosuffered from both keloids and pilomatricoma. We also performed a literature search, which identified the possible involvement of the Wnt/β-catenin signaling pathway in the pathogenesis of these two skin lesions.
Phytophotodermatitis is a clinical diagnosis from phototoxicity of the skin induced by contact with plants or their extracts. Phytophotodermatitis maypresent with burning, erythema, patches, plaques, vesicles, bullae, or hyperpigmented patches in welldemarcated and unusual shapes. Inquiring about occupation, hobbies, and plant or plant extract contact is essential to establishing the diagnosis. Herein we present a case of phytophotodermatitisafter use of carrot extract-containing sunscreen presenting as a hyperpigmented patch in a geometric distribution with accentuation of pigment within the dynamic rhytides.