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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 23, Issue 6, 2017

Case Report

Rituximab in the management of juvenile pemphigus foliaceus

Background

Pemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab’s efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.

Purpose

We describe a patient with juvenile PF treated with rituximab and review the literature for similar cases.

Methods

PubMed was searched for the terms: antibody, B cells, blistering, CD20, foliaceus, juvenile, pemphigus, rituximab, immunosuppression. As the first reported case of rituximab treated pemphigus was in 2001, only cases from 2001 and after were included. Juvenile PF was defined as disease diagnosis between ages 12-17.

Results

Five cases have been reported. The indication for rituximab in most cases was refractory PF unresponsive to systemic glucocorticoids and non-steroidal adjuvant therapies. All cases demonstrated significant improvement or complete remission and most experienced no adverse events.

Conclusions

Rituximab appears to be both well tolerated and efficacious for refractory juvenile PF. Therefore, it may be considered for severe cases of PF to avoid side effects associated with conventional glucocorticoid therapy.

Cutaneous HIV-associated Kaposi sarcoma: a potential setting for management by clinical observation

Kaposi sarcoma (KS) is a malignancy of viral etiology whose course ranges from cutaneous limited lesions to fulminant disease with multi-organ involvement. Four clinical variants of the disease exist: classic, endemic, iatrogenic, and epidemic. Iatrogenic and epidemic variants of Kaposi sarcoma develop in the setting of immune suppression. Transplant recipients who develop iatrogenic KS typically demonstrate improvement of lesions following de-escalation of immunosuppressive therapy. Similarly, HIV-infected patients who begin highly active antiretroviral therapy (HAART) experience immune reconstitution, which can induce KS regression. We describe two patients with varying clinical outcomes of cutaneous-limited HIV-associated KS after immune reconstitution with HAART. We propose that immune reconstitution with HAART, followed by clinical and radiographic surveillance for disease progression, may be an appropriate initial management strategy for limited cutaneous HIV-associated KS. In patients with more extensive disease at presentation or failure of HAART alone, antineoplastic therapy should be instituted.

Angiolymphoid hyperplasia with eosinophilia of the nail unit

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a distinctive lymphocyte rich vasoformative proliferation comprising epithelioid-appearing endothelial cells with partially canalized capillary vessels in a lymphohistiocytic and eosinophil rich environment. ALHE presents clinically as single or multiple pink-brown dome-shaped papules or nodules, most commonly on the ear and peri-auricular area, followed by the face, and scalp. ALHE involving the subungual unit is rare with only 5 previously reported cases and all involved the underlying bone. The authors describe a case of a painful subungual nodule of the left 1st fingernail in a 48-year-old woman. An excisional biopsy was performed confirming a diagnosis of ALHE. There was no bone involvement and immediately following excision of the tumor, there was complete resolution of her symptoms. Our patient’s presentation expands upon the clinical and histopathological spectrum of subungual ALHE.

Case Presentation

Klippel-Trenauney syndrome with axillary hyperhidrosis

Klippel-Trenaunay syndrome (KTS) is a rare, clinically variable congenital disorder involving capillary malformations, soft tissue or bone hypertrophy, and venous malformations or varicose veins. We report a 28-year-old man who presented with a hypertrophic right arm as well as markedly increased ipsilateral axillary hyperhidrosis and erythematous patches on the back, chest, and arm. This case of KTS is unusual because our patient presented with a markedly increased unilateral axillary hyperhidrosis ipsilateral to the hypertrophic limb.

Linear verrucous hemangioma of the upper limb: a rare case

Verrucous hemangioma is a rare, congenital vascular malformation of the cutaneous and subcutaneous tissue. It is usually present at birth and gradually increases in size and number with age; occasionally it presents in later adulthood. It has a predilection for the lower extremity and usually presents as warty or hyperkeratotic, bluish and partly confluent papules and plaques. Verrucous hemangioma occurring in a linear pattern is an even more uncommon presentation and very few cases have been reported. We report a boy with verrucous hemangioma localized to the left upper extremity in a linear pattern. We also discuss management.

Sebaceous carcinoma on the arm of a 10-year-old girl

We report a case of a 10 year-old girl diagnosed with sebaceous carcinoma of the posterior left arm. The presented case reviews the histopathological and immunohistochemical characteristics of this malignancy, including a review of the literature in pediatric patients regarding prognosis and treatment. Sebaceous carcinoma is a malignant neoplasm with sebaceous differentiation, typically occurring in the sixth-to-seventh decades of life. It most commonly arises in the periocular region. It is extremely rare in the pediatric population.

Autoimmune progesterone dermatitis presenting as fixed drug eruption: a case report

Autoimmune progesterone dermatitis (APD) is a rare disorder characterized by periodic skin lesions that erupt during the luteal phase of the menstrual cycle. Clinical manifestations of APD is caused by an unusual allergy to progesterone and has a wide range of clinical manifestations from eczema and urticaria to angioedema and erythema multiforme. A 46-year-old woman described recurrent, round erythematous plaques on the lower lip, both forearms and buttocks. These skin eruptions waxed and waned for 10 months, reoccurring 3-4 days before menstruation. Based on her medical history and physical examination, APD was suspected and the progesterone challenge test showed positive results. After treatment with oral prednisolone (30 mg/day) before menstruation, the severity of eruptions decreased dramatically but recurrence did not cease completely.

Parvovirus B19 infection in an adult presenting with connective tissue disease-like symptoms: a report of the clinical and histological findings

Parvovirus B19 infections in adults are usually associated with nonspecific and mild symptoms. However, cases presenting with a lupus-like syndrome have been described, leading to the hypothesis that parvovirus infection can induce connective tissue disease. Various histopathologic features of cutaneous manifestations of parvovirus have been reported, including features which overlap with those of connective tissue disease. Herein, we discuss an unusual case of Parvovirus  B19 infection in a middle-aged woman. The biopsy results showed granulomatous vasculitis and were consistent with the previously described superantigen id reaction. This case demonstrates that infectious causes should be considered in the differential diagnosis for granulomatous vasculitis and clinicopathologic correlation is required for accurate diagnosis. We also provide a review of the literature highlighting the possible role of parvovirus in induction of a connective tissue disease-like presentation.

High-frequency ultrasound features in a case of gouty panniculitis

Gouty panniculitis is caused by the deposition of urate crystals in the subcutaneous tissue, accompanied by a lobular panniculitis. It presents as subcutaneous nodules, most commonly located on the lower extremities. Being an unusual clinical presentation of gout, the sonographic findings of gouty panniculitis have been scarcely described in the literature. Our report describes the ultrasound features we found in a case of gouty panniculitis and the usefulness of this technique for diagnosis and monitoring of this disease.

Cutaneous varicella zoster virus infection following zoster vaccination: report of post-vaccination herpes zoster skin infection and literature review of zoster vaccination efficacy and guidelines

Background

Herpes zoster vaccine is currently recommended in the United States for immune competent individuals ≥60 years. The efficacy of the herpes zoster vaccine decreases with age and with time following vaccination.

Purpose

An elderly man with herpes zoster following vaccination is described. The guidelines for vaccination and issues regarding re-vaccination are reviewed.

Methods: PubMed was used to search the following terms: efficacy, elderly, herpes zoster, herpes zoster incidence, herpes zoster recurrence, and vaccination. The papers and relevant citations were reviewed. The clinical features of a patient with post-vaccination herpes zoster skin infection are presented; in addition, vaccine efficacy and guidelines are reviewed.

Results

A 91-year-old man, vaccinated for herpes zoster 10 years earlier, presented with crusted erosions on his face corresponding to the area innervated by the ophthalmic division of the left trigeminal nerve. Evaluation using polymerase chain reaction confirmed the diagnosis of herpes zoster.

Conclusions

Herpes zoster vaccine decreases in efficacy with both age and number of years following vaccination. Therefore, booster shots or revaccination in the older population may be of benefit.

Lichen sclerosus et atrophicus: atypical case simulating lichen planus

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis, characterized by shiny, atrophic, hypochromic papules with a predilection for the genital and perineal skin. Extragenital involvement may occur, but is rare in the isolated form. LSA more commonly affects prepubertal and postmenopausal women. We describe an unusual case of isolated extragenital LSA, restricted to the wrists and mimicking lichen planus.

Commentary

Examining the race-specific prevalence of hidradenitis suppurativa at a large academic center; results from a retrospective chart review

Hidradenitis suppurativa (HS) is a chronic, inflammatory, debilitating disease of unknown etiology. HS can occur in people of all ethnicities and ages, and affects approximately 3-4% of the United States. To date, few studies have specifically examined the race prevalence of HS; further epidemiological research is needed to identify specific trends among HS and its racial predilections. At our center, 1.3% of African-American patients were seen for HS, compared to 18% of Caucasian patients (p<0.05), and the percent ratio of African-American versus Caucasian patients with HS was 7.22:1. Our number ratio of African-American patients versus Caucasian patients with HS was 1.19:1. Studies performed at Henry Ford Medical Center and University of Pittsburgh report ratios of 1.64:1 and 1.98:1 respectively. These data support study trends suggesting HS is more common among patients of African-American descent. A large, population-based study across the United States is needed to better assess the associations between ethnicity and HS. Examining this patient population has the potential to improve our understanding of HS pathophysiology, and will enable clinicians to better manage patients with this disease.

Photo Vignette

Carcinoma hemorrhagiectoides: case report of an uncommon presentation of cutaneous metastatic breast carcinoma.

In most cases, cutaneous metastases develop after the diagnosis of the primary internal malignancy has been established, but sometimes they can be discovered earlier or simultaneously. We describe a case of a 90-year-old woman who presented to the emergency room in poor general condition, with cutaneous lesions characterized by hot, infiltrated, violaceous and erythematous plaques involving the left chest wall. The clinical and histopathological findings were consistent with the recently described variant of inflammatory cutaneous metastatic carcinoma named carcinoma hemorrhagiectoides. Microscopic examination demonstrated extensive infiltration of the dermis by tumor cells as well as intralymphatic involvement by neoplastic cells. This is a very rare presentation of cutaneous metastasis from breast cancer.

Extragenital bullous lichen sclerosus on the anterior lower extremities: report of a case and literature review

Lichen sclerosus (LS) is a benign, chronic, inflammatory skin disease with a predilection for the anogenital region in women. Although males can also be affected, the ratio of female to male incidence has been reported to be as high as 6-10:1 and possesses a bimodal age distribution of pre-pubertal girls and postmenopausal women [1, 2]. Affected skin usually demonstrates polygonal papules that coalesce into porcelain white plaques and can be associated with edema, telangiectasias, and comedo-like plug formation [3]. Lichen sclerosus can be debilitating for some patients causing significant pruritus, pain, dysuria, and dyspareunia [4]. Rarely, lichen sclerosus appears in various extragenital areas, although most cases are relatively asymptomatic [3]. Even more uncommonly, as displayed in this case report of a 69-year-old woman, LS can present extragenitally with a bullous or hemorrhagic appearance [5].

Trichomycosis axillaris dermoscopy

Trichomycosis axillaris is a common but underdiagnosed condition of the skin. The dermoscopic image of this disease is not previously described in the scientific literature. We believe that dermoscopy is a convenient and interesting diagnostic method that may aid in the diagnosis.

Multiple papulonodular lesions on central area of the face: what is your diagnosis?

A healthy 31-year-old woman presented with a 20-year history of asymptomatic skin-colored papules and nodules on the central area of the face. Her maternal grandmother, aunts, mother, and sister also had similar lesions. Clinical, histopathological, and genetic features allowed the diagnosis of multiple familial trichoepithelioma. The patient and family were referred to the genetic department for genetic counselling. Close follow-up for the possibility of secondary basal cell carcinoma is warranted.

Lichen planus pigmentosus in a blaschkoid distribution

Lichen planus pigmentosus is a pigmentary disorder of unknown etiology, with diffuse hyperpigmentation of sun-exposed areas, more commonly seen in some ethnic and racial groups. We report an unusual case of lichen planus pigmentosus in a 40-year-old man with Fizpatrick type III skin that was present in a blaschkoid distribution on the trunk, a distribution that has been rarely reported. This unique presentation of lichen planus pigmentosus may contribute to better understanding of the etiology, as the blaschkoid distribution may reflect underlying cutaneous mosaicism that renders those cells more susceptible to an insult that results in lichen planus pigmentosus. This disorder should be considered in the differential diagnosis of macular hyperpigmentation, especially in those from more commonly affected ethnic and racial groups, even when the distribution is atypical and in the absence of history of sun exposure.

Letter

Evaluating cultural competency and patient satisfaction in an urban dermatology clinic

Cultural competency continues to gain increased attention in medicine. Not only does it play a significant role in the delivery of health care and patient outcomes, but it also remains a major determinant of patient satisfaction. This study investigated how patients in an urban dermatology clinic rated their satisfaction with cultural competency. Compared to White patients, satisfaction scores were greater for Hispanic or Latino patients and less for Asian patients, while there was no significant difference for Black or African American patients. There were clear differences in patient satisfaction rates of various dimensions of cultural competency. A follow-up study with a larger sample size is needed for closer examination into the conclusions.

Use of a punch tool for paring plantar warts in an “awl-like” manner

Paring is useful for both diagnosis and treatment of plantar warts. Paring techniques generally involve scalpel blades, abrasive tools, or curettes. However, these tools do not permit the use of an “awl-like” carving technique of the wart afforded by using a punch biopsy tool. We have found this technique to be associated with greater precision and safety, as well as potentially increased efficacy compared to traditional methods.

Rituximab administration in a patient with pemphigus vulgaris following reactivation of occult hepatitis B virus infection

Immunosuppressive drugs are the milestone of treatment of autoimmune diseases, but they can lead to serious complications, including hepatitis B virus reactivation in HBV carriers as well as in patients with occult HBV infection (OBI). A 36-year-old man with OBI was diagnosed with pemphigus vulgaris. He was prescribed prednisolone and his hepatitis B surface antigen turned positive. Viral replication was successfully controlled by lamivudine and adefovir. Mycophenolate mofetil and intravenous immunoglobulin  were not effective in controlling the pemphigus vulgaris. The patient received rituximab 500 mg weekly for four weeks and went into remission without any adverse effect. He safely received another course of rituximab after a relapse one year later. In conclusion, testing for hepatitis B core antibody should be considered mandatory, in addition to HBsAg, for the screening of pemphigus patients to detect rare cases of OBI before starting therapy. Furthermore, rituximab may in some cases be safely used in HBV carriers using antivirals concomitantly.

Tumor necrosis factor-induced alopecia: alternative pathology and therapy

Tumor necrosis factor (TNF) inhibitors are used to treat Crohn disease and psoriasis. Although they are typically well tolerated, adverse effects include the development of alopecia, and paradoxically, psoriatic lesions. We recently described a woman with Crohn disease who developed alopecia and scalp psoriasis during infliximab therapy. After discontinuing infliximab and beginning oral and topical therapies, her alopecia completely resolved. We compared our experience with that of the Craddock et al. who described a woman with Crohn disease and alopecia secondary to adalimumab therapy. Although the authors described typical histopathologic features of TNF inhibitor-induced alopecia, including decreased sebaceous glands, psoriasiform changes, superficial and deep perifollicular infiltrate of peribulbar lymphocytes, prominent plasma cells, and variable eosinophils, we observed atypical findings that included chronic folliculitis and perifolliculitis with dermal scarring and naked hair shafts in the dermis – reminiscent of folliculitis decalvans. Both patients experienced a complete recovery; however, Craddock et al. described continuing adalimumab therapy and using intralesional triamcinolone acetonide whereas our patient discontinued infliximab therapy, used a combination of topical scalp therapies including betamethasone lotion and mineral oil overnight under occlusion, and began oral minocycline. In conclusion, various histopathologies are observed with TNF-inhibitor induced alopecia and multiple, effective, therapeutic avenues exist for this affliction.

Total body skin examination adherence among young adults with an increased risk for skin cancer

Although ultraviolent radiation exposure and skin cancer rates are rising in the US, there is not a consistent national message calling for routine total body skin examinations (TBSE) by a healthcare provider. This pilot study examined TBSE rates among adults at an increased risk for skin cancer (history of sunburn, indoor tanning use, excessive UV exposure, poor sunscreen use). Overall skin cancer prevention behaviors were insufficient among respondents (n=953) and only a quarter of respondents considered at an increased risk for skin cancer reported a TBSE. White, older students with light skin who regularly use sunscreen were significantly more likely to report a TBSE. These findings highlight the need for a national study examining TBSE across the lifespan among high-risk individuals.

Recruitment Advertisement

Dermatologists/MOHS Surgeon

The University of California, Davis, School of Medicine, Department of Dermatology, is recruiting for four academic dermatologists in the Clinical X series or Health Sciences Clinical Professor (HSCP) series at the Assistant/Associate/Professor level based on experience and qualifications. Three of these positions are for general medical dermatologists, and one is for a fellowship-trained Mohs surgeon/procedural dermatologist. The appointments may be made up to 100%.