Post-streptococcal small-vessel vasculitis in a 16-month-old with associated intussusception and hypertension: Henoch Schönlein purpura?

Henoch Schönlein purpura (HSP), also known as IgA vasculitis, is a systemic small-vessel vasculitis typically occurring in children 3-15 years of age, with peak incidence at 4-6 years. It is characterized by a constellation of symptoms including palpable purpura, arthralgias or arthritis, abdominal pain including intussusception, and renal involvement. We report a patient with these clinical findings whose IgA immunofluorescence was negative but with a presumptive diagnosis of HSP at 16 months of age, significantly younger than the classic population. This condition rarely affects this age group, and we highlight the importance of considering vasculitis in children of all ages, as a failure to diagnose could lead to insufficient long-term monitoring, particularly regarding renal function.

No bilingual advantage in children's attentional disengagement: Congruency and sequential congruency effects in a large sample of monolingual and bilingual children.

According to recent accounts, bilingualism in childhood confers an advantage in a specific domain of executive functioning termed attentional disengagement. The current study tested this hypothesis in 492 children (245 boys; Mage = 10.98 years) from Canada, China, and Lebanon by testing for an association between language status and measures of attentional disengagement. Across the entire sample, monolinguals responded more quickly and accurately than bilinguals on a measure of attentional disengagement but differed in age, socioeconomic status, and general cognitive ability. Differences between monolinguals and bilinguals disappeared when the influence of these confounding variables was controlled using a matched samples analysis (ns = 105). Bayesian analyses further confirmed that the evidence was more likely under the null hypothesis than under the alternative hypothesis. In sum, there was little evidence of an association between language status and attentional disengagement in children.

Delayed diagnosis of DRESS syndrome in a patient with skin of color.

We describe a particularly severe case of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome with hemodynamic instability, erythroderma, profound eosinophilia, and severe organ dysfunction. We attribute the severity in part to a delay in diagnosis due to patient's skin of color, as the erythroderma was not noticed until a dermatologist was consulted. This case highlights how even severe skin disease can present less conspicuously in patients with darker skin types. We outline several strategies that can help clinicians to recognize DRESS and other skin disease phenotypes in patients of color, thereby avoiding delays in diagnosis as seen in this case.

Successful radical surgical resection of a giant neurofibroma.

Large neurofibromas often cause significant patient morbidity and present a unique challenge to dermatologists and surgeons. Radical resection offers the lowest rate of recurrence but is not often pursued due to the high risk of intraoperative hemorrhage and difficulty in repairing large defects. Subtotal resection and debulking are more frequently performed, leading to higher rates of recurrence. This case highlights a particularly large neurofibroma and demonstrates how surgical techniques like preoperative embolization and advancement flaps can improve outcomes in the radical resection of large neurofibromas.

Management of relapsed cutaneous T-Cell lymphoma following allogeneic hematopoietic stem cell transplantation: Review with representative patient case.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative treatment option for patients with refractory cutaneous T-cell lymphoma (CTCL) through replacement of the bone marrow responsible for lymphoma cells and possibly induction of a graft-versus-lymphoma effect. However, allo-HSCT is not always curative; relapse of CTCL occurs in about half of patients post-transplant. Treatment of relapsed CTCL after allo-HSCT is challenging because post-transplant patients are at high risk of graft-versus-host disease, and this condition may be precipitated or exacerbated by standard CTCL therapies. The benefit of each potential therapy must therefore be weighed against its risk of graft versus host disease (GVHD). In this article, we review the management of relapsed CTCL after allo-HSCT. We begin with an exemplative patient whose relapsed Sezary syndrome was successfully treated without development of GVHD. We also report high-throughput T-cell receptor sequencing data obtained during the patient's disease relapse and remission. We then review general guidelines for management of relapsed CTCL and summarize all reported cases and outcomes of relapsed CTCL after transplant. We conclude by reviewing the current CTCL therapies and their risk of GVHD.

Resiquimod: a new topical immune-response modifier for the treatment of actinic keratosis.

Resiquimod is a Toll-like receptor (TLR)-7 and TLR-8 agonist that, like imiquimod, belongs to the class of imidazoquinolines, small organic molecules with potent antiviral and anticancer activity. Resiquimod activates myeloid and plasmacytoid dendritic cells while also promoting release of cytokines as interleukin-6, tumor necrosis factor-α and interferon-γ more effectively than imiquimod. Given these immunologic effects, resiquimod is emerging as a new topical pharmacotherapy for actinic keratosis (AK). In the pivotal trial by Stockfleth et al. complete clinical clearance in all the resiquimod-treated arms was more significant than placebo varying from 56% to 74%. Partial clinical clearance, or disappearance of >75% of AKs, was also significantly higher in the resiquimod arms varying from 75% to 87%. Overall, resiquimod is a new molecule that remains a promising therapy for AK, although additional studies are needed to further evaluate its efficacy.

Interleukin-31, a Potent Pruritus-Inducing Cytokine and Its Role in Inflammatory Disease and in the Tumor Microenvironment.

Substantial new information has emerged supporting the fundamental role of the cytokine interleukin-31 (IL-31) in the genesis of chronic pruritus in a broad array of clinical conditions. These include inflammatory conditions, such as atopic dermatitis and chronic urticaria, to autoimmune conditions such as dermatomyositis and bullous pemphigoid, to the lymphoproliferative disorders of Hodgkin's disease and cutaneous T-cell lymphoma. IL-31 is produced in greatest quantity by T-helper type 2 (Th2) cells and upon release, interacts with a cascade of other cytokines and chemokines to lead to pruritus and to a proinflammatory environment, particularly within the skin. Antibodies which neutralize IL-31 or which block the IL-31 receptor may reduce or eliminate pruritus and may diminish the manifestations of chronic cutaneous conditions associated with elevated IL-31. The role of IL-31 in these various conditions will be reviewed.

The efficacy of omalizumab in Cutaneous Mastocytosis: A case series.

BACKGROUND: Mastocytosis describes a heterogeneous group of disorders arising from a clonal proliferation of mast cells. Given the lack of curative treatments for the cutaneous form, there is a significant need for superior therapies. Omalizumab is a recombinant DNA-derived humanized IgG monoclonal antibody that selectively binds to human immunoglobulin E (IgE). It represents a potential treatment for the management of cutaneous mastocytosis, which currently has no standard treatment. METHODS: Two patients were treated with subcutaneous omalizumab 300 mg every 4 weeks. DISCUSSION: Patient 1 experienced 50% reduction in cutaneous infiltration and moderate improvement in pruritus. Patient 2 underwent 90% complete clearance of cutaneous lesions and reported full resolution of pruritus. The median duration of treatment was 24 weeks and time to response was 8 weeks. No significant changes in tryptase levels were observed. Both patients experienced injection site reactions. CONCLUSION: We provide evidence from two cases supporting the efficacy of IgE-mediated therapy in the treatment of cutaneous mastocytosis. Even at a higher-than-standard dose (300 mg vs. 150 mg), the drug was well-tolerated. As we await the results of pivotal clinical trials, omalizumab appears to be a promising treatment option in patients with cutaneous mastocytosis unresponsive to traditional therapies.

Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder in a pediatric patient successfully treated with low-dose radiation.

Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.

Cutaneous manifestations of genodermatoses and primary immunodeficiency.

Immunodeficiency is most commonly related to inherited syndromes, infections, chemotherapy, or aging. As the population of individuals with immunodeficiency continues to grow, physicians are confronted with the task of diagnosing dermatologic disease in this population. Cutaneous involvement in immunodeficiency disorders serves as a useful tool that aids diagnosis and provides prognostic value. Given that cutaneous manifestations often herald an underlying immunodeficiency disorder, understanding the complexities of these diseases is important for appropriate clinical management. Although certain diseases may present with stereotypical cutaneous lesions, others may involve more variable lesions that require specialized consultation for diagnosis and treatment recommendations. In this review, we discuss a number of cutaneous findings associated with primary immunodeficiencies. Awareness of these cutaneous associations may aid in the early detection and prompt treatment of these potentially serious immunologic disorders.

Rapid, successful treatment of atopic dermatitis recalcitrant to topical corticosteroids.

Atopic dermatitis is responsive to midpotency topical corticosteroids, which are the mainstay of treatment, yet many patients have disease that is "resistant" to triamcinolone prescribed for outpatient use. Such resistance is often due to poor adherence, but patients and caregivers may remain adamant that the steroid was ineffective and assure the physician that it was applied as recommended. We describe the case of a young girl with a 2-year history of atopic dermatitis resistant to triamcinolone whose condition rapidly improved with continued use of triamcinolone. Our case raises the ethical dilemma of whether physicians should base treatment plans on what patients report or what evidence on adherence suggests.

Juvenile mycosis fungoides with large-cell transformation: Successful treatment with psoralen with ultraviolet A light, interferon-alfa, and localized radiation.

Mycosis fungoides with large-cell transformation is historically associated with a poor prognosis. Pediatric cases of mycosis fungoides with large-cell transformation are rare, with only three other cases reported in the literature. We present the first case of a child with almost complete remission of his mycosis fungoides with large-cell transformation shortly after administration of psoralen plus ultraviolet A, interferon-alfa, and localized radiation.

Mycosis fungoides occurring at the site of previous herpes zoster eruption.

Numerous clinicopathological variants of mycosis fungoides have been described in the literature. Dermatomal or zosteriform mycosis fungoides is one reported variant but a clear aetiology has never been documented. We report a case of mycosis fungoides proved by biopsy and immunohistochemistry that developed in a 55-year-old man at the site of previous herpes zoster eruption. We also present a review of the relevant literature to add to the understanding of rare variants of mycosis fungoides and aid in the clinical recognition of zosteriform mycosis fungoides.

How to Discern Folliculotropic Mycosis Fungoides From Follicular Mucinosis Using a Pediatric Case.

Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides (MF) with folliculotropic, atypical lymphocytes that may or may not have mucin deposition surrounding the hair follicle. Follicular mucinosis (FM) is a primary or secondary finding in FMF, lupus, or collagen vascular diseases that is only a histological process of mucin deposition surrounding the hair follicles. We present a case of a 6-year-old boy who had features of both FMF and primary follicular mucinosis (PFM). The case reveals key insights on FMF with concurrent FM in pediatric patients and how to differentiate between FMF and PFM.

Bullosis Diabeticorum: A Neglected Bullous Dermatosis.

A 75-year-old African-American man presented with a 3-year history of painless, fluid-filled blisters, for which his primary care physician had treated him with doxycycline, cephalexin, and topical corticosteroids, with no significant improvement. The blisters had ruptured spontaneously and healed with scarring. He denied antecedent trauma. His medical history was remarkable for insulin-dependent type 2 diabetes mellitus, hypertension, hypercholesterolemia, primary cutaneous melanoma status-post excision, and breast cancer status-post mastectomy and chemotherapy. Physical examination revealed nontender bullae, measuring up to 4 cm × 3 cm and containing serous fluid, on the anterior portion of both tibias (Figure 1). The Nikolsky sign was negative. There was no evidence of surrounding inflammation. A biopsy revealed subepidermal bullae formation with sparse inflammatory infiltrate (Figure 2). Direct and indirect immunofluorescence studies were negative for immunoglobulin (Ig) G, IgA, IgM, complement C3, C5b-9, and fibrinogen deposition. Culture of the bullous fluid was negative.

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.