Sarah L. Stein

Severe Eczema Vaccinatum in a Household Contact of a Smallpox Vaccinee

BACKGROUND We report the first confirmed case of eczema vaccinatum in the United States related to smallpox vaccination since routine vaccination was discontinued in 1972. A 28-month-old child with refractory atopic dermatitis developed eczema vaccinatum after exposure to his father, a member of the US military who had recently received smallpox vaccine. The father had a history of inactive eczema but reportedly reacted normally to the vaccine. The childs mother also developed contact vaccinia infection. METHODS Treatment of the child included vaccinia immune globulin administered intravenously, used for the first time in a pediatric patient; cidofovir, never previously used for human vaccinia infection; and ST-246, an investigational agent being studied for the treatment of orthopoxvirus infection. Serological response to vaccinia virus and viral DNA levels, correlated with clinical events, were utilized to monitor the course of disease and to guide therapy. Burn patient-type management was required, including skin grafts. RESULTS The child was discharged from the hospital after 48 days and has recovered with no apparent systemic sequelae or significant scarring. CONCLUSION This case illustrates the need for careful screening prior to administration of smallpox vaccine and awareness by clinicians of the ongoing vaccination program and the potential risk for severe adverse events related to vaccinia virus.

Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma.

Background/Objectives: There are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF). Materials and Methods: This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL). Results: The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%). Conclusions: Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrolment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).

Panniculitis in childhood.

Panniculitis refers to disorders with inflammation of the subcutaneous fat. Such inflammation can be primary or can be a reaction pattern induced by a systemic process. Some types of panniculitis are seen more commonly or exclusively in children. These include erythema nodosum, subcutaneous fat necrosis of the newborn, sclerema neonatorum, poststeroid panniculitis, and cold panniculitis. The most typical clinical finding is tender, erythematous subcutaneous nodules. Clinical clues can aid in the diagnosis of the panniculitides, but pathology is often necessary to confirm the diagnosis. In general, the pediatric panniculitides are treated with supportive care and management of any underlying disorders, but certain types such as infectious panniculitis and malignancy‐related panniculitis require more specific therapies.

Autoimmune bullous diseases in childhood

Autoimmune blistering disorders are a heterogeneous group of diseases that result from autoantibodies generated against target antigens found in the skin and mucous membranes. This process leads to a variety of disruptions in keratinocyte adhesion and cellular integrity, resulting in fluid accumulation and development of blisters. Physicians should have an appreciation and understanding of autoimmune blistering disorders in the pediatric population when formulating a differential diagnosis of a patient who presents with skin blistering. Early detection and discrimination between the varied autoimmune blistering disorders can change the course of treatment and outcome. Due to the similarity in clinical presentation among different diseases within this category, histopathologic evaluation and, especially, immunofluorescence studies are necessary to establish the definitive diagnosis.

Cutaneous reactions to vaccinations

Vaccinations are important for infectious disease prevention; however, there are adverse effects of vaccines, many of which are cutaneous. Some of these reactions are due to nonspecific inflammation and irritation at the injection site, whereas other reactions are directly related to the live attenuated virus. Rarely, vaccinations have been associated with generalized hypersensitivity reactions, such as erythema multiforme, Stevens-Johnson syndrome, urticaria, acute generalized exanthematous pustulosis, and drug hypersensitivity syndrome. The onset of certain inflammatory dermatologic conditions, such as lichen planus, granuloma annulare, and pemphigoid, were reported to occur shortly after vaccine administration. Allergic contact dermatitis can develop at the injection site, typically due to adjuvant ingredients in the vaccine, such as thimerosal and aluminum. Vaccinations are important to promote development of both individual and herd immunity. Although most vaccinations are considered relatively safe, there may be adverse effects associated with any vaccine. Cutaneous manifestations make up a large portion of the types of reactions associated with vaccines. There are many different reasons for the development of a cutaneous reaction to a vaccination. Some are directly related to the injection of a live attenuated virus, such as varicella or vaccinia (for immunity to smallpox), whereas others cause more nonspecific erythema and swelling at the injection site, as a result of local inflammation or irritation. Vaccinations have also been associated in rare reports with generalized hypersensitivity reactions, such as erythema multiforme, Stevens-Johnson syndrome, urticaria, acute generalized exanthematous pustulosis, and drug hypersensitivity syndrome. There have been case reports associating the administration of a vaccine with the new onset of a dermatologic condition, such as lichen planus, granuloma annulare, and Sweet syndrome. Finally, allergic contact dermatitis can develop at the injection site, typically due to adjuvant ingredients in the vaccine, such as thimerosal and aluminum.

Local Vaccine Site Reactions and Contact Allergy to Aluminum

Abstract:  Childhood vaccines are a routine part of pediatric care in the United States; clinicians must be able to recognize and interpret associated localized adverse reactions. Redness and induration at the site of injection are commonly reported and are considered to be the result of local inflammation or hematoma formation, although other atypical reactions can occur. We report the case of a 6‐month‐old infant who developed subcutaneous nodules at the sites of his 4‐ and 6‐month Pentacel (DTaP/Hib/IPV, diphtheria, tetanus, acellular pertussis, Haemophilus b conjugate, and inactivated poliovirus vaccine) and 6‐month Prevnar (heptavalent pneumococcal vaccine) injections. Infectious disease and immunodeficiency examinations were unremarkable. Aluminum contact allergy was considered, and contact allergy testing confirmed sensitivity to aluminum. Although rare, aluminum contact allergy after routine immunization can occur and should be considered in the differential diagnosis of persistent subcutaneous nodules after vaccination.

Narrowband UVB phototherapy as a novel treatment for Netherton syndrome

Netherton syndrome (NS) is a rare congenital ichthyosis that is characterized by impaired skin barrier function. Topical medications are cautiously used in NS since toxicity from systemic absorption is a major concern. Narrowband ultraviolet B phototherapy is an alternative therapeutic option that demonstrated its beneficial and practical use in a patient with NS.

Management of atopic dermatitis

of the Clinical Problem Atopic dermatitis is a chronic inflammatory skin disorder affecting approximately 10% of US adults and children.1,2 The condition is the result of multiple factors including a hyperstimulated cutaneous immune system, a genetically determined compromised skin barrier, and exposure to triggering environmental stimuli. Flares manifest as extreme pruritus of red, rough, flaky and often fissured regions of the skin that become chronically thickened, rough, and discolored. Atopic dermatitis can have a profound effect on the quality of life of patients and their families through effects on sleep, behavior, mood, and absences from school and work.3 The topical therapies discussed in this guideline4 are considered the first line of management.

Net‐like superficial vascular malformation: clinical description and evidence for lymphatic origin

DEAR EDITOR, The International Society for the Study of Vascular Anomalies classifies lymphatic malformations (LMs) as common (cystic) LMs (including macrocystic LM, microcystic LM and mixed cystic LM), generalized lymphatic anomaly, LM in Gorham–Stout disease, channel-type LM, primary lymphoedema (different types) and others (http://www.issva.org). We describe three patients with a distinctive presentation of LM that has not been previously recognized. One boy (case 1) and two girls (cases 2 and 3) had skin lesions that started at 5 years, 6 years and 18 months of age, respectively. The three cases were unrelated and all patients experienced progression of their lesions throughout the ensuing years. At times, partial regression was noted, but overall there was expansion of the area of involvement. The lesions consisted of large, irregularly shaped, angulated, bright red to red/brown to purple patches with a finely reticulated pattern of net-like or arborizing vascular structures. The lesions were located on the upper back (cases 1 and 2) extending to the left shoulder (case 2) and right buttock extending to the right thigh (case 3) (Figs 1–3). Some older areas of involvement lost the arborizing appearance and developed a more confluent rusty red to purple colour. Eventually a few red or purple sessile papules developed focally on the surface of the lesions in cases 2 and 3 (Figs 2, 3). The remainder of the physical examination disclosed no other abnormality in the three children. Dermoscopy in all cases showed deep red, reticulated, net-like or arborizing telangiectatic vessels, with no erythema between the dilated vessels (Figs 1, 2). The deep red colour of the vessels on dermoscopy contrasted with the bright red colour that is typically seen on dermoscopy of capillary malformations. Magnetic resonance imaging and ultrasound in all cases failed to reveal any dermal, subcutaneous or deeper vascular anomaly. Skin biopsies in all cases showed normal epidermis and many dilated thin-walled vessels in the upper dermis lined by a single layer of endothelial cells with prominent nuclei protruding into the lumens (Fig. 4a). These vessels were variably empty or contained blood cells or lymph. The mid-to-deep dermis and the subcutaneous tissue did not show any abnormality or the presence of dilated vessels. Immunohistochemistry demonstrated positivity for the lymphatic endothelial marker podoplanin (D2-40) in the cells lining the dilated vessels of the superficial dermis in all cases (Fig. 4b).

Management of Acne Vulgaris.

of the Clinical Problem About 50 million people in the United States have acne.1 Acne affects 85% of all adolescents and about 12% of adult women.2,3 Acne is a chronic inflammatory condition presenting as comedones (blackheads and whiteheads), papules, pustules, and nodules. It is caused by androgen-induced sebum production, follicular hyperkeratinization, and colonization of the folliculosebaceous unit by the Proprionibacterium acnes bacterium.4 Follicles become impacted with sebum because of follicular keratinization and then become distended, forming comedones. Proprionibacterium acnes growth in the follicle results in cytokine release, causing inflammatory lesions.5 Although it is a benign condition, acne can have considerable morbidity, including pain and discomfort, permanent scarring, and depression and anxiety resulting in poor self-esteem.2