Juvenile dermatomyositis resembling late-stage Degos disease with gastrointestinal perforations successfully treated with combination of cyclophosphamide and rituximab: case-based review.

Dermatomyositis (DM) is a multi-system disease that results in chronic inflammation principally of the skin and striated muscle. Small blood vessel injury in the GI tract has been described in dermatomyositis, manifesting as bleeding, ulceration, pneumatosis intestinalis, and ultimately perforation. Recent histopathological studies have shown deposits in the capillaries of the skin, gastrointestinal tract, and brain of patients with dermatomyositis similar to that found in patients with Degos disease, suggesting these disease processes are closely related or represent varying degrees of severity on the same pathologic spectrum. We report a case of juvenile dermatomyositis (JDM) resembling late-stage Degos disease with gastrointestinal perforations successfully treated with combination rituximab and cyclophosphamide therapy. We systematically reviewed the literature detailing the medical and surgical treatments for gastrointestinal perforation in dermatomyositis, Degos-like dermatomyositis, and Degos disease. In addition to our case, as of October 2019, we identified 36 cases describing gastrointestinal perforation in patients with underlying dermatomyositis, 5 cases of Degos-like dermatomyositis and 17 cases of idiopathic Degos disease. Corticosteroid therapy was used widely for dermatomyositis and Degos-like dermatomyositis, while antiplatelet and anticoagulant medications were chiefly used for patients with idiopathic Degos disease. However, there were no cases that detailed the successful treatment of dermatomyositis or Degos disease with gastrointestinal perforation with rituximab alone or combined with cyclophosphamide. We report that rituximab, in combination with cyclophosphamide, can be used as a novel adjunctive therapy to successfully treat dermatomyositis with Degos-like gastrointestinal perforation.

Scanning Electron Microscopic Hair Shaft Analysis in Ectodermal Dysplasia Syndromes.

OBJECTIVES: The objective of the current study was to catalog hair shaft abnormalities in individuals with ectodermal dysplasia (ED) syndromes using scanning electron microscopy (SEM) and to compare the findings with those in unaffected controls. This is the second of a two-part study, the first of which used light microscopy as the modality and was previously published. METHODS: Scanning electron microscopy was performed in a blinded manner on hair shafts from 65 subjects with seven types of ED syndromes and 41 unaffected control subjects. Assessment was performed along the length of the shaft and in cross section. SETTING: Hair donations were collected at the 28th Annual National Family Conference held by the National Foundation for Ectodermal Dysplasia. Control subjects were recruited from a private dermatology practice and an academic children's hospital outpatient dermatology clinic. RESULTS: SEM identified various pathologic hair shaft abnormalities in each type of ED and in control patients. When hairs with all types of ED were grouped together and compared with those of control patients, the difference in the presence of small diameter and shallow and deep grooves was statistically significant (p < 0.05). When the EDs were separated according to subtype, statistically significant findings were also seen. CONCLUSION: SEM is a possible adjuvant tool in the diagnosis of ED syndromes. There are significant differences, with high specificity, between the hairs of individuals with ED and those of control subjects and between subtypes.

Gianotti-Crosti syndrome following childhood vaccinations.

A 19-month-old boy was evaluated for a skin eruption after recent vaccinations. Clinical and histopathologic findings supported a diagnosis of Gianotti-Crosti syndrome (GCS). This case report examines the link between GCS and vaccinations, particularly the diphtheria, tetanus, and pertussis vaccine and the varicella virus live vaccine.

Gianotti-Crosti syndrome after childhood vaccination.

A 19-month-old boy was evaluated for a skin eruption after recent vaccinations. Clinical and histopathologic findings supported a diagnosis of Gianotti-Crosti syndrome (GCS). This case report examines the link between GCS and vaccinations, particularly the diphtheria, tetanus, and pertussis vaccine and the varicella virus live vaccine.

Light microscopic hair shaft analysis in ectodermal dysplasia syndromes.

The objective of the study was to catalog hair shaft abnormalities in individuals with ectodermal dysplasia (ED) syndromes using light microscopy and to compare findings with those in unaffected controls. Light microscopy was performed in a nonblinded manner on hair shafts from 65 participants with seven types of ED (hypohidrotic ED, ED-ectrodactyly-cleft lip or palate, ankyloblepharon-ectodermal defects-cleft lip and palate, Clouston syndrome, Goltz syndrome, Schopf-Schulz Passarge syndrome, and oculodentodigital dysplasia) and 41 unaffected controls. Hair donations were collected at the 28th Annual National Family Conference held by the National Foundation for Ectodermal Dysplasia. Control participants were recruited from a private dermatology practice and an academic children's hospital outpatient dermatology clinic. Sixty-five affected participants and 41 unaffected controls were included in the analysis. We assessed the hair shafts of ED and control participants for abnormalities visible using LM. Light microscopy identified various pathologic hair shaft abnormalities in each type of ED, although none of the findings were statistically significantly different from those of the control group. Light microscopy is a poor adjuvant tool in the diagnosis of ED syndromes. Most findings are nonspecific and not sufficiently sensitive.

Potential barriers to adherence in pediatric dermatology.

This study sought to identify barriers to treatment in children with chronic inflammatory skin disease, particularly those with atopic dermatitis, psoriasis, and acne vulgaris. Caregivers of 101 patients seen in the Children's Specialty Group Division of Dermatology, Children's Hospital of The King's Daughters, Norfolk, Virginia, completed an 11-item Likert scale questionnaire. This survey addressed complexity and time requirements for treatment, medication cost, vehicle formulation, perceived safety, and caregiver understanding of chronicity of skin disorders. Parents and caregivers indicated that adequate instructions for using the medications were provided but that they felt less comfortable with treating their child's skin disease during a severe flare. The complexity of treatment programs, time required to apply medications, and vehicle type were not considered prohibitive factors. Caregivers were concerned about the cost and safety of prescribed medications and had a less understanding of the chronicity of inflammatory skin disorders.

"Hot tub" Folliculitis from a nonchlorinated children's pool.

Pseudomonas aeruginosa is common cause of folliculitis following contact with contaminated water. We report a case of pseudomonal folliculitis that occurred after swimming in a children's pool filled with water from a well.

Scurvy in a 10-year-old boy.

Scurvy, or hypovitaminosis C, is an uncommon condition that exists today primarily within certain unique populations-particularly the elderly subjects, patients with neurodevelopmental disabilities or psychiatric illnesses, or others with unusual dietary habits. Vitamin C is an essential nutrient in the human body, and is important in synthesizing collagen factor whose faulty production is responsible for most of the clinical manifestations of scurvy. These clinical manifestations can include dystrophic or corkscrew hairs, gingival hyperplasia, and weakened blood vessel walls, causing bleeding in the skin, joints, and other organs. Although rare in the Unites States, the presence of scurvy should not be forgotten because of its presence among susceptible populations. Moreover, with its diagnosis, treatment and cure is one of the simplest in modern medicine. We report a case of scurvy in a 10-year-old autistic child.

Exploring the differential diagnosis of hemorrhagic vesicopustules in a newborn.

Hemorrhagic vesicles in a newborn present a challenging differential diagnosis including both infectious and neoplastic disorders. Patients should be evaluated in an efficient manner to arrive at the correct diagnosis as quickly as possible. We present here an interesting case that outlines the methodical workup that ultimately revealed the diagnosis of congenital Langerhans cell histiocytosis. After a diagnosis of Langerhans cell histiocytosis is made, it is important to evaluate the patient thoroughly for systemic involvement. Historically, the diagnosis of congenital self-healing Langerhans cell histiocytosis was used to delineate a benign self-limited disorder limited to the skin with spontaneous resolution during the first several months of life; this disorder may also be referred to as "self-regressive Langerhans cell histiocytosis." However, some newborns with initial skin-only Langerhans cell histiocytosis progress to have multisystem disease after spontaneous resolution has occurred. For this reason, the nomenclature is changing. We suggest using the term "skin-only Langerhans cell histiocytosis." Periodic long-term follow-up is recommended to monitor for relapse or progression to systemic disease.

A randomized, double-blind study comparing the efficacy of selenium sulfide shampoo 1% and ciclopirox shampoo 1% as adjunctive treatments for tinea capitis in children.

Our objective was to compare the efficacy of selenium sulfide shampoo 1% and ciclopirox shampoo 1% as adjunctive treatments for tinea capitis in children. Forty children aged 1-11 years with clinically diagnosed tinea capitis were randomized to receive selenium sulfide shampoo 1% or ciclopirox shampoo 1% twice a week as adjuncts to an 8-week course of ultramicronized griseofulvin dosed at 10-12 mg/kg/day. At weeks 2, 4, and 8, subjects returned to the clinic for evaluation and scalp cultures. Subjects then returned for follow-up visits 4 weeks after completing treatment. Overall, by 8 weeks, 30 of 33 (90.9%) treated children demonstrated mycological cure. Selenium sulfide shampoo 1% and ciclopirox shampoo 1% were equally effective as adjunctive treatments for tinea capitis in children in our study.

Two reports of phacomatosis pigmentovascularis type IIb, one in association with Sturge-Weber syndrome and Klippel-Trenaunay syndrome.

We present two rare cases of phacomatosis pigmentovascularis type IIb, with one patient demonstrating concurrent Sturge-Weber syndrome and Klippel-Trenaunay syndrome. To the best of our knowledge, this is the second infantile case meeting diagnostic criteria for systemic phacomatosis pigmentovascularis type IIb, Sturge-Weber syndrome and Klippel-Trenaunay syndrome in the English language literature.

Sweet syndrome in infancy.

Sweet syndrome, or acute febrile neutrophilic dermatosis, is characterized by the presence of fever, peripheral leukocytosis, painful erythematous plaques and nodules, and a predominately neutrophilic dermal infiltrate. We report a case occurring in a 10-week-old male child, with preceding upper respiratory tract, and gastrointestinal infection symptoms. Sweet syndrome occurring in an infant should prompt a work-up for immunodeficiency, as well as a review of the peripheral blood smear to rule out the rare case of malignancy.

In vitro pharmacodynamic characteristics of griseofulvin against dermatophyte isolates of Trichophyton tonsurans from tinea capitis patients.

Tinea capitis is the most commonly observed fungal infection in childhood and is primarily caused by the dermatophyte species Trichophyton tonsurans, Microsporum canis, and Trichophyton violaceum. In North America and the United Kingdom T. tonsurans is responsible for more than 90% of cases. Griseofulvin has been the treatment of choice for tinea capitis for more than 40 years and is the sole oral antifungal agent approved by the FDA for the management of tinea capitis. Some researchers have expressed concern about the possibility of emerging resistance in tinea capitis isolates, especially when there is clinical failure to treatment. A total of 151 isolates of T. tonsurans (142), M. canis (7), and T. violaceum (2) collected from tinea capitis patients were evaluated for their susceptibility to griseofulvin using the CLSI M38-A method. MIC ranges and geometric means in parenthesis were observed for T. tonsurans 0.125-16 microg/ml (1.1 microg/ml), M. canis 0.25-2 microg/ml (0.61 microg/ml), and T. violaceum 2-4 microg/ml (2.82 microg/ml), respectively. In a time kill assay with T. tonsurans UAMH 9334, 50% and 90% reduction was observed in the number of colony forming units with >2x MIC after 6 h and 12 h of exposure to the griseofulvin, respectively. Of 142 T. tonsurans isolates studied, only three could grow on SDA containing 4 times to their griseofulvin MIC, representing resistance frequencies of 1.3 x 10(-6), 6.9 x 10(-7), and 9.7 x 10(-7). Furthermore a two-fold increase in MIC was observed in isolates collected at two time intervals in only one of eight patients. Interestingly, these isolates did not show the same increase in their in vitro resistance as exhibited by the three isolated mentioned above. In light of this data, we could not confirm any correlation between increased MIC and therapy failure.

Congenital herpes simplex virus infection: two unique cutaneous presentations associated with probable intrauterine transmission.

Cutaneous manifestations of congenital herpes simplex virus (HSV) have been classically described as grouped vesicles on an erythematous base. We report two cases of HSV infection wherein both infants presented at birth with widespread erosions and an absence of vesicles or vesicopustules. The presence of skin lesions at birth, neurologic changes seen on radiographic imaging, and a cesarean section delivery in one case suggests intrauterine transmission in both neonates.

Human papillomavirus vaccine: recommendations, issues and controversies.

PURPOSE OF REVIEW: The human papillomavirus vaccine is now Food and Drug Administration-approved for girls and young women ages 9-26 years and Advisory Committee on Immunization Practices-recommended for girls ages 11-12 years of age. Several issues have shrouded its debut and continue to provide controversy that may hinder effective delivery of this vaccine to young people in industrialized as well as third world countries. RECENT FINDINGS: The quadrivalent and divalent vaccines are compared, particularly with respect to juvenile recurrent respiratory papillomatosis. Recommendations for administration are summarized. Immunization of men and cost effectiveness are discussed. Issues regarding compulsory vaccination and attitudes of parents about human papillomavirus vaccination are reviewed. SUMMARY: Studies of the human papillomavirus vaccine are very promising, showing excellent efficacy and very few adverse events. It remains to be determined if it will be licensed in the United States for use in boys and men. Some stumbling blocks to implementing the vaccine in the United States need to be addressed in order to gain widespread acceptance.