Wynnis L. Tom

Guidelines of care for the management of atopic dermatitis: section 2. Management and treatment of atopic dermatitis with topical therapies.

Atopic dermatitis is a common and chronic, pruritic inflammatory skin condition that can affect all age groups. This evidence-based guideline addresses important clinical questions that arise in its management. In this second of 4 sections, treatment of atopic dermatitis with nonpharmacologic interventions and pharmacologic topical therapies are reviewed. Where possible, suggestions on dosing and monitoring are given based on available evidence.

Guidelines of care for the management of atopic dermatitis: Section 3. Management and treatment with phototherapy and systemic agents

Atopic dermatitis is a chronic, pruritic inflammatory dermatosis that affects up to 25% of children and 2% to 3% of adults. This guideline addresses important clinical questions that arise in atopic dermatitis management and care, providing recommendations based on the available evidence. In this third of 4 sections, treatment of atopic dermatitis with phototherapy and systemic immunomodulators, antimicrobials, and antihistamines is reviewed, including indications for use and the risk-benefit profile of each treatment option.

Guidelines of care for the management of atopic dermatitis: Section 4. Prevention of disease flares and use of adjunctive therapies and approaches

Atopic dermatitis is a common, chronic inflammatory dermatosis that can affect all age groups. This evidence-based guideline addresses important clinical questions that arise in its management. In this final section, treatments for flare prevention and adjunctive and complementary therapies and approaches are reviewed. Suggestions on use are given based on available evidence.

Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon

Propranolol is a non‐selective beta‐adrenergic antagonist successfully used in a case of kaposiform hemangioendothelioma (KHE) associated with Kasabach–Merritt phenomenon (KMP). We report 11 patients treated with propranolol for KHE and the related variant tufted angioma (TA), six of whom also had KMP. The varied responses to treatment, with only 36% responding in our series, demonstrate the need for further study of this medication before routine use for these indications. Pediatr Blood Cancer 2012; 59: 934–938.

Prospective study of infantile haemangiomas: incidence, clinical characteristics and association with placental anomalies†

The aetiology and exact incidence of infantile haemangiomas (IHs) are unknown. Prior studies have noted immunohistochemical and biological characteristics shared by IHs and placental tissue.

Report from the third international consensus meeting to harmonise core outcome measures for atopic eczema/dermatitis clinical trials (HOME)

This report provides a summary of the third meeting of the Harmonising Outcome Measures for Eczema (HOME) initiative held in San Diego, CA, U.S.A., 6–7 April 2013 (HOME III). The meeting addressed the four domains that had previously been agreed should be measured in every eczema clinical trial: clinical signs, patient‐reported symptoms, long‐term control and quality of life. Formal presentations and nominal group techniques were used at this working meeting, attended by 56 voting participants (31 of whom were dermatologists). Significant progress was made on the domain of clinical signs. Without reference to any named scales, it was agreed that the intensity and extent of erythema, excoriation, oedema/papulation and lichenification should be included in the core outcome measure for the scale to have content validity. The group then discussed a systematic review of all scales measuring the clinical signs of eczema and their measurement properties, followed by a consensus vote on which scale to recommend for inclusion in the core outcome set. Research into the remaining three domains was presented, followed by discussions. The symptoms group and quality of life groups need to systematically identify all available tools and rate the quality of the tools. A definition of long‐term control is needed before progress can be made towards recommending a core outcome measure.

Oral azathioprine for recalcitrant pediatric atopic dermatitis: Clinical response and thiopurine monitoring

BACKGROUND Azathioprine is prescribed as a corticosteroid-sparing agent for many inflammatory conditions, including refractory atopic dermatitis (AD). There are limited prospective data on its appropriate use and monitoring for children with AD. OBJECTIVES This study was designed to assess clinical response to azathioprine, determine the necessity for repeated measurement of thiopurine methyltransferase (TPMT) activity during treatment, and test the utility of measuring levels of the metabolites 6-thioguanine nucleotide and 6-methylmercaptopurine. METHODS Twelve children with severe, recalcitrant AD were treated with oral azathioprine and followed prospectively. Disease severity was determined by the SCORing AD index. Baseline TPMT activity was measured and this was repeated along with 6-thioguanine nucleotide and 6-methylmercaptopurine measurement at times of stable improvement, inadequate response, or change in response. RESULTS Azathioprine therapy was associated with clinical improvement in all but 1 patient. There were few adverse effects. Three patients showed a significant change in TPMT activity during treatment: 2 had a mild decrease and 1 demonstrated enzyme inducibility with an increase from the intermediate to the normal activity range. These changes, but not 6-thioguanine nucleotide or 6-methylmercaptopurine levels, inversely correlated with the clinical response to therapy. LIMITATIONS Small sample size is a limitation. CONCLUSIONS Azathioprine can be of benefit in the treatment of recalcitrant pediatric AD. Repeat assessment of TPMT activity may be helpful for evaluation of nonresponse or change in response and warrants further study. In contrast, measurement of thiopurine metabolites during treatment was not clinically useful.

Clinical Manifestations of Pediatric Psoriasis: Results of a Multicenter Study in the United States

The clinical features of pediatric psoriasis warrant further attention. A national study was conducted to determine the prevalence of scalp and nail involvement and a history of guttate psoriasis at onset according to age, sex, and disease severity. One hundred eighty‐one children ages 5 to 17 years with plaque psoriasis were enrolled in a multicenter, cross‐sectional study. Subjects and guardians were asked about a history of scalp and nail involvement and whether the initial presentation was guttate. Peak psoriasis severity was assessed and defined historically as mild psoriasis (MP) or severe psoriasis (SP) according to the Physicians Global Assessment and body surface area measures. One hundred forty‐three (79.0%) subjects reported a history of scalp involvement, and 71 (39.2%) described a history of nail involvement. Boys were less likely than girls to report a history of scalp involvement (odds ratio [OR] = 0.40, 95% confidence interval [CI] = 0.19–0.84) but more likely to have had nail involvement (OR = 3.01, 95% CI = 1.62–5.60). Scalp and nail involvement was not related to psoriasis severity. In contrast, subjects with SP (35.9%) more often reported a history of guttate lesions than did those with MP (21.8%) (p = .02). Antecedent streptococcal infection was more common in children with guttate than those with plaque psoriasis at onset (p = .02) but did not correlate with severity. Sex‐related differences in scalp and nail involvement suggest koebnerization. Preceding streptococcal infection predicts guttate morphology but not severity, and initial guttate morphology is associated with eventual greater severity of disease. More aggressive monitoring and management should be considered for guttate psoriasis, given its later association with more severe disease.

Pediatric "STUMP" lesions: evaluation and management of difficult atypical Spitzoid lesions in children.

Spitz nevi represent a distinct type of melanocytic nevi more commonly seen in childhood. Although typically benign, a subset of Spitz lesions raise concern and create a diagnostic dilemma as a result of confusing histology that involves characteristics of classic Spitz nevi intermixed with features of cutaneous melanoma. Such atypical Spitz lesions, or Spitzoid tumors of uncertain malignant potential, are difficult to classify and their biologic potential is uncertain. Nonetheless, these are critical tasks for both prognosis and clinical management. New tools, such as immunohistochemical stains, comparative genomic hybridization, and fluorescence in situ hybridization, have been used to provide further insight into these controversial lesions and to aid in their evaluation. In this review, we present our experience managing 6 cases of Spitzoid tumor of uncertain malignant potential and discuss the potential use of various diagnostic modalities, including sentinel lymph node biopsy, immunostaining, and molecular analysis.

Management of Pilomatrix Carcinoma: A Case Report of Successful Treatment with Mohs Micrographic Surgery and Review of the Literature

Pilomatrix carcinoma is a rare malignant variant of pilomatrixoma, a cutaneous adnexal tumor that originates from hair matrix cells. Reports of similar lesions date as far back as 1927, but Lopansri and Mihm first defined the tumor in 1980 as an aggressive form of pilomatrixoma with a tendency toward recurrence. Pilomatrix carcinoma has also been referred to in the literature as pilomatrical carcinoma, malignant pilomatricoma, malignant pilomatrixoma, matrical carcinoma, and calcifying epitheliocarcinoma of Malherbe. Lesions occur most commonly on severely sun-damaged skin and may arise through transformation of a pilomatrixoma or as a solitary, newly formed entity. These tumors are most frequently located on the head, neck, and back, although occurrence on the anterior trunk and upper and lower extremities has also been reported. Pilomatrix carcinoma manifests clinically as an asymptomatic mass that may resemble basal cell carcinoma, epidermal cyst, trichilemmal cyst, or pilomatrixoma. Lesions may arise over a period of months to many years, with a propensity for aggressive local recurrence and metastasis. Given its rarity, an optimal treatment regimen for pilomatrix carcinoma has not been established. Recurrence rates of >50% have been found after simple excision, with follow-up time ranging from a few months to many years. Most publications advocate wide local excision, with recommended margins varying between 5 mm and 2 cm. Mohs micrographic surgery (MMS) has been shown to be the most effective therapy for a number of rare malignant cutaneous tumors, including rare adnexal tumors such as sebaceous carcinoma and microcystic adnexal carcinoma. In 2004, Sable and Snow first reported on the successful management of pilomatrix carcinoma using MMS, with no evidence of recurrence at follow-up 5 months later. Given the tendency for aggressive local recurrence of pilomatrix carcinoma and the superior margin control available with MMS, MMS may represent an ideal treatment modality for this rare condition. Here, we report on a second case of pilomatrix carcinoma treated with MMS and provide a comprehensive literature review of the epidemiology, pathogenesis, clinical presentation, histopathologic findings, prognosis, follow-up, and management options for pilomatrix carcinoma.