Robert A. Silverman

Guidelines of care for the management of atopic dermatitis: Section 1. Diagnosis and assessment of atopic dermatitis

Atopic dermatitis (AD) is a chronic, pruritic, inflammatory dermatosis that affects up to 25% of children andxa02% to 3% of adults. This guideline addresses important clinical questions that arise in the managementxa0and care of AD, providing updated and expanded recommendations based on the available evidence. In this first of 4 sections, methods for the diagnosis and monitoring of disease, outcomes measures for assessment, and common clinical associations that affect patients with AD are discussed.xa0Known risk factors for the development of disease are also reviewed.

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.

Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi: The “LAMB” syndrome

We describe a 13-year-old girl with a cardiocutaneous syndrome characterized by an atrial myxoma, pigmented lesions of the skin and genital mucosa, and opalescent papules and dermal nodules of the skin and tongue. Her pigmented lesions included black macules of the face and vulva, brown macules of the lips and perioral skin, multiple blue nevi, and a congenital nevomelanocytic nevus. The black and brown macules of the face and vulva consisted of lentiginous proliferations of large, intensely dopa-reactive melanocytes. The opalescent papules and dermal nodules had histologic, ultrastructural, and histochemical characteristics of myxomas. During follow-up, the patient developed thyroid nodules, which were composed of mixed papillary and follicular hyperplasia. This case emphasizes the necessity of a cardiac evaluation for a potentially fatal (and surgically treatable) atrial myxoma in individuals with multiple melanocytic and myxomatous tumors of the skin and mucosa.

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.

LUMBAR: Association between Cutaneous Infantile Hemangiomas of the Lower Body and Regional Congenital Anomalies

OBJECTIVEnTo define the clinical spectrum of regional congenital anomalies associated with large cutaneous hemangiomas of the lower half of the body, clarify risk for underlying anomalies on the basis of hemangioma location, and provide imaging guidelines for evaluation.nnnSTUDY DESIGNnWe conducted a multi-institutional, retrospective case analysis of 24 new patients and review of 29 published cases.nnnRESULTSnHemangiomas in our series tended to be segmental and often minimal growth in morphology. Such lesions were often extensive, covering the entire leg. Extensive limb hemangiomas also showed potential for extracutaneous anomalies, including underlying arterial anomalies, limb underdevelopment, and ulceration. The cutaneous hemangioma and underlying anomalies demonstrated regional correlation. Myelopathies were the most common category of associated anomalies.nnnCONCLUSIONSnWe propose the acronym LUMBAR to describe the association of Lower body hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies. There are many similarities between LUMBAR and PHACE syndrome, which might be considered regional variations of the same. Although guidelines for imaging are suggested, prospective studies will lead to precise imaging recommendations and help determine true incidence, risk and long-term outcomes.

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.

Abatacept and Sodium Thiosulfate for Treatment of Recalcitrant Juvenile Dermatomyositis Complicated by Ulceration and Calcinosis

We report the successful use of abatacept and sodium thiosulfate in a patient with severe recalcitrant juvenile dermatomyositis complicated by ulcerative skin disease and progressive calcinosis. This combination therapy resulted in significant reductions in muscle and skin inflammation, decreased corticosteroid dependence, and halted the progression of calcinosis.

Effects of occlusive and semiocclusive dressings on the return of barrier function to transepidermal water loss in standardized human wounds

Clinical observation and histologic examination of excised wounds have confirmed that occlusive dressings promote rapid wound reepithelialization. However, normalization of barrier function has not been routinely assessed in studies of occlusive dressing effects on wound healing. We examined the effects of occlusive dressings on the reestablishment of the cutaneous barrier to transepidermal water loss (TEWL) after standardized skin wounds were produced in human subjects. We confirmed previous observations that occlusive dressings augment reepithelialization. No significant improvement in the rate of reestablishment of the barrier to TEWL was measured between the covered test or uncovered control sites in each subject, however. TEWL declined in an exponential fashion after wounding. Measurements of TEWL were over twice that of adjacent normal skin when epithelialization was judged to be overtly complete and did not return to normal until 4 weeks after wounds were produced.

Transient blueberry muffin appearance of a newborn with congenital monoblastic leukemia

A full-term male infant was born with skin findings suggesting a blueberry muffin appearance. Biopsy of a cutaneous nodule was consistent with monoblastic leukemia cutis, and bone marrow examination confirmed the diagnosis of leukemia. The infant has remained well 2 years after spontaneous resolution of the cutaneous eruption. Infiltrative neoplasms should be considered along with congenital infections and hematologic disorders in the differential diagnosis of a newborn with a blueberry muffin appearance.