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This Month in Archives of Dermatology
May 2006

This Month in Archives of Dermatology

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Copyright 2006 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2006

Arch Dermatol. 2006;142(5):550. doi:10.1001/archderm.142.5.550
Development of Atopic Dermatitis During the First 3 Years of Life

Atopic dermatitis (AD) is a chronic, relapsing, inflammatory skin disease, which typically presents within the first years of life. In this prospective, longitudinal, birth cohort study of high-risk children born to atopic mothers, Brydensholt Halkjœr et al describe the progression of AD during the first 3 years of life. The cumulative incidence rate was 40% by age 3 years. In infants, the progression of skin lesions was found to begin at the scalp, forehead, ear, and neck in a balaclava-like pattern. Lesions continued to the extensor extremities, the face and trunk, and finally the flexor extremities. Dermatitis at the arms and around the joints had the highest predictive value for the diagnosis of AD by age 3 years, thus giving new insight into early prediction of AD and improving the ability to diagnose and intervene early.

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Randomized Controlled Trial of Adjuvant Oral Dexamethasone Pulse Therapy in Pemphigus Vulgaris

Pemphigus vulgaris (PV) is a potentially fatal, chronic, autoimmune mucocutaneous bullous disease. Systemic corticosteroids (CS) remain the mainstay of therapy. Pulse therapy with CS for PV was initially used in the 1980s, delivering discontinuous intravenous infusions of very-high-dose CS over a short time. However, no double-blind, placebo-controlled trials have ever demonstrated the efficacy of such regimens. In this multicenter, randomized, double-blind, placebo-controlled trial, Mentink et al evaluated the efficacy of adjuvant CS pulse therapy in addition to a conventional regimen of daily prednisolone and azathioprine in patients with PV and showed no benefit, thus arguing against the use of pulse CS therapy in this patient population.

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Staging Accuracy in Mycosis Fungoides and Sézary Syndrome Using Integrated Positron Emission Tomography and Computed Tomography

Mycosis fungoides (MF) is a rare non-Hodgkin lymphoma of T-cell origin that primarily involves the skin. Accurate staging of MF is important not only for prognostic purposes but also to determine appropriate treatment. Traditional staging involves skin and general physical examination, with a contrast computed tomographic (CT) scan of the chest, abdomen, and pelvis. However, CT scan–based staging provides anatomic information only and cannot distinguish between nonspecific inflammatory changes and malignant infiltration. In addition, false-negative test results may be seen when involved lymph nodes have not become sufficiently enlarged to meet the CT size criteria for a pathologic lymph node. In this single-center prospective cohort analysis, Tsai et al demonstrate that positron emission tomography may offer improved sensitivity and specificity in the detection of nodal involvement in MF.

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Kindler Syndrome: A New Mutation and New Diagnostic Possibilities

The constellation of clinical findings that constitute Kindler syndrome (KS) include neonatal acral bullae, photosensitivity, skin fragility, and later development of progressive poikiloderma. In 2003, KS mapped to the short arm of chromosome 20, where several mutations in a gene called KIND1 have been demonstrated. KIND1 encodes kindlin-1, a novel protein involved in actin cytoskeleton-extracellular matrix interactions. In this case report, Burch et al describe a patient with KS in whom immunostaining with a new antibody directed against kindlin-1 showed markedly reduced labeling in the affected child's skin. Ultrastructural clues to the diagnosis of KS were demonstrated in a skin biopsy specimen taken at age 10 months, suggesting that electron microscopy can enable the accurate early diagnosis of KS even if antibodies to kindlin-1 are not readily available.

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Clinical features of KS. A, Patient at age 7 years: notable healing blisters on the nose, with facial telangiectasia. B, Fine wrinkling of the skin and brownish keratoses on the forearms and dorsal area of the hands developed between age 6 and 7 years.

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Mucocutaneous Neuromas: An Underrecognized Manifestation of PTEN Hamartoma-Tumor Syndrome

The spectrum of clinical findings associated with germline mutations in PTEN, a tumor suppressor gene, is referred to as the PTEN hamartoma-tumor syndrome (PHTS). Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, and some cases of Proteuslike syndrome fall into this spectrum. In this case report, Schaffer et al describe a 5-year-old boy with macrocephaly, prominent corneal nerves, and the progressive development of multiple painful, dome-shaped, translucent pink to skin-colored papules at the vermilion border, fingers, palms, and shins. Genetic analysis revealed a novel heterozygous nonsense mutation in PTEN. This is the first report of multiple neuromas as the sole mucocutaneous manifestation of PHTS.

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