Cystic fibrosis (CF) is an autosomal recessive disease resulting from CFTR gene mutation and leading to defective epithelial chloride channel production. The classic clinical triad includes pulmonary disease, exocrine pancreatic insufficiency, and abnormally high sweat electrolyte concentrations. Rarely, patients may present with protein-energy malnutrition (PEM) characterized by edema, anemia, and hypoproteinemia. In this case report, Wenk et al describe a 3-month-old infant with dermatitis heralding the onset of PEM and the diagnosis of CF. Zinc, essential fatty acid, and protein deficiencies may contribute to the development of this eruption, and CF should be considered in the differential diagnosis for any infant presenting with an eruption and other signs of malnutrition.
Langerhans cell histiocytosis (LCH) is a group of diseases characterized by diverse clinical presentations and outcomes. In infancy, the self-regressive form (SR-LCH) is characterized by cutaneous lesions only with no systemic involvement. Unfortunately, cutaneous involvement is also the most common presentation of non–SR-LCH (NSR-LCH). In this observational retrospective series, Battistella et al suggest that some morphologic traits of the skin lesions (necrosis, solitary lesions, and extremity involvement) might indicate SR-LCH. While no difference was noted on routine histopathologic analysis between SR- and NSR-LCH, E-cadherin expression was a marker of LCH limited to the skin.
Debilitating migraine headaches affect 2% to 15% of the world's population. Clinical trials using botulinum toxin type A (BTX) for the treatment of hyperfunctional facial lines revealed a correlation between pericranial BTX injections and alleviation of migraine symptoms. Patient characteristics that may predict favorable treatment responses remain unclear. In this observational study of patients receiving BTX injections for cosmetic purposes, Kim et al demonstrated that patients with imploding (described as crushing and vicelike) and ocular (described as eye-popping) migraines were more responsive to BTX than those with exploding migraines (described as a buildup of pressure inside the head). These data support the hypothesis that BTX injections at doses appropriate for cosmetic purposes may be sufficient to prevent these migraine types.
Waldenström macroglobulinemia (WM) is a chronic lymphoproliferative disorder characterized by a monoclonal IgM secreted by malignant B cells. Cutaneous manifestations are rare and are divided into specific and nonspecific findings. Specific manifestations include neoplastic B-cell infiltrates and monoclonal IgM deposition. In this case series, Gressier et al describe 2 patients with WM and cutaneous macroglobulinosis (CM) that waxed and waned with the evolution of the underlying disease. Diagnosis of CM should prompt a search for associated peripheral neuropathy or associated autoimmune processes.
Antibodies to the humanized monoclonal IgG1 antibody and tumor necrosis factor antagonist adalimumab have been associated with nonresponse or loss of response in a substantial portion of patients with rheumatoid arthritis or Crohn disease. In this prospective observational cohort study, Lecluse et al demonstrated that antibodies to adalimumab are formed in a large portion of patients with plaque psoriasis treated with this drug. High titers of these antibodies were associated with impaired treatment outcomes, and the authors suggest testing for these antibodies when patients lose response to adalimumab because spontaneous improvement is unlikely to occur in this setting.
This Month in Archives of Dermatology. Arch Dermatol. 2010;146(2):121. doi:10.1001/archdermatol.2009.384