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This Month in Archives of Dermatology
Arch Dermatol. 2009;145(3):239.
The Misnomer "Macrocephaly–Cutis Marmorata Telangiectatica Congenita Syndrome"
Macrocephaly–cutis marmorata telangiectatica congenita syndrome is characterized by macrocephaly and cutaneous vascular anomalies. In this case series of 12 new patients, Wright et al demonstrate that all patients showed reticulated or confluent port-wine stains or persistent central facial vascular stains (salmon patches) rather than cutis marmorata telangiectatica congenita. The authors convincingly demonstrate that the term macrocephaly–capillary malformations more accurately reflects the clinical features of this syndrome.
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Prevalence of Adrenal Insufficiency Following Systemic Glucocorticoid Therapy in Infants With Hemangiomas
Hemangiomas are common tumors of the vascular endothelium found in approximately 10% of infants, most typically in the first 2 months of life. The lesions undergo a proliferative phase lasting 6 to 12 months, and because most of them resolve spontaneously, no specific therapy is required. However, large, disfiguring, or functionally debilitating lesions require medical intervention, often with high-dose systemic glucocorticoids. In this 18-month prospective trial, Lomenick et al demonstrate that infants with hemangiomas treated with prednisolone were at low risk of adrenal insufficiency as measured by a combination of low-dose/high-dose corticotropin stimulation tests.
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Evaluation of the Efficacy of Acitretin Therapy for Nail Psoriasis
Psoriasis affects the fingernails or toenails in up to 78% of patients, manifesting with pitting, salmon-colored patches on the nail bed; onycholysis; nail-bed hyperkeratosis; nail thickening and crumbling; paronychia; and, rarely, leukonychia and erythema of the lunula. Pain, poor cosmetic appearance, and restricted activities of daily living can impair the quality of life of patients with psoriatic nail involvement. In this open study of 36 patients with moderate to severe nail psoriasis, Tosti et al demonstrate that low-dose acitretin therapy resulted in lowered Nail Psoriasis Severity Index scores, on par with biological agents. These data suggest that low-dose systemic acitretin should be considered in the treatment of nail psoriasis.
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Successful Treatment of Complex Aphthosis With Colchicine and Dapsone
Recurrent aphthous stomatitis (RAS) is the most common inflammatory ulcerative condition of the oral mucosa. Simple aphthosis involves a few to several bouts of aphthae yearly, with distinct ulcer-free periods. Complex aphthosis refers to a small subset of RAS cases with an almost constant presence of multiple oral ulcers, recurrent genital aphthae, and exclusion of Behçet syndrome. Although systemic corticosteroids offer symptomatic relief and ulcer healing, serious risks are associated with long-term use. In this retrospective review, Lynde et al demonstrate that most patients with complex aphthosis did well with colchicine and dapsone, either alone or in combination. These 2 well-established drugs should be considered important "rungs" on the therapeutic ladder for complex aphthosis.
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Lupus Erythematosus Tumidus
Lupus erythematosus tumidus is a highly photosensitive skin disorder that represents a subtype of cutaneous lupus erythematosus. Edematous, annular, urticarial papules and plaques are distributed in sun-exposed areas and tend to resolve without the scarring and pigmentary changes of discoid or subacute cutaneous lupus. Although no treatment guidelines exist, antimalarial drugs are commonly recommended as first-line systemic therapy. In this retrospective study, Kreuter et al demonstrate that 3 months of treatment with chloroquine phosphate or hydroxychloroquine sulfate resulted in a significant reduction in the Cutaneous Lupus Erythematosus Disease Area and Severity Score. Smokers responded less well, and these data suggested that patients who smoke should be encouraged to join smoking cessation programs because they will respond better to antimalarial treatment.
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SECTION EDITOR: ROBIN L. TRAVERS, MD
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