IgG4-Related Skin Disease Presenting as a Pseudolymphoma in a White Adolescent Girl.

Immunoglobulin gamma (Ig) type 4-related disease (IgG4-RD) is a chronic immunologic systemic disorder that has gained worldwide recognition in the past decade. This entity can affect almost every organ system, and its characteristic lesions have been found in a variety of organs such as lacrimal and salivary glands, pancreas, liver, bile ducts, lungs, kidneys, retroperitoneum, breast, aorta, thyroid, and prostate. This case reports a very rare case of IgG4-RD presenting with a cutaneous lesion in a young female patient mimicking a lymphoproliferative disorder. IgG4-RD affecting the skin is a rare entity and has mostly been reported in Japanese men of middle to older age. IgG4-RD with cutaneous involvement should be in the differential of non-neoplastic, lymphomas, autoimmune, and infectious disorders of the skin.

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multicenter Retrospective Study of 377 Adult Patients from the United States.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, severe mucocutaneous reaction with few large cohorts reported. This multicenter retrospective study included patients with SJS/TEN seen by inpatient consultative dermatologists at 18 academic medical centers in the United States. A total of 377 adult patients with SJS/TEN between January 1, 2000 and June 1, 2015 were entered, including 260 of 377 (69%) from 2010 onward. The most frequent cause of SJS/TEN was medication reaction in 338 of 377 (89.7%), most often to trimethoprim/sulfamethoxazole (89/338; 26.3%). Most patients were managed in an intensive care (100/368; 27.2%) or burn unit (151/368; 41.0%). Most received pharmacologic therapy (266/376; 70.7%) versus supportive care alone (110/376; 29.3%)-typically corticosteroids (113/266; 42.5%), intravenous immunoglobulin (94/266; 35.3%), or both therapies (54/266; 20.3%). Based on day 1 SCORTEN predicted mortality, approximately 78 in-hospital deaths were expected (77.7/368; 21%), but the observed mortality of 54 patients (54/368; 14.7%) was significantly lower (standardized mortality ratio = 0.70; 95% confidence interval = 0.58-0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and intravenous immunoglobulin (standardized mortality ratio = 0.52; 95% confidence interval 0.21-0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, although substantial, was significantly lower than predicted. Although the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and intravenous immunoglobulin, among other therapies, may warrant further study.

Eczematous dermatitis in an infantile hemangioma with minimal or arrested growth.

Infantile hemangiomas (IHs) with minimal or arrested growth differ from standard IHs in that they have a reduced or absent growth phase. An association between IHs with minimal or arrested growth and superimposed eczematous dermatitis has not been described in the literature. We present a case detailing this association in which an IH with minimal or arrested growth responded well to treatment with propranolol and the superimposed eczema cleared completely with topical hydrocortisone.