Linear IgA bullous dermatosis

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Linear IgA bullous dermatosis
Linear IgA bullous dermatosis.jpg
(a) Widespread vesiculobullous eruption on the lower limbs with elements in a “string of pearls” arrangement;
(b) targetoid vesicular lesions on erythematous skin involving the dorsa of the hands;
(c) blisters with a “string of pearls” configuration and crusts in the perioral area;
(d) erythematous, vesicular lesions partially eroded on the posterior aspects of both thighs;
(e) vesicles involving the vulvar area in a child.[1]
SpecialtyImmunology, dermatology Edit this on Wikidata

Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected.[2]:135 It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:[3]:587

  • Adult linear IgA disease is an acquired, autoimmune blistering disease that may present with a clinical pattern of vesicles indistinguishable from dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance.[2] This disease can often be difficult to treat even with usually effective medications such as rituximab.[4]
  • Childhood linear IgA disease (also known as "Chronic bullous disease of childhood") is an acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13.[2]

See also[edit]

References[edit]

  1. ^ Genovese, Giovanni; Venegoni, Luigia; Fanoni, Daniele; Muratori, Simona; Berti, Emilio; Marzano, Angelo Valerio (2019). "Linear IgA bullous dermatosis in adults and children: a clinical and immunopathological study of 38 patients". Orphanet Journal of Rare Diseases. 14 (1): 115. doi:10.1186/s13023-019-1089-2. ISSN 1750-1172. PMC 6534856. PMID 31126328.
  2. ^ a b c James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  3. ^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  4. ^ He Y, Shimoda M, Ono Y, Villalobos IB, Mitra A, Konia T, Grando SA, Zone JJ, Maverakis E (2015). "Persistence of Autoreactive IgA-Secreting B Cells Despite Multiple Immunosuppressive Medications Including Rituximab". JAMA Dermatol. 151 (6): 646–50. doi:10.1001/jamadermatol.2015.59. PMID 25901938.

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