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Acquired hemophilia occurring during the course of linear IgA bullous dermatosis

Peternel, Sandra; Pejkic, Gabrijela; Saint-Georges, Valentina; Simonić, Edita; Grohovac, Dragana; Valković, Toni
Acquired hemophilia occurring during the course of linear IgA bullous dermatosis // Journal of the American Academy of Dermatology, Volume 79, Issue 3, Supplement 1
San Diego, Sjedinjene Američke Države, 2018. str. AB28-AB28 doi:10.1016/j.jaad.2018.05.155 (poster, međunarodna recenzija, sažetak, stručni)

Acquired hemophilia occurring during the course of linear IgA bullous dermatosis

Peternel, Sandra ; Pejkic, Gabrijela ; Saint-Georges, Valentina ; Simonić, Edita ; Grohovac, Dragana ; Valković, Toni

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Journal of the American Academy of Dermatology, Volume 79, Issue 3, Supplement 1 / - , 2018, AB28-AB28

2018 Annual Meeting of the American Academy of Dermatology

Mjesto i datum
San Diego, Sjedinjene Američke Države, 16-20.02.2018

Vrsta sudjelovanja

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Linear immunoglobulin A bullous dermatosis ; Acquired hemophilia A ; rituximab

Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune blistering skin disease characterized by deposition of IgA autoantibodies along the basement membrane zone (BMZ). Although LABD is most often idiopathic, it can be triggered by infections and drugs, or occur in association with other autoimmune diseases, lymphoproliferative disorders and internal malignancies. Acquired hemophilia A (AHA) is a rare and potentially life-threatening coagulation deficiency caused by the production of anti-factor VIII autoantibodies. Similarly to LABD, it has been associated with malignancies, drugs, or other autoimmune diseases. A 57-year-old male patient presented with sudden onset of pruritic vesicles and bullae distributed on the upper trunk, proximal extremities, axillary vaults and inguinal folds. Lesions were partially distributed in herpetiform fashion, resembling “cluster of jewels” or in annular and polycyclic arrangement creating the “string of beads sign”. There were also multiple erosions and vesicles on the oral mucosa and bilateral conjunctivitis. Histopathological findings of lesional skin showed subepidermal blister and a dermal infiltrate rich in neutrophils and eosinophils. Direct immunofluorescence analysis showed linear deposits of IgG and IgA, and focal granular deposits of C3 along the BMZ. Indirect immunofluorescence, ELISA for IgG antibodies against BP 180, Desmoglein 1 and 3, ANA and ENA screen were all negative. A diagnosis of LABD was made, and based on the patient history of recent introduction of perindopril, moxonidine and amlodipine, it was presumed to be drug-induced. A relatively rapid remission was achieved within a month, after discontinuation of the incriminated drugs and a course of systemic glucocorticoids. However, as the erosions healed, the patient developed melena and widespread suffusions, accompanied by tingling sensation and pain. Laboratory analyses revealed significantly prolonged activated partial thromboplastin time, a decrease of factor VIII activity and the presence of factor VIII inhibitors, findings indicative of AHA. Neither a combination of glucocorticoids and cyclophosphamide, nor intravenous immunoglobulin therapy demonstrated sufficient clinical response. Clinical and laboratory remission was finally achieved with rituximab. The coincidence of autoimmune bullous diseases and AHA is very rare, and to the best of our knowledge, this is the second reported case of AHA occurring in association with LABD. References: 1. Makita S et al. Acquired hemophilia associated with autoimmune bullous diseases: a report of two cases and a review of the literature. Intern Med. 2013 ; 52(7):807-10. 2. Arakaki O et al. Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia. J Dermatol 2008 ; 35(7):437-46. 3. Filipczak A et al. Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report. J Dermatol 2015 ; 42(6):638-41. 4. Qiu X et al. Acquired hemophilia associated with bullous pemphigoid: a case report. Int J Clin Exp Pathol 2012 ; 5(1):102-4.

Izvorni jezik

Znanstvena područja
Kliničke medicinske znanosti


Medicinski fakultet, Rijeka,
Klinički bolnički centar Rijeka

Časopis indeksira:

  • Current Contents Connect (CCC)
  • Web of Science Core Collection (WoSCC)
    • Science Citation Index Expanded (SCI-EXP)
    • SCI-EXP, SSCI i/ili A&HCI
  • Scopus