[Oesophageal bullous detachment with massive haemorrhage revealing acquired haemophilia A in autoimmune bullous dermatosis]. / Décollement bulleux de l'œsophage responsable d'une hémorragie massive révélant une hémophilie A acquise au cours d'une dermatose bulleuse auto-immune.

INTRODUCTION: Acquired haemophilia A (AHA) is a rare coagulopathy caused by the development of factor VIII antibodies. Various aetiologies have been established but a number of cases have been reported in association with autoimmune bullous dermatosis (AIBD). We report a new case of this type of association revealed by oesophageal involvement of AIBD. PATIENTS AND METHODS: A male patient was treated for AIBD. Due to the inefficacy of local steroids and the emergence of oral and laryngeal blisters, the patient was treated with systemic steroids. He developed a gastrointestinal haemorrhage complicated by haemorrhagic shock. Endoscopy revealed complete peeling of the oesophagus. Laboratory tests showed lengthening of ACT, reduced factor VIII levels, and the presence of anti-factor VIII antibodies. A diagnosis was made of AHA associated with AIBD. Prolongation of systemic corticosteroids and initiation of rituximab resulted in normalisation of haemostasis. DISCUSSION: AIBD and AHA frequently develop concomitantly, as was the case with our patient. The haemorrhagic complications were severe. The aim of AHA treatment is to stop acute bleeding and eliminate antibodies, and for this reason rituximab was chosen. CONCLUSION: Oesophageal bullous detachment is rare in AIBD but, as seen here, it may be responsible for massive haemorrhage, especially in the event of associated AHA. This feature underscores the need for evaluation of haemostasis in the early stages and during relapses for all patients with AIBD.

[Granulomatous slack skin associated with metastatic testicular seminoma]. / Mycosis fongoïde chalazodermique associé à un séminome testiculaire métastatique.

BACKGROUND: Granulomatous slack skin (GSS) is an extremely rare subtype of T-cell lymphoma, a variant of mycosis fungoides (MF). Herein, we describe the first reported case of GSS associated with metastatic testicular seminoma. PATIENTS AND METHODS: A 28-year-old male patient presented with circumscribed erythematous loose skin masses, especially in the body folds and which had been relapsing for 4years. Skin biopsy showed a loss of elastic fibers and an atypical granulomatous T-cell infiltrate with epidermotropism, enabling a diagnosis of GSS to be made. A biopsy of a retroperitoneal lymphadenopathy showed testicular seminoma metastasis. DISCUSSION: Patients suffering from GSS have a statistically higher risk of developing a second primary cancer, especially Hodgkin's lymphoma. The association found between GSS and a lymphoproliferative malignancy requires long-term follow-up and determines the patient's prognosis. CONCLUSION: It is not possible to prove a formal link between GSS and testicular seminoma. However, this case illustrates the value of screening for a second cancer, particularly where extra-cutaneous lesions appear during GSS treatment. Lymph node biopsy should be performed routinely in the event of GSS with possible lymph node involvement.