C. Hotz

Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients

Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts.

Acute generalized exanthematous pustulosis: a retrospective audit of practice between 1994 and 2011 at a single centre

tosensitive rash that did not warrant vemurafenib withdrawal. This highlights the importance of careful patient monitoring, even though most vemurafenib-induced rashes resolve spontaneously. We chose to switch to dabrafenib because no crossreaction with vemurafenib had previously been described. Also, although this BRAF inhibitor has been used more recently than vemurafenib, cutaneous side-effects appear less frequent and less severe. Indeed, to our knowledge, no cases of TEN or DRESS have hitherto been reported with dabrafenib. To conclude, BRAF inhibitor molecules appear to have different skin toxicity profiles. When permanent discontinuation of vemurafenib is required after TEN, drug switching to dabrafenib appears to be a useful alternative treatment option.

Cutaneous Macroglobulinosis A Report of 2 Cases

BACKGROUND Specific cutaneous lesions of Waldenström macroglobulinemia are rare and include neoplastic cell infiltrates, IgM bullous disease, and so-called IgM-storage papules, which characterize cutaneous macroglobulinosis (CM). OBSERVATIONS We report 2 patients with CM. In patient 1, CM started as small papules, as reported in most of the previously published case studies of CM. In patient 2, lesion evolution was remarkable by its severity, with large ulcerated nodules, and the disease progressed rapidly. As mentioned for half the previously described patients, peripheral neuropathy was suspected in patient 2 and demonstrated in patient 1, with production of antibodies to myelin-associated glycoprotein. CONCLUSIONS To the best of our knowledge, rituximab treatment of Waldenström macroglobulinemia associated with CM has not been described previously. Rituximab caused complete remission of the lesions in patient 1, whereas disease rapidly progressed in patient 2, and the patient died. These observations suggest that evolution of the cutaneous IgM-storage lesions reflects that of the underlying Waldenström macroglobulinemia, and CM is not a prognostic marker.

Pulmonary cryptococcoma in a patient with Sézary syndrome treated with alemtuzumab

ejd.2011.1538 Auteur(s) : Claire Hotz1,a q claire.hotz@gmail.com, Saskia Ingen-Housz-Oro1,a, Jeanne Tran Van Nhieu2, Caroline Charlier3, Francoise Foulet4, Alain Rahmouni5, Benhalima Zegai5, Tu-Anh Duong1, Pierre Wolkenstein1, Martine Bagot6,a, Olivier Chosidow1,a 1 Department of Dermatology 2 Department of Pathology, Henri-Mondor hospital, 51 avenue du marechal-de-Lattre-de-Tassigny, 94010 Creteil, France 3 Department of Infectious Diseases, Necker–Enfants Malades Hospital, Paris, France 4 [...]

Epidermal necrolysis and autoimmune diseases: two more observations supporting the concept that ‘toxic’ epidermal necrolysis can be ‘non-toxic’

Toxic epidermal necrolysis (TEN, Lyell syndrome) and Stevens-Johnson syndrome are severe cutaneous adverse reactions to drugs characterized by epidermal necrolysis (EN). However, in 15% of cases, no causative drug is identified. In these cases, other triggers such as Mycoplasma pneumoniae have been described. Furthermore, the role of lupus as triggering factor has been suggested. We report 2 cases of EN without any drug causality, revealing autoimmune diseases as a cause. This article is protected by copyright. All rights reserved.