[Transition from pemphigus vulgaris to pemphigus foliaceus: a case report]. / Transition phénotypique d'un pemphigus vulgaire en pemphigus superficiel.

BACKGROUND: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the two main subtypes of auto-immune pemphigus, each having different clinical, histological and immunopathological features. We report the case of a patient initially with typical PV who relapsed within 2years, presenting clinically, histologically and immunologically typical PF. PATIENTS AND METHODS: A 47-year old man presented in March 2008 with clinically, histologically and serologically typical PV and treated with systemic corticosteroids alone (prednisone: 1mg/kg per day) then combined with a cycle of rituximab, which resulted in complete remission. After discontinuation of therapy (duration: 26months), he relapsed 6 months later with PF presenting clinical, histological and serological characteristics typical of this condition. DISCUSSION: This is a rare case of complete transition from PV to PF in clinical, histological and serological terms, and the first case occurring after initial treatment with rituximab.

[Erosive pustular dermatosis of the legs: retrospective study of 16 cases]. / Dermatose érosive et pustuleuse de jambes : étude rétrospective de 16 cas.

BACKGROUND: Erosive pustular dermatosis of the leg (EPDL) is a chronic clinical entity comprising combined erosion, pustules and crusts on the legs. There is still some discussion of the independent existence of this condition in the absence of specific diagnostic criteria. The purpose of this study is to describe the clinical and laboratory characteristics of EPDL based on a series of patients presenting a clinical picture consistent with this diagnosis. PATIENTS AND METHODS: This retrospective study included all patients seen in our department between 2005 and 2009 presenting a clinical picture consistent with EPDL, in accordance with the initial description. We collated and carried out descriptive analysis of the clinical features and progression of the disease and of laboratory results (microbiology, immunology and vascular tests). RESULTS: In all of the 16 patients included (mean age: 81 years; sex ratio M/F: 0.2), lesions were consistently located in the middle third of the anterior aspect of the leg and associated with ochre dermatitis and skin atrophy; they were bilateral in 10 of the 16 patients. For the most part, laboratory tests were negative or inconclusive, with the exception of direct cutaneous immunofluorescence (DIF). DIF was performed in 14 patients and in three cases showed linear C3 deposits, thus confirming the diagnosis of pretibial bullous pemphigoid. In the 13 remaining cases, a diagnosis of idiopathic EPDL was made. Three of these 13 patients were either presenting or had previously presented squamous cell carcinoma of the leg. Topical corticosteroids were effective in 12 of these 13 cases (mean treatment duration: six months). Relapse was common (6/12). DISCUSSION: Our study demonstrates the need for skin biopsy with DIF for patients presenting a clinical picture evocative of EPDL, since the clinical presentation can be very similar to that of pretibial pemphigoid. Trophic disorders associated with venous stasis are common in EPDL, although they are difficult to interpret because of the high prevalence of this condition among the elderly. Mention must be made of associated marked sun damage, suggesting a possible relationship between EPDL and erosive pustular dermatosis of the scalp.

T-cell receptor gamma gene rearrangement in cutaneous T-cell lymphoma: comparative study of polymerase chain reaction with denaturing gradient gel electrophoresis and GeneScan analysis.

BACKGROUND: The usefulness of T-cell receptor gene rearrangement (TCR-GR) analyses for differentiating cutaneous T-cell lymphoma (CTCL) from benign inflammatory disorders (BID) has been insufficiently studied to date. OBJECTIVES: To evaluate the diagnostic value of TCR-GR analyses, comparing polymerase chain reaction (PCR) with denaturing gradient gel electrophoresis (DGGE) analysis and BIOMED-2 standardized protocol PCR with GeneScan analysis (BIOMED-2-GS). METHODS: Both types of PCR were performed in 157 patients evaluated for initial features suggestive of CTCL between 1996 and 2007. After clinical and histological review, the final diagnosis was CTCL in 77 cases and BID in 80 cases. RESULTS: DGGE and BIOMED-2-GS had a similar diagnostic value for distinguishing CTCL from BID, with a sensitivity of 74% and 77%, respectively, and a specificity of 86%. The observed concordance between both methods was 90% and the kappa coefficient was 0.79. Positivity rates did not depend on the PCR method but varied according to the type of CTCL (73-75% in mycosis fungoides, 90-100% in Sézary syndrome, 40-60% in lymphomatoid papulosis and 100% in other types). The positivity rate in BID was 14% with both methods. The most frequent BID with a monoclonal pattern were drug-induced cutaneous lymphoid hyperplasia, erythrodermic psoriasis and pityriasis lichenoides chronica. CONCLUSIONS: BIOMED-2-GS analysis of the TCRgamma gene is as sensitive and specific as DGGE for CTCL diagnosis. In addition, BIOMED-2-GS is less time-consuming and gives more information concerning the size and nature of TCR-GR.