Rheumatoid Arthritis: Lipsker D

Kieffer C, Cribier B, Lipsker D. · The Université Louis Pasteur, Hôpitaux Universitaires, Strasbourg, France. · Medicine (Baltimore). · Pubmed #19352297 No free full text.

Abstract: We conducted the current study to define within the spectrum of the neutrophilic dermatoses a group of patients with an urticarial rash clinically and a neutrophilic dermatosis histopathologically. We reviewed the literature on neutrophilic urticaria and we report here a series of patients with this unique presentation. We reviewed all cutaneous biopsies submitted to our department between 2000 and 2006 in which histopathologic evaluation was compatible with this entity. We then retrieved the patient medical records and obtained information about follow-up and associated diseases. This allowed us to identify 9 patients with an urticarial eruption that was characterized histopathologically by a perivascular and interstitial neutrophilic infiltrate with intense leukocytoclasia but without vasculitis and without dermal edema. Four patients also had small foci of necrobiotic collagen bundles. The eruption consisted of pale, flat or only slightly raised, nonpruritic macules, papules, or plaques. Elementary lesions resolved within 24 hours. Purpura, angioedema, and facial swelling were not seen, but dermographism was present in 1 patient. Six patients had fever, 7 had polyarthritis, and 6 had leukocytosis. Seven patients had associated systemic diseases: adult-onset Still disease (3 patients), systemic lupus erythematosus (3 patients), and Schnitzler syndrome (1 patient).A similar rash has been reported previously in the literature, mostly in patients with systemic inflammatory diseases, but the majority of patients reported under the undefined designation of "neutrophilic urticaria" did have a different clinicopathologic presentation. Thus, we suggest naming this eruption "neutrophilic urticarial dermatosis," to emphasize that this entity expands the broad group of cutaneous manifestations of neutrophilic aseptic disease. This entity bears important medical significance as it is strongly indicative of an associated systemic disease, mainly Schnitzler syndrome, adult-onset Still disease, lupus erythematosus, and the hereditary autoinflammatory fever syndromes.

Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. · Clinique Dermatologique, Hôpitaux Universitaires, Strasbourg, France. · Medicine (Baltimore). · Pubmed #11204501 No free full text.

Abstract: The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. Patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. Fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.

Lipsker D, Mitschler A, Grosshans E, Cribier B. · Clinique Dermatologique et Faculté de Médecine, Université Louis Pasteur, Strasbourg, France. · Dermatology. · Pubmed #16778421 No free full text.

Abstract: BACKGROUND AND OBJECTIVE: The objective of this study was to evaluate the relationship between Jessner's lymphocytic infiltration of the skin (JLI) and lupus erythematosus (LE), which has been the subject of debate since its initial description in 1953. MATERIAL AND METHODS: This is a retrospective study including all patients with a histopathologically ascertained diagnosis of JLI performed at the Laboratoire d'Histopathologie Cutanée of the Strasbourg University Hospital between 1993 and 2003. Information about patient characteristics and follow-up data were retrieved between 2004 and 2005. Special attention was paid to features indicative of LE. RESULTS: 210 consecutive patients (102 women and 108 men) with a mean age 42 years were diagnosed with JLI in the reference period. 175 patients (83%) had multiple lesions and 32 patients (15%) had only a single lesion at the time of diagnosis (data not available in 3 patients). The head, neck and upper part of the thorax were involved in 171 patients (81%). An annular or arciform configuration and/or arrangement were present in 111 patients (53%). Lesions consisted of red (100%) papules or plaques (98%). Mean follow-up was 4 years. Sixteen patients (7.6%) had proven LE. Only 2 patients (1%) developed >4 ACR criteria of systemic LE. Furthermore, 1 patient had antiphospholipid antibody syndrome and 2 patients had rheumatoid arthritis. CONCLUSIONS: This high frequency of patients with typical features of LE strongly argues that JLI could be a dermal variant of LE and not an autonomous entity. It might be the cutaneous marker of a subset of LE patients with excellent prognosis.