Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark.

Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients seen at our department since 1972. A total of 23 patients were identified; 7 children (F:M ratio 0.75) and 16 adults (F:M ratio 0.78). Mean age at disease onset in the two age groups were 2.7 and 56.8 years. Estimated incidence rate in our region: 0.67 per million per year. The most commonly used treatment modalities were corticosteroids, dapsone and sulphapyridine.

Management of dermatitis herpetiformis.

The major treatment strategies for DH are gluten restriction or medical treatment with sulfones. Control of the cutaneous manifestations, but not the gastrointestinal changes, is rapid with dapsone. In addition to control of the cutaneous signs and symptoms of DH, dietary gluten restriction also induces improvement of gastrointestinal morphology and is possibly protective against the development of lymphoma.

Management of epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita is a rare and debilitating autoimmune mucocutaneous blistering disease caused by autoantibodies directed against type VII collagen or anchoring fibrils in the subepidermal basement membrane zone. Treatment is quite challenging because this disease can be recalcitrant to multiple modalities. This article discusses the current management of this disease.

[Linear IgA bullous dermatosis: a review]. / Dermatose à IgA linéaire: revue de la littérature.

BACKGROUND: Linear IgA bullous dermatosis (LABD) is a rare auto-immune bullous disease occurring in adults or childhood. OBJECTIVE: Review of literature about physiopathology, triggering factors, clinical data and treatment of LABD. METHODS: Research on Medline and Embase database without any time limit until April 2010. Because of the lack of randomized therapeutic trials in LABD, retrospective series and case reports have been analyzed. RESULTS: LABD is due to IgA auto-antibodies typically directed against a proteolytic fragment of BP180 antigen, a 97 or 120kD protein, and/or other components of dermal-epidermal junction. The disease may be either idiopathic or triggered by several medication, most often vancomycin, but also other antibiotics, non-steroid anti-inflammatory, anti-hypertensive and anti-epileptic drugs… Clinically, eruption is typical in childhood with cluster and herpetiform arrangement of blisters and involvement of evocative anatomical sites. In adults, eruption is polymorphic, very atypical presentations are described. Diagnosis is confirmed by direct immunofluorescence which shows linear IgA deposition on the basement membrane zone. Immunoblot and immunoelectron microscopy are evocative in case of diagnosis hesitation. LABD may be associated with some inflammatory bowel disorders. There is no increased risk of cancer or lymphoma. For drug-induced LABD, withdrawal of the medication is followed by a quick healing of the lesions. Dapsone is quickly efficient in idiopathic LABD. Colchicine, sulfapyridine and systemic corticosteroids are used in case of intolerance or inefficiency of dapsone. Some authors emphasize the efficiency of first-intent antibiotics in LABD of childhood. CONCLUSION: A triggering drug should be always suspected and stopped. Dapsone is the reference treatment in idiopathic cases of LABD.

Erupción vesiculosa asociada a inyección conjuntival / Vesicular eruption associated with conjunctival injection

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Immunosuppressive therapy for ocular mucous membrane pemphigoid strategies and outcomes.

PURPOSE: To evaluate the effectiveness and toxicity of a stepladder immunosuppression strategy, including the use of mycophenolate mofetil and combination therapy, in the treatment of ocular mucous membrane pemphigoid. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Two hundred twenty-three eyes of 115 patients. METHODS: Patients with a diagnosis of ocular mucous membrane pemphigoid commencing immunosuppression between January 1994 and July 2005 were identified. A treatment episode was defined by the use of a particular therapy or combination of therapies. MAIN OUTCOME MEASURES: For each treatment episode, success of immunosuppressive therapy in controlling ocular inflammation was graded as a success (S), qualified success (QS), or failure (F). Initial and final visual acuities (VAs), stage of cicatrization (Foster, Mondino), grade of conjunctival inflammation, and side effects were recorded. RESULTS: In 70% (80/115) of patients, inflammation was controlled by the end of the study. At least 6 months remission off treatment occurred in 16 patients (14%). Of the 388 treatment episodes, 50% were classified as S; 27%, QS; and 23%, F. The most successful therapies were based on cyclophosphamide (S, 69%; QS, 21%; F, 10%), followed by mycophenolate (S, 59%; QS, 22%; F, 19%), azathioprine (S, 47%; QS, 24%; F, 29%), dapsone (S, 47%; QS, 30%; F, 23%), and sulfapyridine (S, 38%; QS, 27%; F, 35%). Combination sulfa-steroid-myelosuppressive agent therapy increased the response from 73% with single-agent therapy to 87%. Side effects were the reason for 29% of changes in therapy. These were most prominent with azathioprine (40%) and least with mycophenolate (15%). Initial best-corrected VA (BCVA) was 6/60 or less in 17% (37/223) of eyes, pemphigoid being the cause in 13% (29/223). Final BCVA was 6/60 or less in 34% (76/223) of eyes, pemphigoid being the cause in 26% (57/223). By the end of the study, Mondino stage cicatrization had progressed in 41% (92/223) of eyes and 53% (61/115) of patients. CONCLUSIONS: Mycophenolate mofetil seems to be an effective and well-tolerated immunosuppressant for moderately active ocular mucous membrane pemphigoid. Combination sulfa-steroid-myelosuppressive agent therapy in a stepladder regimen is a useful strategy to improve disease control. Cicatrization and VA may still progress and worsen despite adequate control of inflammation.

Dermatosis ampollosa IgA lineal del adulto tratada con colchicina / Linear IgA bullous dermatosis of adults treated with colchicine

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Dapsone and sulfapyridine.

Dapsone and sulfapyridine are structurally related compounds with anti-microbial and anti-inflammatory effects. Dapsone remains the most important drug for leprosy and is useful in the prophylaxis of Pneumocystis pneumonia in patients with HIV disease. The medical treatment of choice for dermatitis herpetiformis is dapsone; and sulfapyridine also can be used for those patients who are intolerant of dapsone. Other neutrophilic disorders also may respond to these drugs. Toxic side effects of both dapsone and sulfapyridine are mediated through the hydroxylamine metabolite. These include hemolysis, methemoglobinemia, and agranulocytosis. Careful monitoring for possible adverse reactions includes frequently performing complete blood counts and regular blood chemistry profile determinations.

Treatment with sulfasalazine or sulfapyridine, but not 5-aminosalicyclic acid, inhibits basic fibroblast growth factor-induced endothelial cell chemotaxis.

OBJECTIVE: Rheumatoid arthritis (RA) is characterized by leukocyte recruitment and angiogenesis. We investigated the effects of sulfasalazine (SSZ) and its metabolites, sulfapyridine (SP) and 5-aminosalicylic acid (5-ASA), on components of angiogenesis, namely, endothelial cell (EC) chemotaxis and proliferation, as well as on EC chemokine and soluble adhesion molecule expression. METHODS: SSZ, SP, and 5-ASA were assayed for their effects on basic fibroblast growth factor (bFGF)-induced human dermal microvascular endothelial cell (HMVEC) chemotaxis and proliferation. EC were plated on Matrigel to assess the effect of SSZ on EC tube formation. Enzyme-linked immunosorbent assays were performed to determine changes in HMVEC production of interleukin-8 (IL-8), monocyte chemoattractant protein-1 (MCP-1), growth-related oncogene alpha (GROalpha), epithelial neutrophil-activating peptide 78 (ENA-78), soluble E-selectin (sE-selectin), and soluble intercellular adhesion molecule 1 (sICAM-1) upon treatment with SSZ or its metabolites. RESULTS: HMVEC incubated with SSZ or SP exhibited reduced bFGF-induced chemotaxis (59%, [n = 7] and 22%, [n = 3], respectively) (P<0.05). SSZ and SP decreased basal HMVEC proliferation, while 5-ASA increased proliferation (P<0.05; [n = 5]). SSZ decreased bFGF-induced HMVEC proliferation (P<0.05 [n = 5]). SSZ inhibited phorbol 12-myristate 13-acetate-induced HMVEC tube formation (P<0.05; [minimum n = 5]). Tumor necrosis factor alpha-stimulated HMVEC shedding of sICAM-1 was reduced by incubation with either SSZ (19%) or 5-ASA (23%) (P<0.05; [n = 6]). SP inhibited cytokine-stimulated HMVEC expression of IL-8 and MCP-1 (P<0.05; [n = 4]). Neither SSZ nor its metabolites had any effect on HMVEC production of sE-selectin, GROalpha, or ENA-78. CONCLUSION: These results demonstrate that SSZ and its metabolite SP may affect the pathogenesis of RA by inhibiting EC chemotaxis, proliferation, tube formation, and expression of sICAM-1, IL-8, and MCP-1.

Linear IgA disease histopathologically and clinically masquerading as lichen planus.

In each of 2 cases reported, the patient presented with features of erosive lichen planus or lichenoid drug eruptions and an incisional biopsy taken from the patient was diagnosed histologically as lichen planus. Subsequent recurrences or exacerbations were associated with vesiculobullous lesions. Simultaneous or subsequent direct immunofluorescence studies--from the same tissue sample in one case and from a similar site in the other case--demonstrated classic features of linear IgA disease. Both patients were originally treated for lichen planus with systemic and/or topical corticosteroids with limited success. One patient was treated with sulfapyridine with minimal improvement. Both patients were subsequently treated with dapsone and demonstrated significant clinical improvement. We propose that linear IgA disease may be more common than reported in the oral cavity, inasmuch as many cases of recalcitrant lichen planus, erosive lichen planus, and lichenoid drug eruptions, especially those with a vesiculobullous component, may in reality represent linear IgA disease. We recommend that direct immunofluorescence be done in any case in which bullous lichen planus is suspected.

Pioderma gangrenoso: revisión a propósito de dos casos / Pyoderma gangrenosum

El pioderma gangrenoso es una enfermedad inflamatoria infrecuente, caracterizada por la aparición de pápulas, pústulas, nódulos y ulceraciones necróticas, que frecuentemente está asociada a enfermedades sistemáticas. La evolución es crónica con recurrencias y resistencia a los distintos tratamientos. Presentamos dos casos de pioderma gangrenoso: un paciente con la forma clásica y otro con la forma granulomatosa superficial. Esta enfermedad requiere un estudio exhaustivo del paciente para lograr el diagnóstico correcto. Sugerimos la terapéutica apropiada, pero debido a que se desconoce la fisiopatogenia aquélla resulta muchas veces infructuosa (AU)

Pioderma gangrenoso: revisión a propósito de dos casos / Pyoderma gangrenosum

El pioderma gangrenoso es una enfermedad inflamatoria infrecuente, caracterizada por la aparición de pápulas, pústulas, nódulos y ulceraciones necróticas, que frecuentemente está asociada a enfermedades sistemáticas. La evolución es crónica con recurrencias y resistencia a los distintos tratamientos. Presentamos dos casos de pioderma gangrenoso: un paciente con la forma clásica y otro con la forma granulomatosa superficial. Esta enfermedad requiere un estudio exhaustivo del paciente para lograr el diagnóstico correcto. Sugerimos la terapéutica apropiada, pero debido a que se desconoce la fisiopatogenia aquélla resulta muchas veces infructuosa

[Ulcerative colitis and intestinal bleeding].

Ulcerative colitis is a nonspecific inflammatory disease of large intestine. Its inflammation is limited to intestinal mucosa. The most essential symptom is hematochezia, bloody stool and intestinal bleeding. Differential diagnosis among diseases having intestinal bleeding is clinically important. These diseases include Crohn disease, ischemic colitis, intestinal Behçet disease, Enterohemorrhagic E. coli including O157, antibiotics associated hemorrhagic colitis and so on. Drugs for the treatment of ulcerative colitis are sulphapyridine, 5-aminosalicylic acid, prednisolone, betamethasone and immunosuppressive drugs. Recently, leukocytapheresis and massive immunoglobulin 7S treatment are available for the treatment of ulcerative colitis.

The effect of sulphasalazine on neutrophil superoxide generation in rheumatoid arthritis.

The production of superoxide by the peripheral blood neutrophils of 19 patients with active rheumatoid arthritis was measured during treatment with sulphasalazine (SASP). The response to drug treatment was determined by change in plasma viscosity, CRP, early morning stiffness and articular index over a 10-point scale. Of the 19 patients studied, eight were considered to have responded well to SASP and seven to have responded poorly or not at all. Over the treatment period, plateau levels of superoxide production fell in seven of the eight responders (P = 0.028) compared with a non-significant fall in 3/7 of the non-responder groups. The initial rate of superoxide production also fell in the responder group, but this was not statistically significant. Initial values in both the responder and non-responder groups were comparable with those seen for normal controls. Analysis of drug levels showed all patients to be compliant with drug treatment; however, drug levels and neutrophil activity were not correlated. Studies of the effect of SASP and sulphapyridine on superoxide production in vitro showed no difference between good and poor responders. These results suggest that there is no inherent difference between good and poor responders regarding the susceptibility of their neutrophils to SASP. SASP's action on neutrophils, therefore, appears not to be its main mechanism of disease-modifying activity in RA.