Co-existent cutaneous cryptococcosis of the forearm and cutaneous alternariosis of the leg in patient with metastatic thymoma.

BACKGROUND: Cryptococcosis and alternariosis are rare opportunistic infections often observed in immunocompromised patients. Because Cryptococcus and Alternaria are ubiquitous fungi found in soil, the presence of fungi in the dermis has to be observed on histological examination to confirm a real cutaneous, invasive, infection. PATIENT: We report the first case of concomitant cutaneous cryptococcosis and cutaneous alternariosis, in an immunocompromised patient treated for a metastatic thymoma. CONCLUSION: This observation underlines the fact that the possible co-existence of several rare infections in immunocompromised patients should take into consideration pathogen identification in order to adapt the therapy to individual patient requirements.

[Localized bullous pemphigoid following radiotherapy]. / Pemphigoïde localisée après radiothérapie.

BACKGROUND: Bullous pemphigoid is the most frequent autoimmune blistering dermatologic disease. Induction of pemphigoid has been observed after administration of certain drugs but also after various irradiation procedures. We report a case of pemphigoid strictly confined to the irradiated area. CASE REPORT: A 66-year-old woman had been irradiated 16 years before because of a breast carcinoma. She presented with a blistering rash strictly confined to the irradiated area. The diagnosis of pemphigoid was confirmed with pathologic examination, direct immunofluorescence, direct immunofluorescence of sodium chloride-separated skin, electron microscopy and immunoelectron microscopy. Indirect immunofluorescence and serum immunoblot were also positive. The patient was successfully treated with topical steroids. DISCUSSION: The location on the area of radiotherapy and the limited character of lesions were unusual. A few cases of pemphigoid strictly localized to the area of radiotherapy have been published. However, in our case, only direct immunofluorescence and immunoelectron microscopy reliably confirmed the diagnosis. The unusual location of this pemphigoid is not due to the regional variation in the expression of the antigen. Other hypotheses concerning the pathogenesis of localized pemphigoid should be examined.

[Evaluation of histological criteria for bullous pemphigoid. Correlation with antigens recognized by immunoblotting of anti-epidermal autoantibodies]. / Evaluation des critères histologiques de pemphigoïde bulleuse. Relation avec les antigènes reconnus en immunotransfert par les auto-anticorps anti-épiderme.

The aim of this study was to evaluate the histological findings observed in patients with bullous pemphigoid in whom the diagnosis of bullous pemphigoid could be confirmed by direct immunofluorescence and immunoblot serum analysis. Seven histological criteria were considered for selection of skin biopsy specimens: 1) cleavage of dermal epidermal junction; 2) migration of eosinophils along dermal epidermal junction; 3) presence of intra epidermal eosinophils (with or without associated spongiosis); 4) absence of keratinocyte necrosis; 5) absence of acantholysis; 6) absence of dermal fibrosis; 7) absence of vasculitis. Depending on the number of criteria observed the histological picture was considered as: highly suggestive, suggestive or poorly suggestive of bullous pemphigoid. The histological picture was considered as highly suggestive in 50% of cases, suggestive or poorly suggestive in 37% and 13% of cases respectively. Migration of eosinophils along dermal epidermal junction was observed in 23 biopsy specimens (50%). Histological findings considered as poorly suggestive of bullous pemphigoid consisted of a prurigo-like or an eczematous-like or a drug induced-like picture or no specific cutaneous erosions. An histological picture highly suggestive of bullous pemphigoid was observed in 67% of patients whose serum contained anti-BPAG2 antibodies and in only 36% patients of without anti-BPAG2 antibodies (p=0,04). On the contrary, only one bullous pemphigoid patient (4%) with circulating anti-BPAG2 antibodies had a histological picture poorly suggestive of bullous pemphigoid. These findings are in accordance with the pathogenic properties of anti-BPAG2 antibodies demonstrated in animal models. This study showed that: 1) typical histological findings of bullous pemphigoid are only observed in 50% of skin biopsy specimens. 2) The diagnosis of bullous pemphigoid should be considered in elderly patients even when a poorly specific prurigo-like or eczematous-like histological picture is observed. Moreover, it underlines the usefulness of direct immunofluorescence of skin biopsy specimens and indirect immunofluorescence and immunoblot analysis of serum in such atypical cases of bullous pemphigoid.

[Epidermal nevus associated with a type I neurofibromatosis and a nephroblastoma: a new epidermal nevus syndrome?]. / Association d'un naevus épidermique, d'une neurofibromatose de type I et d'un néphroblastome: un nouveau syndrome du naevus épidermique?

We report the case of a 6-year-old boy who showed a large epidermal nevus mixed with a plexiform neurofibroma, which was associated with "café au lait" macules and a nephroblastoma. This association could not be classified in one of the five well defined epidermal nevus syndrome. To our knowledge this is the first time that this type of epidermal nevus syndrome has been described, which raises the question of the relationship between neurofibromatosis 1, nephroblastoma and epidermal nevus.

A prospective study of cutaneous intolerance to topical mechlorethamine therapy in patients with cutaneous T-cell lymphomas. French Study Group of Cutaneous Lymphomas.

OBJECTIVE: To study the exact frequency and the histological features of cutaneous intolerance to mechlorethamine (CIM) hydrochloride therapy in patients with cutaneous T-cell lymphomas, including Langerhans cell histiocytosis. DESIGN: A multicenter prospective study was conducted from January 1, 1994, to May 31, 1996, in 12 different hospitals in France. PATIENTS: Of the 52 patients with cutaneous T-cell lymphomas or Langerhans cell histiocytosis, 35 were men and 17 were women, aged 18 to 87 years. Of the 52 patients, 35 had mycosis fungoides, 8 had nonepidermotropic cutaneous lymphoma, 7 had lymphomatoid papulosis, 1 had Sézary syndrome, and 1 had Langerhans cell histiocytosis. METHODS: Patients were treated with topical applications of a 0.02% aqueous solution of mechlorethamine. The diagnosis of CIM was determined by the presence of erythema and pruritus. Patients who developed CIM underwent closed patch testing with three 10-fold dilutions of 0.02% mechlorethamine solution. A positive patch test result was the presence of erythema and pruritus, a weak result was the presence of simple erythema without pruritus, and a negative result was the absence of erythema and pruritus. Skin biopsy specimens from patients with positive patch test results were obtained in patients who developed CIM. The biopsy specimens were reviewed, and the results determined by 2 pathologists (E.T. and J.W.). The histopathological findings were classified in 3 categories: (1) spongiotic dermatitis, (2) irritant dermatitis, and (3) insignificant or normal. In September 1998, the referring physicians were contacted if mechlorethamine therapy had been continued in patients with CIM. RESULTS: Of the 52 patients, 43 were evaluated for tolerance to mechlorethamine therapy. Of the 43 patients, CIM developed in 23, from 4 days to 9 months after the initiation of mechlorethamine therapy. Of those 23 patients, CIM developed within 3 months in 21 and within 1 month in 13. Closed patch tests were performed in 21 of the 23 patients who developed CIM. The results of the patch test were positive in 12, weak in 4, and negative in 5. Of these 21 patients, 14 skin biopsy specimens were obtained in 14 different patients who had positive or weak patch test results. The specimens showed histological features that were consistent with spongiotic dermatitis in 9 patients, irritant dermatitis in 2, and insignificant or normal in 3. All 9 patients with histological features of spongiotic dermatitis discontinued mechlorethamine therapy. All 5 patients without histological features of spongiotic dermatitis were able to resume mechlorethamine therapy. These results do not correlate with those of previous study results. CONCLUSIONS: Mechlorethamine therapy is a cost-effective and easily administered treatment for cutaneous T-cell lymphomas. Our study shows that allergic dermatitis caused by mechlorethamine therapy is an early and frequent adverse reaction in patients with cutaneous T-cell lymphomas. The most common histological feature of patients with CIM is spongiotic dermatitis.

[Evaluation of the diagnosis of pigmented tumors of the skin and factors leading to a decision to excise. Dermatologists of the Postgraduate Association of Haute-Normandie]. / Evaluation du diagnostic des tumeurs pigmentées de la peau et des éléments conduisant à une décision d'exérèse. Dermatologues de l'Association de FMC de Haute-Normandie.

INTRODUCTION: The necessity of excising melanomas characterized by a slight thickness at an early stage, leads dermatologists to remove pigmented lesions which do not correspond to melanomas. The aims of this study were: a) to prospectively assess the accuracy of melanoma diagnosis, b) to quantify the number of excisions performed according to the degree of melanoma suspicion, c) to determine the specific clinical sign or signs of relevant diagnostic value. PATIENTS AND METHODS: This study was conducted prospectively from January 1996 to August 1997 by dermatologists in private practice and dermatologists from a University Hospital staff. When it was decided to excise a pigmented lesion, a form was filled out choosing the most appropriate clinical diagnosis, the degree of melanoma suspicion, and clinical signs which lead to surgery. Based on histological findings as the reference, the sensitivity, specificity, accuracy of melanoma diagnosis and the kappa test that evaluates the concordance between clinical and histological diagnosis, were performed. The diagnostic value of clinical signs was assessed by variance analysis. RESULTS: Of the 353 excised lesions, 38 (10.7 p. 100) were identified as melanoma on histologic examination. The sensitivity, the specificity and diagnostic accuracy were: 79 p. 100, 94 p. 100 and 53 p. 100 respectively. The kappa test concordance between clinical and histological diagnosis was 0.66. Two hundred and two lesions (57 p. 100) were excised even though the clinical suspicion of melanoma was poorly considered. Only one of these 202 lesions was identified histologically as a true melanoma. Thirty seven (24.5 p. 100) of the 151 remaining excised lesions with an "average" or "strong" suspicion were true melanomas. The clinical signs considered, alone or associated, had a poor predictive positive value (< 38 p. 100). An analytical approach performed with a logistic model permitted the identification of two associated signs suggesting a best diagnostic value. DISCUSSION: This is the only study, to our knowledge, reported in the literature which prospectively assesses the sensitivity, specificity and concordance between clinical and histological diagnosis of melanoma. Results were considered from average to good. The originality of this study was to assess the number of pigmented lesions excised according to the degree of melanoma suspicion, suggesting the possibility of reducing the number of nevi removed when the melanoma risk was considered clinically poor. Finally, this study emphasizes the limits of clinical semiology and the need for future diagnostic methods in the assessment of melanoma.

Pseudoepitheliomatous hyperplasia in cutaneous T-cell lymphoma. A clinical, histopathological and immunohistochemical study with particular interest in epithelial growth factor expression. The French Study Group on Cutaneous Lymphoma.

Pseudoepitheliomatous hyperplasia has occasionally been reported in cutaneous T-cell lymphoma (CTCL). This association raises the question of the relationship between epidermal hyperplasia and the lymphomatous infiltrate. Because epidermal growth factor (EGF) and transforming growth factor-alpha (TGF-alpha) have been demonstrated to be involved in epidermal proliferation through binding to EGF receptor (EGFr), we tested the hypothesis that these cytokines could be secreted by lymphomatous cells, and induce the overlying pseudoepitheliomatous hyperplasia. The purposes of this study were: (i) to describe the clinical and immunohistological features of pseudoepitheliomatous hyperplasia; (ii) to determine its frequency in a large series of CTCLs; and (iii) to evaluate the expression of EGF, TGF-alpha and EGFr in CTCL with or without pseudoepitheliomatous hyperplasia. Eleven cases of CTCL with pseudoepitheliomatous hyperplasia were collected from a series of 353 cases of cutaneous lymphoma registered from 1990 to 1996. They consisted of eight of 28 (28.5%) CD30+ large T-cell lymphomas and three of 148 (2%) cases of mycosis fungoides. Epidermal expression of EGF, EGFr and TGF-alpha was stronger in CTCL than in control normal human skin. Lymphomatous T cells expressed EGF and TGF-alpha whereas no expression of these cytokines could be detected in cutaneous and nodal B-cell lymphomas, nor in a normal lymph node. In addition, epidermal expression of EGFr was stronger in CTCL with pseudoepitheliomatous hyperplasia than in control cases of CTCL without pseudoepitheliomatous hyperplasia, suggesting that these cytokines, in association with other factors, are probably involved in the epidermal hyperplasia observed in some cases of CTCL.

Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus.

Tunisian pemphigus is a newly described form of endemic pemphigus whose clinical, histological and epidemiological characteristics have recently been detailed. The objective of this study was to analyse the binding properties of autoantibodies present in sera from patients with endemic Tunisian pemphigus using immunoblotting and indirect immunoelectron microscopy (IEM). Thirty patients with pemphigus foliaceus (PF) and six with pemphigus vulgaris (PV) seen in the dermatology department of Tunis Hospital between 1992 and 1994 were selected for this study. Seven of 30 (23%) and six of 12 (50%) PF sera tested bound to the 160 kDa band of desmoglein 1 when tested on bovine tongue and human epidermal extracts, respectively. Two of six and two of three PV sera tested bound to the 130 kDa desmoglein 3 in these two extracts. Immunoblot and indirect IEM showed that 24 of 30 (80%) PF sera contained IgG1, IgG3 or IgG4 antibodies that bound to a 185-kDa polypeptide localized on the desmosomal plaque. This immunological analysis showed that most endemic Tunisian pemphigus sera correspond to PF sera and are characterized by a high frequency of autoantibodies directed against a recently identified 185-kDa antigen of the desmosomal plaque.

[Eczema-like cutaneous graft versus host disease treated by UV-B therapy in a 2-year-old child]. / GVH cutanée eczématiforme traitée par UV-B thérapie chez un enfant de deux ans.

INTRODUCTION: Chronic graft versus host disease (GVHD) has rarely been reported in children. Optimal treatment should minimize infectious complications and preserve the child's growth. We report a case of cutaneous GVHD in a two year-old boy, who presented an eczema-like eruption and responded well to broad band UV-B therapy. CASE REPORT: A two year-old boy with acute myeloblastic leukemia had a heterologous bone marrow transplantation with a graft issued from an unrelated female donor. Three month later, he developed eczema-like lesions of the trunk, arms and legs associated with diffuse alopecia, despite oral corticosteroids and cyclosporine treatment. Histologic findings were consistent with GVHD. Topical corticosteroids and broad band UV-B therapy were initiated, while oral corticosteroids and cyclosporine doses were tappered off. GVHD lesions cleared, allowing withdrawal of oral corticosteroids and cyclosporine 3 and 12 months respectively after initiation of UV-B therapy. No relapse occurred 24 months after systemic treatment discontinuation and 12 months after broad band UV-B therapy was stopped. CONCLUSION: This observation suggests that broad band UV-B therapy is an effective treatment for eczema-like, cutaneous GVHD.

Polymerase chain reaction analysis of immunoglobulin gene rearrangement in cutaneous lymphoid hyperplasias. French Study Group for Cutaneous Lymphomas.

UNLABELLED: The differential diagnosis of cutaneous lymphoid hyperplasia and B-cell lymphoma may be difficult. Whether the detection of clonal immunoglobulin gene rearrangement in the cutaneous lesion is predictive of a malignant outcome remains controversial. We therefore studied cases of cutaneous lymphoid hyperplasia by polymerase chain reaction analysis. DESIGN: Retrospective study of patients seen between 1988 and 1996. SETTING: Two dermatology university departments. PATIENTS: Twenty-four patients with cutaneous lymphoid hyperplasias were included according to clinical, histopathological, and immunophenotypic criteria. MAIN OUTCOME MEASURES: Clinical, histopathological, and laboratory findings. RESULTS: There were 13 men and 11 women (mean age, 49 years) who presented with erythematous or violaceous papules or nodules. The lesions were unique in 13 cases and multiple in 11 cases. All patients had immunochemical evidence of a mixed T- and B-cell infiltrate with polytypic B cells. Polyclonality was demonstrated in 23 patients, whereas a dominant B-cell clone was detected in 1 patient. No lymphoma developed during the follow-up (median, 4 years). In the same period, we studied 53 cases of B-cell lymphomas. Thirty-five (66%) of the 53 cases had a detectable clonal immunoglobulin gene rearrangement. CONCLUSIONS: In the majority of our cases, polyclonality demonstrated by polymerase chain reaction analysis was in accordance with the diagnosis of cutaneous lymphoid hyperplasia. In 1 of the 24 patients, the presence of a B-cell clone could be evidenced. This fact did not modify the treatment as there were no histological or immunophenotypic signs suggestive of a lymphoma.

CD4+ CD56+ cutaneous neoplasms: a distinct hematological entity? Groupe Français d'Etude des Lymphomes Cutanés (GFELC).

We report seven cases of particular cutaneous tumors selected from the register of the French Study Group on Cutaneous Lymphomas. The patients (three men, four women) were aged 37-86 years. They initially presented with cutaneous nodules or papules. Three cases presented with regional lymph nodes. Stagings were negative, except for one patient with bone marrow involvement. Histological features were relevant with pleomorphic medium T-cell lymphoma, but these cells exhibited a distinguishing phenotype. They were positive for CD4, CD56, and also CD45, CD43, and HLA-DR. All other T-cell and B-cell markers were negative. The myelomonocytic markers (CD13, CD14, CD15, CD33, CD117, myeloperoxidase, and lysozyme) were negative excepted CD68, which was clearly positive in four cases and weakly in two cases. Others natural killer cell markers (CD16, CD57, TiA1, granzyme B), TdT, and CD34 were negative. Polymerase chain reaction studies did not detect any B or T clonal rearrangement. The cytogenetic studies, performed in five cases, showed a del(5q) in two cases. All patients were treated successfully by polychemotherapy, but relapsed quickly in the skin, between 4 and 28 months. Five patients developed bone marrow involvement, with leukemia in three cases, and they died in 5-27 months. One patient died at 17 months with skin progression. The seventh patient is alive at 33 months, with cutaneous progression. The origin of these cells is unclear. Despite expression of CD4 or CD56, we failed to demonstrate a T-cell, natural killer cell origin. However, CD4 and CD56 are not specific for T or natural killer lineages. Although these two markers are also known to be expressed by monocytic cells, classic myeloid antigens were negative. These seven cases, together with other rare similar cases already reported, seem to represent a distinct entity likely developed from hematological precursor cells.

Lichenoid erythrodermic bullous pemphigoid of the African patient.

BACKGROUND: Lichen planus (LP) pemphigoides is defined by the concurrence of bullous pemphigoid (BP) and LP lesions. However, other autoimmune bullous skin diseases such as paraneoplastic pemphigus or bullous lupus erythematosus may also be associated with lichenoid eruptions. OBJECTIVE: Our purpose was to describe 3 African patients with a similar form of lichenoid erythrodermic BP. METHODS: Medical records of the 3 patients were reviewed for clinical, histologic, and biologic data. The ultrastructural localization of autoantibody deposits was studied by direct and indirect immunoelectron microscopy. The antigenic specificities recognized by the patients' sera were analyzed by immunoblotting on human epidermal extracts. RESULTS: Three black African men had a severe lichenoid erythroderma associated with BP lesions involving the skin and mucosa. Histologic and direct immunofluorescence examination of skin and mucosal lesions were consistent with the diagnosis of BP associated with a lichenoid dermatitis. Immunoblot analysis of sera detected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microscopy showed IgG deposits localized in the lamina lucida and the hemidesmosomes. The 3 patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype. CONCLUSION: The clinical and histologic features of these 3 patients were distinct from classic LP pemphigoides. Their common geographic origin and the presence of the HLA-DR10 haplotype suggest that these cases represent a particular type of lichenoid erythrodermic BP, which is probably determined by genetic factors.

Evaluation of clinical criteria for diagnosis of bullous pemphigoid. French Bullous Study Group.

OBJECTIVE: To check the potential usefulness of clinical criteria for the diagnosis of bullous pemphigoid when state-of-the-art techniques such as Western immunoblotting, immunoprecipitation, and indirect immunofluorescence on salt-split skin or direct immunoelectron microscopy are not available. DESIGN: Comparison of the clinical criteria between 2 groups (with and without bullous pemphigoid) as defined by immunoelectron microscopy used as standard criterion, in a prospective study. Multivariate logistic regression analysis was carried out by including all items that were statistically significant (at P < .05 level) in univariate analysis. SETTING: Five dermatology departments in teaching hospitals. PATIENTS: The 231 patients studied had subepidermal autoimmune bullous diseases with linear IgG or C3 deposits in the basement membrane zone (157 with bullous pemphigoid, 33 with cicatricial pemphigoid, 30 with epidermolysis bullous acquisita, 5 with lupus erythematosus, and 6 others). A second set of patients was used to calculate predictive values. RESULTS: The multivariate logistic stepwise analysis resulted in a final set of predictors that included only 4 items: absence of atrophic scars, absence of head and neck involvement, absence of mucosal involvement, and age greater than 70 years. No additional variables met the .05 significance level to enter into the model. If 3 of these 4 characteristics were present, a diagnosis of bullous pemphigoid could be made with a sensitivity of 90% and a specificity of 83%; these predictive values were calculated on a sample of 70 new cases. CONCLUSIONS: With and estimated incidence of bullous pemphigoid among subepidermal autoimmune bullous diseases of 80%, the presence of 3 of the 4 significant criteria allows the diagnosis of bullous pemphigoid, with a positive predictive value of 95%. Our set of clinical criteria thus allows the diagnosis of bullous pemphigoid with good validity for both clinical practice and therapeutic trials.

Differences in Epstein-Barr virus expression between primary and secondary cutaneous angiocentric lymphomas. French Study Group of Cutaneous Lymphomas.

BACKGROUND: Epstein-Barr virus (EBV) has been demonstrated in angiocentric immunoproliferative lesions, suggesting that it could be a causative factor. We investigated for the presence of EBV in 12 primary and 2 secondary cutaneous angiocentric lymphomas (CALs). OBSERVATIONS: In the 2 secondary CALs, strong reactivity for EBV RNAs and latent membrane protein 1 were detected on paraffin-embedded sections. In contrast, 10 of 12 primary CALs were completely negative for EBV RNAs and latent membrane protein 1. In 2 primary CALs, EBV RNAs and latent membrane protein 1 were detected in few tumor cells. In the group of primary CALs, 8 of 12 were still alive at last follow-up, 3 died of systemic lymphoma, and 1 died of another cause, whereas both patients with secondary CALs died of disease within 1 year. CONCLUSION: Differences in the presence of EBV and clinical behavior between primary and secondary CALs suggest that different mechanisms are operative in the pathogenesis of these conditions, and indicate that the 2 groups should be considered separately.

Relationship between the in vivo localization and the immunoblotting pattern of anti-basement membrane zone antibodies in patients with bullous pemphigoid.

OBJECTIVE: To compare the localization of anti-basement membrane zone (BMZ) antibodies bound in vivo with the antigenic specificities of circulating anti-BMZ antibodies in patients with bullous pemphigoid (BP). DESIGN: Comparison of the results of an examination of the skin specimens of the patients using direct immunoelectron microscopy and direct immunofluorescence on 1-mol/L sodium chloride-split skin with the results of an analysis of the corresponding serum samples using the immunoblot technique. SETTING: Immunodermatology department in a teaching hospital. PATIENTS: Thirty-six patients with typical BP and circulating anti-BMZ antibodies. RESULTS: Serum samples from 22 patients with BP indicated only BP antigen 1 in the results of immunoblot analysis. Using direct immunofluorescence, an analysis of the peribullous skin samples obtained from these 22 patients showed deposits of IgG exclusively located along the epidermal side of sodium chloride-split skin; the results of direct immunoelectron microscopic examination showed deposits of IgG located on the intracellular portion of hemidesmosomes in 18 (82%) of these 22 specimens, whereas 4 biopsy specimens had linear IgG deposits located both intracellularly and extracellularly along the keratinocyte plasma membrane. The results of immunoblot analysis of the serum samples from 5 patients with BP indicated BP antigen 2 alone; the results of direct immunoelectron microscopic examination of peribullous skin samples from these 5 patients showed linear intracellular and extracellular deposits along the keratinocyte membrane, corresponding to an epidermal fluorescence labeling pattern of peribullous sodium chloride-split skin in 2 patients and a combined (dermal and epidermal) pattern in 3 patients. CONCLUSION: The 2 different patterns of reactivity of anti-BMZ antibody deposits bound in vivo closely corresponded to the antigenic specificities indicated in the corresponding serum samples of the patients. These results are in accordance with those previously obtained in vitro and argue for identical binding profiles of circulating antibodies that are bound in vivo in BP.

Identification of a new antibody population directed against a desmosomal plaque antigen in pemphigus vulgaris and pemphigus foliaceus.

Pemphigus vulgaris and pemphigus foliaceus are characterized by autoantibodies directed against transmembrane glycoproteins of desmosomes. F12, a human monoclonal autoantibody that binds to the desmosomal plaque, recognizes a 180-190-kDa doublet when immunoblotted against bovine tongue epithelium. Because F12 was derived from the peripheral blood lymphocytes of a patient with pemphigus vulgaris, we looked for the presence of anti-180-190-kDa antibodies in pemphigus vulgaris and pemphigus foliaceus serum. By immunoblot analysis, a third of the pemphigus serum contained anti-180-190-kDa antibodies that belonged to IgG subclass 1 or 3, unlike those that recognized desmogleins 1 and 3 (IgG4). By immunoelectron microscopy analysis on human oral mucosa and human skin with mAb to human IgG3, pemphigus serum containing anti-180-190 kDa antibodies recognized desmosomal plaques. The presence of antibodies with F12 properties in pemphigus serum was further demonstrated by a rabbit anti-F12 idiotype antiserum that allowed detection of F12 idiotype in serum with anti-180-190-kDa antibodies. These results indicate that some pemphigus vulgaris and pemphigus foliaceus serums contain antibodies that react with both intra- and extracellular structures of desmosomes and further demonstrate the heterogeneity of the autoimmune response in both types of pemphigus.

Indomethacin treatment of eighteen patients with Sweet's syndrome.

BACKGROUND: The standard treatment for Sweet's syndrome (acute febrile neutrophilic dermatosis) is oral corticosteroids. Despite a good initial response, the disease is characterized by frequent relapses. OBJECTIVE: Our purpose was to test the therapeutic effect of the nonsteroidal antiinflammatory drug indomethacin on Sweet's syndrome. METHODS: All patients with Sweet's syndrome observed during a 4-year period were given indomethacin, 150 mg/day for the first week and 100 mg/day for two additional weeks. The therapeutic response was assessed on days 4, 7, 14, 30, and 180. RESULTS: Seventeen of 18 patients had a good initial response; fever and arthralgias were markedly attenuated within 48 hours and eruptions cleared between 7 and 14 days. The remaining patient's cutaneous lesions continued to develop and were successfully treated with prednisone (1 mg/kg/day). The only side effect of indomethacin treatment was epigastric pain in two patients. No patient had a relapse after discontinuation of indomethacin (mean followup, 20.1 months). CONCLUSION: Indomethacin is a safe and effective treatment for Sweet's syndrome.

[Aneurysm of superficial temporal artery in angiolymphoid hyperplasia lesions]. / Anévrisme de l'artère temporale superficielle au sein de lésions d'hyperplasie angiolymphoïde.

INTRODUCTION: Subcutaneous angiolymphoid hyperplasia with eosinophilia is a disease which usually affects small vessels of neck and facial area. CASE REPORT: A 44 year-old with cutaneous lesions corresponding to angiolymphoid hyperplasia with eosinophilia is reported. The lesions were located near the temporal region. This case was particular because of the development of an anevrism of the superficial temporal artery. DISCUSSION: Involvement of the superficial temporal artery by angiolymphoid hyperplasia with eosinophilia has been reported in only one case which simulated a Horton disease because of an occlusion of the temporal artery. The development of an anevrism of this artery in a patient with angiolymphoid hyperplasia with eosinophilia is a very unusual presentation of the disease which has not been previously reported.