Design  Prospective, open, multicenter study.

Setting  Thirteen dermatology departments in France.

Patients  Twenty-five patients with either (1) stage II to stage IV CTCL previously unsuccessfully treated with at least 2 lines of treatments or (2) histologically transformed epidermotropic CTCL requiring chemotherapy.

Intervention  Administration of Caelyx intravenously once every 4 weeks at a dose of 40 mg/m².

Main Outcome Measures  The response to treatment was evaluated by clinical evaluation.

Results  At the end of treatment, we observed an objective response (primary end point) in 56% of the patients (14 of 25): 5 complete responses and 9 partial responses. The median overall survival time was 43.7 months. For the 14 patients who experienced an objective response, the median progression-free survival time after the end of treatment was 5 months.

Conclusions  This prospective study demonstrates the effectiveness of Caelyx in treating CTCL, with an overall response rate of 56% in spite of the high proportion of patients with advanced-stage disease. Responses were observed in 2 subpopulations of patients in which the prognosis is known to be poorer: Sézary syndrome (overall response rate, 60%) and transformed CTCL (overall response rate, 50%). Moreover, this study shows that dose escalation to 40 mg/m² does not seem to improve the effectiveness but increases toxic effects (especially hematologic toxic effects) compared with the dose previously tested of 20 mg/m².

Setting  A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.

Patients  Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.

Results  Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (≤IIA) had a 10-year survival of 82%, which took a steep drop off to 41% by 15 years. Patients with late-stage disease (≥IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage.

Conclusions  The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.

Design  Retrospective, cross-sectional analysis of the North American Contact Dermatitis Group database, 1994-2004.

Patients  Five hundred seventy-five patients with anogenital signs or symptoms were referred for patch testing; 347 had anogenital disease only.

Main Outcome Measure  Currently relevant allergic patch test reaction in patients with anogenital signs or symptoms.

Results  Sex percentages and mean age were not significantly different in patients with anogenital involvement only compared with those without anogenital involvement. In patients with anogenital involvement only, a final diagnosis of "other dermatoses" was statistically significantly more common in female patients compared with male patients (n = 347; relative risk, 1.99; 95% confidence interval, 1.37-2.91), but the diagnosis of allergic contact dermatitis was not associated with sex. Specific allergens that were statistically significantly more common in patients with anogenital involvement included cinnamal (or cinnamic aldehyde), dibucaine, benzocaine, hydrocortisone-17-butyrate, and budesonide (all P < .005). Those that were statistically significantly less frequent included quaternium-15, cobalt chloride, formaldehyde, p-phenylenediamine, and thiuram mix (all P < .04). Seventy-three patients had anogenital allergic contact dermatitis, defined as anogenital involvement only, allergic contact dermatitis as the only diagnosis, and at least 1 positive reaction of current clinical relevance. In that subgroup, the most common allergen sources were cosmetics, medications, and corticosteroids.

Conclusion  In patients in the North American Contact Dermatitis Group with anogenital involvement only, male and female patients were equally likely to have allergic contact dermatitis but female patients were more likely to have other dermatoses. Common allergens and sources consisted of those likely to have contact with the anogenital area.

Design  Convenience sample.

Setting  Hospital-based practice.

Participants  The study included 60 healthy men (age range, 20-60 years) without evidence of alopecia.

Main Outcome Measure  Range of normal for a standardized 60-second hair count.

Results  Among the 20- through 40-year-old men, the shedding range was 0 to 78 hairs, with a mean of 10.2 hairs. Among the 41- through 60-year-old men, the range was 0-43 hairs, with a mean of 10.3 hairs. Low intrapatient variability for hair counts was found in both age groups, indicating consistent results on consecutive days for all participants. When repeated 6 months later in both age groups, the hair counts did not change much. The hair counts were repeated and verified by a trained investigator, with results similar to those of subject hair counts.

Conclusion  A properly performed 60-second hair count is a simple, practical, and reliable tool for the assessment of hair shedding.

Observations  Three patients with idiopathic solar urticaria underwent phototesting with UV-B and UV-A radiation. The minimal urticarial dose (MUD) was determined 15 minutes after irradiation. The patients were subsequently tested with 5 times the MUD, and the reaction was graded every minute for 15 minutes. The patients were then treated with a high-protection, broad-spectrum sunscreen and a nonsedative antihistamine alone and in combination and underwent similar phototesting. The use of sunscreen allowed the patients to tolerate much higher doses of UV radiation (32-38 times the MUD on untreated skin). Antihistamine use did not increase the patients' MUD but did suppress wheal formation and itch, and only immediate erythema sharply located in the irradiated areas occurred. The combination of sunscreen and antihistamine acted synergistically and increased the tolerance to UV radiation markedly (80-267 times the MUD on untreated skin).

Conclusion  High-protection, broad-spectrum sunscreens and antihistamines protect patients with solar urticaria in different ways and are highly effective when combined.

Observations  Two patients presented with fever together with systemic and cutaneous manifestations suggestive of CM. Although findings from blood cultures remained negative, we were able to identify N meningitidis in the skin lesions by a newly developed PCR assay. In 1 patient, an N meningitidis strain of the same serogroup was also isolated from a throat swab specimen. Both patients rapidly improved after appropriate antibiotherapy.

Conclusions  To our knowledge, we report the first cases of CM diagnosed by PCR testing on skin biopsy specimens. It is noteworthy that, although N meningitidis–specific PCR is highly sensitive in blood and cerebrospinal fluid in acute infections, our observations underscore the usefulness of PCR performed on skin lesions for the diagnosis of chronic N meningitidis infections. Whenever possible, this approach should be systematically employed in patients for whom N meningitidis infection cannot be confirmed by routine microbiologic investigations.

Observations  We describe 2 patients with vancomycin-associated linear IgA bullous dermatosis who presented with a morbilliform eruption that lacked blistering. Lesional and perilesional tissue from each patient was examined by light microscopy and direct immunofluorescence. Histopathologic examination findings revealed vacuolar interface dermatitis with a mixed inflammatory infiltrate and occasional eosinophils, consistent with a drug eruption. Direct immunofluorescence revealed IgA deposited in a linear pattern at the dermoepidermal junction. In both patients, the results of indirect immunofluorescence using both IgG and IgA were negative.

Conclusions  These cases highlight the existence of a new form of linear IgA bullous dermatosis presenting as a morbilliform drug eruption. Both patients were following extensive medication regimens, including use of multiple antibiotics. The diagnosis of linear IgA bullous dermatosis allowed us to target vancomycin as the likely allergen and begin treatment. In light of these findings, direct immunofluorescence may be a useful diagnostic adjunct in determining the cause of drug eruptions.

Observations  We describe a 70-year-old white woman with metastatic renal cell carcinoma who was treated with a combination of sorafenib and tipifarnib (a farnesyltransferase inhibitor). She had no history of skin cancer. However, within 3 months after starting this therapy, she developed 3 erythematous nodules on her legs. Pathologic examination showed deeply invasive, well-differentiated squamous cell carcinomas. The tumors were excised, and sorafenib-tipifarnib treatment was discontinued. No new lesions have developed to date.

Conclusions  Targeted agents, such as sorafenib and tipifarnib, are increasingly being used in the management of visceral malignant neoplasms. A temporal relationship was observed between the initiation of the targeted treatments and the emergence of these cutaneous cancers. Further study of the mechanisms responsible for the rapid appearance of squamous cell cancers in this setting may provide insights into the pathogenesis of skin tumors.

Setting  Dermatology department of a university hospital.

Design  Tissue sections were stained with a polyclonal B burgdorferi antibody using standard histological equipment and then scanned simultaneously in 2 planes: horizontally in a serpentinelike pattern and vertically by focusing through the thickness of the section, ie, focus-floating microscopy. Part of the material was also investigated by Borrelia-specific polymerase chain reaction.

Patients  The study population comprised 61 cases of lichen sclerosus and 118 controls (60 negative controls and 68 positive controls).

Main Outcome Measure  The presence of B burgdorferi sensu lato within tissue specimens.

Results  Using focus-floating microscopy, we detected Borrelia species in 38 of 60 cases (63%) of lichen sclerosus and in 61 of 68 (90%) of positive controls of classic borreliosis, but Borrelia species were absent in all negative controls. Borrelia species were detected significantly more often in early inflammatory-rich (31 of 39 [80%]) than in late inflammatory-poor (7 of 21 [33.3%]) cases (P = .001). Polymerase chain reaction findings were positive in 25 of 68 positive controls (37%) and negative in all 11 cases of lichen sclerosus and all 15 negative controls.

Conclusions  Focus-floating microscopy is a reliable method to detect Borrelia species in tissue sections. The frequent detection of this microorganism, especially in early lichen sclerosus, points to a specific involvement of B burgdorferi or other similar strains in the development or as a trigger of this disease.

Design  We undertook a case-control study of herpes zoster, which represents reactivation of latent varicella zoster virus residing in dorsal root ganglia following primary infection, involving 504 patients and 523 controls. Interviews were conducted by trained medical investigators using a structured questionnaire.

Setting  The Center for Clinical Studies, an outpatient clinic and research center in Houston, Texas.

Participants  Nonimmunocompromised patients with confirmed cases of herpes zoster were included in the study. Controls were nonimmunocompromised clinic patients with new diagnoses of skin diseases other than herpes zoster.

Results  Cases were more likely to report blood relatives with a history of zoster (39%) compared with controls (11%; P < .001). Risk was increased with multiple blood relatives (odds ratio, 13.77; 95% confidence interval, 5.85-32.39) compared with single blood relatives (odds ratio, 4.50; 95% confidence interval, 3.15-6.41).

Conclusions  The results suggest an association between herpes zoster and family history of zoster. Future studies will be needed to investigate this association.

Design  Retrospective study (January 1, 1999-December 31, 2003).

Setting  The Department of Dermatology and Venerology, Karolinska University Hospital.

Patients  A total of 138 consecutive patients with bullous pemphigoid were included and were grouped according to the treatment they received: methotrexate, prednisone, a combination of both, or topical glucocorticoids (for mild disease).

Result  Methotrexate was the most effective treatment, with only a few adverse effects and a tendency toward better survival rates in patients with moderate to severe disease.

Conclusion  Methotrexate is an effective and safe drug and provides an excellent treatment option in patients with bullous pemphigoid.

Design  Randomized, double-blind clinical trial.

Setting  University dermatology department.

Patients  Twenty healthy volunteers with Fitzpatrick skin types I (highly sensitive, always burns easily, tans minimally) through III (sun-sensitive skin, sometimes burns, slowly tans to light brown).

Intervention  Seven 34-cm² areas were marked on the upper aspect of the back of each participant. An untreated area was tested to determine UV sensitivity. Two areas were treated with excess amounts (2 mg/cm²) of either a moderate-potency corticosteroid or a high-potency corticosteroid 30 minutes before UV-B exposure as controls. Six or 23 hours after exposure to radiation, the remaining areas were treated with the 2 corticosteroid preparations.

Main Outcome Measures  The sunburn improvement factor (SIF) was determined by the following equation: SIF = MED (minimal erythema dose) on treated skin/MED on nontreated skin. An SIF greater than 1 indicated an effect of topical corticosteroids in sunburn relief.

Results  The SIFs in the areas treated with either topical corticosteroid 30 minutes before UV-B exposure or high-potency corticosteroid 6 hours after UV-B exposure were significantly different from SIFs in areas that received no treatment (SIF 1.1-1.7; P < .05). Only the median SIF of 1.7 in the areas treated with high-potency corticosteroid 30 minutes before UV-B exposure was clinically relevant. The areas treated 23 hours after UV-B exposure and the areas treated with a moderate-potency corticosteroid 6 hours after UV-B exposure showed no significant reduction in redness.

Conclusion  Treatment with topical moderate-potency or high-potency corticosteroids does not provide a clinically useful decrease in the acute sunburn reaction when applied 6 or 23 hours after UV exposure.

Clinical Trial Registry  clinicaltrials.gov Identifier: NCT00206882

Design  Retrospective population-based study using survey of cancer registries and pathology laboratories, and questionnaires to physicians.

Setting  Five regions covering 19.2% of the French territory and including 8.2 million inhabitants.

Patients  Incident cases of patients with stage I to stage II (hereinafter, stage I-II) tumors staged according to the American Joint Committee on Cancer Staging guidelines and nodal stage III CM in 2004.

Main Outcome Measures  Modalities of diagnosis and excision, surgical margins, sentinel lymph node biopsy, adjuvant therapies and surveillance procedures, and their variations according to age, sex, residence, location of primary CM, Breslow thickness, type of physicians, modalities of decisions, and health care patterns.

Results  Clinical stage I-II CMs (n = 710 cases) slightly predominated in females (53%), with a lower mean Breslow thickness (1.4 mm) than in males (1.9 mm). Initial excisions were most often performed by private dermatologists and wide excisions by surgeons. Narrow margins (8%) were associated with advanced age, higher Breslow thickness, and head location. Sentinel lymph node biopsy was performed in 34% of CMs thicker than 1.0 mm, depending on geographical regions, distance from reference centers, and health care patterns. Adjuvant therapies (mainly low-dose interferon) were proposed in 53% of thick CMs (>1.5 mm), depending on the patient's age and geographical region. In contrast with French recommendations, surveillance procedures frequently included systematic medical imaging. Stage III nodal CMs (n = 89 cases) predominated in males (62%). After lymphadenectomy, adjuvant therapies (including high-dose interferon in 32% of cases and chemotherapies in 24% of cases) were proposed in 68% of cases, depending on the patient's age and geographical region. A complete 1-year high-dose interferon regimen was administered in less than 10% of cases.

Conclusion  Large disparities still exist in the management of CM in France, depending to a greater extent on medical and geographical environment than on the characteristics of either patients or tumors.

Design  Prospective case series of patients injected with PAIs.

Setting  A university tertiary teaching hospital.

Patients  A prospectively acquired series of 25 patients with severe and/or persistent delayed adverse effects after PAI injection. The patients underwent clinical follow-up, a battery of blood tests, and when possible, biopsy and chest radiography.

Main Outcome Measures  Clinical evaluation of granulomas, skin manifestations, and other local and systemic immune-mediated disorders possibly related to PAIs.

Results  The average latency period for onset of symptoms was 13.4 months. Eight patients were previously injected with another implant. Tender inflammatory nodules were seen in 24 patients. Systemic or distant manifestations appeared in 6 cases. Laboratory abnormalities were found in 20 cases. After an average of 21.3 months of follow-up, 11 patients appeared to be free of adverse effects, and 10 still had recurrent bouts.

Conclusion  Although infrequent, delayed and recurrent chronic inflammatory and granulomatous reactions may complicate PAI fillers.

Observations  Four patients with pyodermic eruptions or neutrophilic dermatosis had chromosomal abnormalities in bone marrow cells, including del(20)(q11;q13.3) in 2 patients, trisomy 8 in 1 patient, and t(11;22)(q23;q11) in 1 patient. Three patients without morphologic findings suggestive of MDS were diagnosed as having refractory anemia. One female patient had refractory anemia with ringed sideroblasts associated with del(20). Two patients with refractory anemia had a normal karyotype in peripheral blood lymphocytes. Two patients with elevated serum levels of granulocyte colony-stimulating factor had more active or widespread cutaneous diseases.

Conclusions  Karyotypic analysis of bone marrow cells, but not of peripheral blood lymphocytes, is essential in proving a diagnosis of MDS-associated pyodermic lesions. The overexpression of granulocyte colony-stimulating factor, which may compensate for impaired hematopoiesis in patients with MDS, seems to be a key cytokine leading to neutrophilic infiltration.

Observations  A 70-year-old woman with erythematous plaques and papules of the submammary and inguinal skin attended our outpatient clinic and was diagnosed as having LCH. Organ involvement was found in the infundibulum of the pituitary gland, associated with diabetes insipidus centralis, bone marrow infiltration, and several micronodules of the thoracic and lumbar spine and lungs. Based on the Histiocyte Society's LCH-A1 study in adults, the patient was treated for 12 months with a combination of corticosteroids, vinblastine, and mercaptopurine. No major adverse effects were observed. The skin was also treated with a combination of psoralen–UV-A and local corticosteroids. Restaging revealed regression of all clinical symptoms (skin involvement and diabetes insipidus centralis) and regression of organ infiltration (pituitary gland, bone marrow, and lungs).

Conclusion  Effective treatment of adult multisystemic LCH disease was achieved using prednisolone, vinblastine, and mercaptopurine, which was well tolerated.

Observations  A 49-year-old man developed KS on his wrist after 2 years of long-term immunosuppressive therapy with prednisone, methotrexate, and dapsone for well-controlled PV. Three months after the substitution of methotrexate with sirolimus, the KS gradually resolved. With the patient on a maintenance regimen of sirolimus, in conjunction with low-dose prednisone and dapsone therapy, KS and PV have remained in remission, without further recurrence, during a 24-month follow-up period.

Conclusion  The present case introduces a novel therapy for this patient population, highlighting the efficacy of sirolimus in treating iatrogenic KS without sacrificing the immunosuppression necessary to maintain control of PV.

Observations  For 16 months, we observed a 78-year-old patient with autoantibody-mediated bullous pemphigoid who was treated with IVIg and an adjuvant therapy on 2 separate occasions as well as IVIg alone on 2 other occasions. We observed the greatest depression of bullous pemphigoid antibodies when IVIg was combined with an immunosuppressive agent.

Conclusion  These results support the hypothesis that agents that suppress antibody synthesis can offset the rebound in the level of individual antibody that follows their depletion and thus can improve the effectiveness of IVIg treatment while reducing the cost and the potential toxic effects of therapy.