Folliculocystic and Collagen Hamartoma: A Subset of Fibrous Cephalic Plaque.

Tuberous sclerosis complex is known to cause a variety of cutaneous hamartomas, most commonly hypomelanotic macules, angiofibromas, shagreen patches, and fibrous cephalic plaques. In recent years, a new cutaneous hamartoma that bears physical and histological resemblance to fibrous cephalic plaque has been proposed called folliculocystic and collagen hamartoma. The primary difference between the two diagnoses is the histologic presence of infundibular cysts in the latter. However, some authors have called into question if the two diagnoses are truly distinct. In this case report, we present a patient with tuberous sclerosis complex and fibrous cephalic plaque with infundibular cysts and propose that the presence of cysts should be incorporated into the possible histologic features of fibrous cephalic plaque.

Firm Cutaneous Nodule on the Dorsal Hand: A Case Report on Fibroblastic Rheumatism.

Fibroblastic rheumatism (FR) is a rare dermatoarthropathy of unknown etiology. It is characterized by the onset of firm cutaneous nodules in patients with rheumatologic symptoms such as arthralgias or symmetric polyarthritis. Clinicopathologic correlation is critical in establishing the diagnosis, as the clinical manifestations can resemble other fibrosing conditions. In this report, we review the clinical and histologic features of FR, as well as conditions similar to those of the case we present, with dermatologic and rheumatologic manifestations. As part of our research, a PubMed search of the following terms was performed: "arthralgia, arthritis, arthropathy, cutaneous, disease, fibroblast, fibrosing, nodule, rheumatism, and rheumatology". This report discusses a unique case of FR diagnosed in a 37-year-old man with a single cutaneous nodule.

Superficial Clear Cell Sarcoma (Melanoma of Soft Parts) of the Large toe in an 80-Year-Old Female With a Rare Cytogenetic Translocation.

We present a case of clear cell sarcoma (CCS) on the left large toe of an 80-year-old female. CCS, also known as "melanoma of soft parts," is a rare soft tissue neoplasm that exhibits melanocytic differentiation. Most cases occur on the distal extremities of young female adults. CCS shares histopathologic and immunohistochemical features with malignant melanoma that cause diagnostic difficulties distinguishing between these entities; therefore, cytogenetic studies of specific translocations are paramount in obtaining the correct diagnosis. The majority of CCS cases reveal a t(12;22)(q13;q12) EWSR1/ATF1 translocation, while a rare subset of CCS demonstrate a t(2;22) (q32:q12) EWS/CREB1 translocation. Our patient presented with a 50-year history of a nodule on the dorsum of her left large toe, with increasing size and tenderness over the past nine months. Histopathology and immunoperoxidase staining indicated CCS as a differential diagnosis. Cytogenetic analysis revealed a translocation in t(2;22) (q32;q12) resulting in the EWSR1/CREB1 gene, confirming a diagnosis of CCS. The translocation, histologic location, and long-standing clinical course exhibited in this case are exceptionally rare, and we hope to inform dermatologists of an uncommon presentation of CCS in an unexpected age group.

Annular Lichenoid Dermatitis of Youth: A Chronic Case Managed Using Pimecrolimus.

Annular lichenoid dermatitis of youth, first described in 2003, is a rare and occasionally chronic skin disease. We report a case of annular lichenoid dermatitis of youth relapsing over the course of 5 years successfully treated and maintained with topical pimecrolimus cream.

Laser-assisted tattoo removal with topical 5% imiquimod cream.

BACKGROUND: Laser-assisted tattoo removal is effective but can be costly and time-consuming and can result in disfiguring scars and pigment alterations. Imiquimod, an immune response modifier, may play a role in tattoo removal. OBJECTIVE: The objective was to evaluate the safety and efficacy of topical 5% imiquimod cream used daily in conjunction with laser therapy to remove unwanted tattoos. MATERIALS AND METHODS: Twenty subjects with two similar tattoos were enrolled in this randomized, prospective, double-blinded, case-controlled study. Tattoos were treated with either imiquimod or placebo daily and laser therapy every 4 to 6 weeks for a total of six sessions. The primary efficacy parameter was tattoo clearance (5-point scale, poor through complete). Secondary efficacy parameters included textural changes (5-point scale, minimal through severe), pain during and between laser procedures, and undesirable pigment alterations. RESULTS: Nineteen subjects completed the study. The mean score for tattoo clearance with imiquimod versus placebo was 3.2 versus 2.9 and, for textural changes, was 1.37 versus 1.21 (differences not statistically significant). There was no difference in subjective pain during and between laser sessions and no undesirable pigment alterations were reported. Adverse reactions were more frequent with imiquimod compared to placebo. CONCLUSION: Topical imiquimod is an ineffective adjunct to laser-assisted tattoo removal.

Eosinophilic folliculitis: before and after the introduction of antiretroviral therapy.

OBJECTIVE: To characterize the relationship of new eosinophilic folliculitis (EF) cases between June 30, 1994, and January 5, 2000, and antiretroviral therapy (ART) status and immune reconstitution. DESIGN: Retrospective cohort analysis. SETTING: Dermatology clinics at a county hospital. SUBJECTS: Fifty-seven consecutive subjects with biopsy-proved EF from the pathology database. Subject groups were as follows: naïve to ART, receiving ART without protease inhibitors/nonnucleoside reverse transcriptase inhibitors, and receiving ART containing protease inhibitors/nonnucleoside reverse transcriptase inhibitors. MAIN OUTCOME MEASURES: Onset of EF, CD4 cell count and nadir at EF onset, and time of ART initiation. RESULTS: Among the 3 groups previously described, mean CD4 cell counts (86.26/microL vs 113.82/microL vs 145.65/microL, respectively [Kruskal-Wallis rank sum test, P = .15]) and nadir (68.43/microL vs 66.18/microL vs 64.17/microL, respectively [Kruskal-Wallis rank sum test, P = .41]) at EF diagnosis were not statistically different. Fifty-two subjects (91%), regardless of treatment group, had a nadir below 200/microL. Of the subjects undergoing ART, 28 (82%) developed EF within 6 months of initiating ART; their average CD4 cell count increase was 108/microL. Of the 23 subjects receiving protease inhibitor/nonnucleoside reverse transcriptase inhibitor-containing ART regimens, 17 (74%) were diagnosed as having EF within 3 months, with 4 additional subjects diagnosed as having EF within 6 months (a total of 21 [91%] of the 23 subjects). This is not significantly different from the 7 (64%) of 11 subjects diagnosed as having EF at 3 and 6 months of starting ART without protease inhibitors/nonnucleoside reverse transcriptase inhibitors (P = .07) (odds ratio, 0.18; 95% confidence interval, 0.01-1.54). CONCLUSIONS: Our study shows an association between low nadir (66.28/microL) and low CD4 cell count (115.54/microL) and the development of EF, regardless of subjects' ART status. However, most subjects receiving ART were diagnosed as having EF within 3 to 6 months of ART initiation, regardless of the regimen.

Dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris.

OBJECTIVE: To determine the effect of dapsone on glucocorticoid-dependent patients with active or maintenance-phase pemphigus vulgaris. DESIGN: Retrospective study of consecutive patients treated with dapsone. SETTING: University of Pennsylvania, Philadelphia (a tertiary referral hospital). Patients We observed 9 consecutive adult patients with pemphigus vulgaris being treated with immunosuppressants who were unable to taper prednisone use without abrupt worsening of their disease.Interventions Dapsone treatment added to prednisone and other immunosuppressive therapy. Main Outcome Measure Steroid dosage. RESULTS: All patients were unable to taper their steroid dose during the 3 months prior to the initiation of dapsone therapy or had active disease that was not well controlled by prednisone prior to dapsone treatment. With the exception of 1 patient with uncontrolled disease, all 9 patients were able to taper their steroid dose below the adrenal replacement level during dapsone treatment. Maintenance-phase patients taking 15 mg/d or more of prednisone (n = 5) experienced a mean +/- SEM drop of 67% +/- 7.1% in prednisone dose by 4 months of maximal dapsone treatment and an 84% +/- 3.5% drop in prednisone dose after 8 months of dapsone treatment. CONCLUSIONS: These retrospective study findings suggest that dapsone reduces steroid dependence in patients with pemphigus vulgaris, provided they are in the maintenance phase of their disease. These data support the need for a prospective, randomized trial to confirm these findings.

Cyclosporine-induced folliculodystrophy.

We describe a 34-year-old kidney transplant patient who developed a distinct cutaneous side-effect to cyclosporine manifested as an infiltrated appearance to the skin with abundant flesh-colored, follicular papules predominantly affecting the ears, nose, and surrounding areas of the face, but also the trunk and extremities. The clinical and histologic findings in this case closely match those presented in 2 previous case reports, in which immunosuppressive doses of cyclosporine appeared to be causative. We present a detailed report of the clinical and histologic findings that are unique to these 3 cases and we introduce a theory, based on the recent in vitro studies involving cyclosporine, to help explain the pathogenic events induced by cyclosporine in these patients. We propose the term "cyclosporine induced folliculodystrophy" or CIF as an appropriate name for this distinct clinicohistopathologic entity. Finally, we set forth 3 clinical and 4 histopathologic criteria upon which the diagnosis of CIF can be made.