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Pyoderma gangrenosum is an uncommon ulcerative skin disorder that is often associated with underlying systemic diseases, the most common of which is inflammatory bowel disease. We report an illustrative case of a 36-year-old woman with pyoderma gangrenosum occurring at surgical sites and at sites of trauma. She had associated Crohn disease. Multiple surgical procedures were performed on this patient, without improvement. Pyoderma gangrenosum can mimic a necrotizing soft tissue infection. Early recognition of the characteristic lesion may prevent unnecessary operations and facilitate effective control with appropriate medical therapy.

Seventy-four adult patients with facial, truncal, and extremity port-wine stains (PWS) were treated with the flashlamp-pumped pulsed dye laser (PDL) with laser output ranging from 6.0 to 7.5 J/cm2. Response to treatment was analyzed by comparing the area of involvement following each treatment with the area of involvement measured in the first treatment session. All the PWS responded with 25 to 90 percent lightening, and 85.1 percent of patients achieved 25 percent clearing. However, only 36.5 percent achieved 50 percent clearing, and none of the patients achieved 100 percent clearing. None of four patients with PWS greater than 100 cm2 achieved 50 percent clearing following a mean of 17.2 +/- 5.7 treatments. These data emphasize the importance of objectively documenting clinical response to PDL treatment of PWS. Adult patients need to be made aware that complete clearing may not be obtainable by PDL treatment alone. This is especially important for adult patients with PWS larger than 100 cm2.

All forms of cutaneous T-cell lymphoma are rare in childhood. We describe an 8-year-old boy with plaque-stage mycosis fungoides stage IIA whose cutaneous eruption had been present for 5 years. Histologic examination revealed the presence of a granulomatous infiltrate together with atypical lymphocytes within the dermis. The child had an excellent response to combination psoralen-UVA (PUVA) with interferon-alpha 2a treatment and is currently in remission.

Topical corticosteroids have been one of the cornerstones of dermatologic treatment for nearly 40 years. At present the most widely used topical steroid ranking system is the vasoconstrictor assay. To determine whether this is a satisfactory measure of the agents' potency in clinical settings, we examined rankings from the published literature achieved by four different methods: vasoconstriction, clinical outcome, therapeutic index (a ratio of efficacy to systemic safety), and cost, safety, and efficacy. Overall clinical outcome (efficacy) rankings in this study corresponded in only 11 (62%) of 17 topical steroid preparations with the expected vasoconstrictor rankings. The therapeutic index rankings did not correspond with the clinical outcome (33%) or the vasoconstrictor assay (33%) rankings. It was difficult to compare studies because of lack of standardization of clinical trials. We urge that topical steroid rankings not be based solely on vasoconstrictor assays, and that a standard method be developed for clinical trials of the drugs to allow for comparison among preparations.

Objective: To review recent studies of systemic therapy for mycosis fungoides and the Sézary syndrome (cutaneous T-cell lymphomas)., Data Sources: English-language articles indexed in MEDLINE from 1988 through 1994., Study Selection: All therapeutic studies were selected., Data Extraction: The data were abstracted without judgments on response criteria or patient numbers. Data quality and validity were assessed by independent author reviews., Data Synthesis: No systemic therapy cures patients with cutaneous T-cell lymphomas. Single and combined chemotherapeutic agents produce high response rates. Whether any of these is preferred is not established. A randomized trial comparing combination chemotherapy plus radiation therapy with topical therapy showed no survival benefit for the combination. Several adenosine analogs and retinoids were active, but their optimal use is uncertain. Interferons are as active as chemotherapeutic agents and may be less toxic. Interferon combined with psoralen plus ultraviolet A light therapy produces high complete response rates and long-lasting remissions. Combinations with other systemic therapies do not increase response rates. Photopheresis therapy should be regarded as experimental. Promising preliminary results were seen with interleukin-2 fusion toxins and several antibody conjugates., Conclusions: Systemic therapy should be considered effective and palliative. The principles of treating all low-grade lymphomas can be applied. Randomized trials are needed to evaluate new agents (such as a comparison of psoralen plus ultraviolet light with or without interferon), and large phase II trials are needed for new agents such as photopheresis, interleukin-2 fusion toxin, temozolomide, and others.

Minoxidil is a new, effective, investigational antihypertensive drug. A serious, cosmetic side effect in women is hypertrichosis. Five female patients with this complication had successful removal of the excessive hair with a calcium thioglycolate depilatory agent.

Herpes gestationis (HG) is a rare, blistering disease of pregnancy and the puerperium that has immunologic findings of C3 with or without IgG deposited in a linear, band-like distribution along the dermoepidermal basement membrane zone (BMZ). A circulating IgG factor has been demonstrated that will fix complement by the classic pathway in vitro. This study involves a patient who had classic HG with C3 at the BMZ, and who had a circulating factor with activity via the alternative complement pathway similar to the previously described C3 nephritic factor found in patients with partial lipodystrophy, systemic lupus erythematosus, and membranoproliferative glomerulonephritis. Like the C3 nephritic factor, the activity was detected in IgG fractions of serum samples.

A case report of photosensitive recurrent erythema multiforme occurring in a 31-year-old man is presented herein. Lesions clinically and histologically resembling erythema multiforme were reproduced with light testing. The patient's lesions appeared to respond to both oral prednisone and hydroxychloroquine.

Pyrimethamine, a folate antagonist similar to methrotrexate without known hepatotoxcity in man, was administer orally, once a week, to seven patients with severe, long-standing psoriasis. Four patients obtained a good to excellent response; a fifth withdrew from the study early after a partial response; a sixth had minimal response to high doses, and the seventh was withdrawn due to a probable drug-induced hepatotoxic reaction. Hepatotoxic reaction was suspected in another patient with abnormal liver biopsy and experienced severe gastrointestinal toxic reactions and two developed significant thrombocytopenia, which resolved after oral treatment with folic acid or discontinuation of the drug. These did not recur at lower doses. Pyrimethamine appears to be effective in severe psoriasis, but offers no advantage, and several disadvantages over methotrexate. Pyrimethamine is probably hepatotoxic. It should be used with caution in patients with liver disease or impaired renal function; pretreatment liver biopsy is recommended.

Angiolymphoid hyperplasia with eosinophilia (AHE) is a distinct clinical and pathologic entity that demonstrates proliferation of endothelial cells associated with varying degrees of lymphocytic, histiocytic, and eosinophilic infiltration. Lymphoid hyperplasia with germinal centers often is present in older lesions and the patients may have an associated blood eosinophilia. Multiple deep nodules developed in a young man on the palmar aspect of the left hand, and subsequently the forehead, histologically diagnostic of AHE. Direct immunofluorescence of the tumor removed from the forehead demonstrated granular deposits of IgA, IgM, and C3 associated with small vessels. Cryoglobulins (IgA, IgM, and IgG) were found in the patient's serum. This new information lends support to the hypothesis that AHE is an inflammatory reactive lesion, possibly secondary to an immunologic injury, rather than a true vascular neoplasm with an associated inflammatory reaction.

The four male patients with multiple hamartoma syndrome (Cowden's disease) in this report, have most of the previously reported findings associated with this syndrome and several important unreported findings that include multiple cutaneous trichilemmomas, cafe-au-lait spots, cutaneous squamous cell carcinoma, pathologic fracture, craniomegaly, probable malignant lung tumor, retinal glioma, drusens of the optic disk and retina, pseudotumor cerebri, mediastinal mass, and multiple small papillomatous lesions of the esophagus, stomach, and duodenum.

Two patients with tuberous sclerosis but without mental retardation were dermabraded for adenoma sebaceum. The treatment was effective and cosmetically satisfying.

Past and current therapies for recurrent mucocutaneous herpes simplex infections are reviewed. At present, no topical or systemic therapy has been proved safe and effective in scientific double-blind studies. Recent treatment with flexible collodion is discussed.

Three cases of an unusual Pseudomonas skin infection acquired from a health spa whirlpool were reported, and an epidemiologic survey showed 17 additional cases. This dermatosis is characterized by the abrupt onset of urticarial papules and superficial and deep papulopustules that spare only the head and neck and occur eight to 48 hours after using the whirlpool. Cultures from one patient's pustules and from the spa whirlpool environment were positive for P aeruginosa, serotype O-11. The condition cleared in all but one patient, without treatment, within seven to ten days. This patient continues to have recurrent follicular pustules three months after exposure. Samples from whirlpools at six other selected establishments were also positive for P aeruginosa.