Solar urticaria: Epidemiology and clinical phenotypes in a Spanish series of 224 patients. / Urticaria solar. Epidemiología y fenotipos clínicos en una serie española de 224 pacientes.

BACKGROUND: Solar urticaria is a chronic inducible urticaria also classified as an idiopathic dermatosis. The objective of this paper is to define the phenotypic characteristics of solar urticaria and to evaluate its incidence. MATERIAL AND METHOD: This was a retrospective multicenter study in which data were gathered on the epidemiology and clinical, photobiologic, laboratory, and therapeutic characteristics of solar urticaria. RESULTS: A total of 224 patients (141 women and 83 men) were included from 9 photobiology units. The mean age of the patients was 37.9 years (range, 3-73 years). A history of atopy was detected in 26.7%, and the most common presentation was allergic rhinitis (16.5%). Clinical signs were limited to sun-exposed areas in 75.9% of patients. The light spectrum most commonly implicated was visible light only (31.7%), and in 21% of cases it was only possible to trigger solar urticaria with natural light. The treatments most widely used by photobiology experts were oral antihistamines (65.46%), followed by different forms of phototherapy (34%). Complete resolution was observed most often in patients with solar urticaria triggered exclusively by visible or natural light, with statistically significant differences with respect to other wavelengths (P<.05). No increase in the annual incidence of solar urticaria was observed. CONCLUSIONS: We have presented the largest series of solar urticaria published to date. The epidemiological, clinical, and photobiologic findings confirm previously reported data, although there was a particularly high rate of negative phototests in our series. Reactivity exclusively to visible or natural light was associated with a higher probability of resolution. No increasing trend was observed in the annual incidence.

Determination of minimal erythema dose and anomalous reactions to UVA radiation by skin phototype.

BACKGROUND: Phototesting is a technique that assesses the skin's sensitivity to UV radiation by determining the smallest dose of radiation capable of inducing erythema (minimal erythema dose [MED]) and anomalous responses to UV-A radiation. No phototesting protocol guidelines have been published to date. METHODOLOGY: This was a multicenter prospective cohort study in which 232 healthy volunteers were recruited at 9 hospitals. Phototests were carried out with solar simulators or fluorescent broadband UV-B lamps. Each individual received a total of 5 or 6 incremental doses of erythemal radiation and 4 doses of UV-A radiation. The results were read at 24hours. RESULTS: At hospitals where solar simulators were used, the mean (SD) MED values were 23 (8), 28 (4), 35 (4), and 51 (6) mJ/cm(2) for skin phototypes i to iv, respectively. At hospitals where broadband UV-B lamps were used, these values were 28 (5), 32 (3), and 34 (5) mJ/cm(2) for phototypes ii to iv, respectively. MED values lower than 7, 19, 27, and 38 mJ/cm(2) obtained with solar simulators were considered to indicate a pathologic response for phototypes I to IV, respectively. MED values lower than 18, 24, and 24mJ/cm(2) obtained with broadband UV-B lamps were considered to indicate a pathologic response for phototypes ii to iv, respectively. No anomalous responses were observed at UV-A radiation doses of up to 20J/cm(2). CONCLUSIONS: Results were homogeneous across centers, making it possible to standardize diagnostic phototesting for the various skin phototypes and establish threshold doses that define anomalous responses to UV radiation.

Foreign body granulomatous reaction to silica, silicone, and hyaluronic acid in a patient with interferon-induced sarcoidosis.

We report the case of a patient who developed sarcoid granulomas 11 months after starting treatment with pegylated interferon alfa and ribavirin for chronic hepatitis C. The sites of the lesions were related to 3 different foreign bodies: silica in old scars on the skin, hyaluronic acid that had been injected into facial tissues, and silicone in an axillary lymph node draining the area of a breast implant. Systemic sarcoidosis was diagnosed on the basis of a history of dry cough and fever and blood tests that revealed elevated angiotensin converting enzyme and liver enzymes. Interruption of the antiviral therapy led to normalization of liver function tests and disappearance of the skin lesions and lymphadenopathies. Dermatologists and cosmetic surgeons should be aware of the risk of sarcoid lesions related to cosmetic implants in patients who may require treatment with interferon in the future.

Bullous pemphigoid and multiple sclerosis: a report of two cases with ELISA test.

We report two patients with longstanding multiple sclerosis (MS) who developed vesicles and bullae consistent with the diagnosis of bullous pemphigoid (BP). Both patients showed linear IgG at the dermal-epidermal junction, located on the epidermal side of patients' skin previously treated with 1M NaCl. In the two cases, the ELISA test was positive for the extracellular fragment of BP 180. However, the indirect immunofluorescence test (IIF) was repeatedly negative. Therapy either with prednisone plus dapsone or prednisone alone was initiated and the disease was controlled after 23 and 15 months of therapy, in patients 1 and 2, respectively. However, the first patient had a flare-up 2 months after treatment was stopped. The association of MS and BP has been described previously in 35 cases. We compare our two cases with the 25 patients previously reported in detail in the literature. We emphasize the role of the ELISA test in establishing the diagnosis of BP.

[Elastosis perforans serpiginosa in a patient with pseudoxanthoma elasticum]. / Elastosis perforans serpiginosa en una enferma afecta de pseudoxantoma elástico.

A 34-year-old female previously diagnosed of pseudoxanthoma elasticum developed an annular plaque with serpiginous borders of 42 by 30 mm in diameter on the inner left arm. A similar lesion later appeared on the inner right arm. Histopathological examination of a papule showed short, fragmented, granular, basophilic and calcified elastic fibers in the mid-reticular dermis. The epidermis showed hyperplasia surrounding degenerated and normal elastic fibers. Transepidermal elimination channels of these elastic fibers were also observed. These findings were consistent with the diagnosis of elastosis perforans serpiginosa. Abundant multinucleated giant cells were observed surrounding the area of epidermal hyperplasia and in the reticular dermis. The patient was treated with tazarotene, and the plaques disappeared in 9 months.

Refractory Cutaneous IgA Vasculitis Treated with Omega-3 Fatty Acids.

BACKGROUND: Omega-3 fatty acids (O3FA) have been used to treat IgA nephropathy (IgAN) but not cutaneous IgA vasculitis (IgAV). CASE REPORT: A 47-year-old female was referred for cutaneous vasculitis. She had a 24-year history of flares of palpable purpura, arthralgia associated with hematuria, and proteinuria. We diagnosed cutaneous IgAV associated with IgAN. We administered prednisone at doses ranging from 10 to 45 mg/day to control the flares. To reduce prednisone exposure, different therapeutic strategies (colchicine, diphenhydramine, hydroxyzine, azathioprine, benzathine penicillin, and mycophenolate mofetil) were applied without success. After 11 years, therapy with O3FA capsules containing 460 mg eicosapentaenoic acid and 380 mg of docosahexaenoic acid t.i.d. was introduced, allowing the prednisone to be stopped 2 years later. When the dose of O3FA was decreased to 1 capsule on alternate days, the cutaneous flares reappeared, but they were again controlled when the patient took 1 O3FA capsule daily. CONCLUSIONS: O3FA can be useful to control cutaneous IgAV.

Detection of Mycobacterium tuberculosis DNA in lobular granulomatous panniculitis (erythema induratum-nodular vasculitis).

OBJECTIVE: To determine, using polymerase chain reaction (PCR) amplification, if Mycobacterium tuberculosis complex DNA is present in the skin biopsy specimens of lobular granulomatous panniculitis. DESIGN: A retrospective descriptive study. SETTING: A university-based hospital. PATIENTS: From the 65 patients included in the study, we examined 72 paraffin-embedded skin biopsy specimens with a histologic diagnosis of erythema induratum or nodular vasculitis. The biopsy specimens were from the histopathological archives of the Departments of Dermatology and Pathology of the Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, from 1976 to 1994. Twenty-two biopsy specimens were excluded from the final analysis because we could not amplify the internal control. MAIN OUTCOME MEASURES: Detection of a 123-base pair fragment of the IS6110 insertion sequence specific for M tuberculosis complex. RESULTS: The results of PCR amplification were positive for M tuberculosis complex DNA in 77% of the skin biopsy specimens. No significant difference could be detected with respect to the age of the patients, ulceration of the nodules, reactivity to purified protein derivative, abnormal results of a chest x-ray examination, personal and family history of tuberculosis, and PCR results. The presence and degree of necrosis on histologic examination were significantly higher in the PCR-positive group (P = .04). None of the following variables were associated with PCR results: presence of vasculitis, degree of granulomatous infiltrates, number of giant cells, and presence of well-organized granulomas. CONCLUSIONS: The DNA of M tuberculosis can be detected in a considerable number of skin biopsy specimens of lobular granulomatous panniculitis. None of the clinical and histologic variables evaluated could accurately predict the results of PCR amplification.

Cutaneous ulcers with type I cryoglobulinemia treated with plasmapheresis.

Severe necrotic cutaneous ulcers and kidney involvement secondary to type I cryoglobulinemia can be a therapeutic challenge. Plasmapheresis has been reported useful to treat autoimmune diseases such as thrombotic thrombocytopenic purpura, systemic lupus erythematosus, myasthenia gravis and Goodpasture's syndrome. We report the case of a patient who presented necrotic lesions with kidney involvement due to type I cryoglobulinemia (Ig G kappa) that evolved to a multiple myeloma. Treatment with high doses of corticosteroid plus cyclophosphamide did not control the disorder. Therapy with plasmapheresis produced a marked decrease in cryoglobulin levels and a subsequent relevant clinical improvement of cutaneous lesions and renal function. In cryoglobulinemia, plasmapheresis can be used as effective adjunt therapy to minimize cutaneous, renal and/or neurologic involvement.

Oral ulcerations as the first sign of a foramen magnum meningioma.

We report the case of a patient with oral ulcerations that were likely traumatic in origin caused by biting of the tongue. Physical examination showed only deviation, fasciculations, and hemiatrophy of the right side of the tongue. Neurologic examination disclosed an isolated XII nerve palsy. A magnetic resonance image showed a lesion in the posterior fossa displacing the brain stem at a bulbar level. This was consistent with the diagnosis of a meningioma, later confirmed by the histopathologic study. It is very unusual that a meningioma produces a selective involvement of the XII nerve. Early diagnosis of a foramen magnum meningioma is important, both to improve prognosis and to avoid neurologic sequelae.

[Hematologic deficiencies in patients with recurrent oral aphthae]. / Estudio de los déficit hematológicos en los enfermos afectados de aftas orales recidivantes.

BACKGROUND: The prevalence of iron, folic acid and vitamin B12 deficiencies and their role in the development of recurrent oral ulcerations is not well known. The aim of this study was to determine the prevalence of these deficiencies in our patients. PATIENTS AND METHODS: Iron, folic acid and vitamin B12 levels were studied in 80 patients with recurrent oral ulcerations (ROU) and the results were compared with a control group of 29 patients with different oral diseases. RESULTS: In the recurrent oral ulcers patients, deficiencies were detected in 21/80 patients (26.2%). In 18 cases they were pure: iron (4), folic acid (10) and vitamin B12 (4). In 3 patients, combined deficiencies were detected, being secondary to pernicious anaemia in two patients. In the control group, deficiencies were observed in 4/29 cases (13.7%). In three cases they were isolated (one case suffered from ferropenic anaemia and two patients of pernicious anaemia). CONCLUSIONS: Patients with recurrent oral ulcerations have more frequently iron, folic acid and vitamin B12 deficiencies than those with other diseases of oral mucosa. However, there were not significant differences when the frequency of deficiency of each one of such elements were taken into account separately.

[Usefulness of polymerase chain reaction for the diagnosis of Bazin erythema induratum]. / Utilidad de la reacción en cadena de la polimerasa para el diagnóstico del eritema indurado de Bazin.

BACKGROUND: Erythema induratum of Bazin (BEI), is included in the group of cutaneous granulomatous lobulillar panniculitis. The aethiopathogenic association between EI and tuberculosis can not rely on the clinicohistological features of these panniculitis and M. tuberculosis has never been isolated from BEI lesions. Detection of the mycobacterial DNA by PCR on cutaneous biopsy samples would allow to confirm this association. PATIENTS AND METHODS: Fourteen patients with clinical BEI were chosen retrospectively. Seventeen lesional biopsy samples were obtained, stained with the Kinyoun carbolfuchsin acid-fast technique and haematoxylin and eosin and tested by PCR. A fragment of the IS6110 insertion sequence specific of M. tuberculosis was amplified and confirmed by digestion with Sal I restriction endonuclease. The efficiency of the procedure, the presence of inhibitory substances and the preservation of DNA were checked by PCR of the beta-actin gene. RESULTS: M. tuberculosis DNA was detected in 12 of the 17 samples tested (70.5%) which corresponded to 10 of the 14 patients (71.4%). According to beta-actin PCR results, the rate of extracted DNA was inadequate on four of the five negative biopsies. CONCLUSIONS: The results of these series suggest the probable involvement of M. tuberculosis on the BEI pathogenesis and give support to the usefulness of the PCR in the diagnosis of this pathology concerning the need of specific treatment.

[Mucocutaneous manifestations in acute HIV infection. 3 case reports]. / Manifestaciones mucocutáneas en la infección aguda por el VIH. Presentación de tres casos.

We report three patients who developed a generalized rash with oral, genital or perianal ulcerations as a result of acute infection due to HIV. The primary infection was diagnosed by seroconversion (by means of EIA and Western blot techniques). Definitive diagnosis was established on days 52, 85 and 97 after the appearance of the rash. The p24 protein of the HIV was only detected in the early phase of the disorder in the two cases in which this study was carried out.

[Woolly hair nevus associated with systemic epidermal nevus]. / Nevus de pelo lanoso asociado a nevus epidérmico sistematizado.

A 22-year-old female with two woolly hair nevus of the scalp and a systematized epidermal nevus is reported. This association has been rarely described. Scanning electron microscopy of the woolly hair demonstrates oval and triangular hair shaft sections and longitudinal grooves. The structure of the cuticle was not disturbed.