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.

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.

Reacción granulomatosa a cuerpo extraño a sílice, silicona y ácido hialurónico, en paciente con sarcoidosis inducida por interferón / Foreign Body Granulomatous Reaction to Silica, Silicone, and Hyaluronic Acid in a Patient With Interferon-Induced Sarcoidosis

Se describe el caso de una paciente que desarrolló granulomas sarcoideos 11 meses después de haber iniciado interferón α pegilado y ribavirina, como tratamiento de la hepatitis crónica C. Las lesiones se localizaban en relación a 3 cuerpos extraños diferentes: sílice en cicatrices cutáneas antiguas, ácido hialurónico que se había inyectado previamente en la cara, y silicona que se detectó en una adenopatía axilar donde había drenado de un implante mamario previo. La paciente también aquejaba tos seca, fiebre y en la analítica se detectó un incremento de la enzima convertidora de angiotensina y de las enzimas hepáticas. A partir de estos hallazgos se diagnosticó de sarcoidosis sistémica y se suspendió el tratamiento antiviral con posterior normalización de las pruebas hepáticas, desaparición de las lesiones cutáneas y de las adenopatías. Los dermatólogos y cosmetólogos deben ser conscientes del riesgo de aparición de manifestaciones sarcoideas en las áreas donde se han realizado implantes estéticos, en los sujetos que en un futuro requieran tratamiento con interferón (AU)

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 (AU)

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.

[A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis]. / Hematopoyesis extramedular cutánea en mielofibrosis idiopática: a propósito de un caso.

Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.

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.

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

Mujer de 34 años de edad diagnosticada de pseudoxantoma elástico (PXE) consultó por presentar una placa anular de márgenes serpiginosos, que alcanzó 42 × 30 mm de diámetro en la cara interna del brazo izquierdo. Posteriormente le apareció otra lesión similar de menor tamaño en la cara interna del brazo derecho. El examen histopatológico de una pápula evidenció una alteración de las fibras elásticas, junto a otras de aspecto normal, en la dermis reticular alta y media. Eran cortas, deshilachadas, onduladas, agrupadas unas con otras. La epidermis mostraba una hiperplasia epitelial, que englobaba cúmulos de fibras elásticas degeneradas y otras de aspecto normal. También se apreciaban canales con eliminación transepidérmica de dichas fibras elásticas. Estos hallazgos eran compatibles con el diagnóstico elastosis perforans serpiginosa (EPS). Destacaba la presencia de abundantes células gigantes multinucleadas alrededor de la zona de hiperplasia epitelial y en la dermis reticular. Se trató con tazaroteno y las placas desaparecieron en 9 meses

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

Direct immunofluorescence in granuloma faciale: a case report and review of literature.

A 49-year-old male presented at our department with erythematous brownish plaques in the malar areas and left cheek of 9 years' evolution. Histopathological study revealed a dense, predominantly perivascular, inflammatory infiltrate in the reticular dermis. The infiltrate was composed of abundant neutrophils, lymphocytes, histiocytes, and eosinophils. Leukocytoclasia and fibrin in some vessel walls were also observed. The patient was diagnosed with granuloma faciale (GF). Direct immunofluorescence (DIF) study showed heavy immunoglobulin G (IgG) and less intense deposits of IgA, IgM, C(3) and C(1q) surrounding superficial and deep vessels of the reticular dermis. Perivascular and diffuse fibrinogen deposits were also present. No deposits were detected at the dermoepidermal junction. Our findings lend support to the hypothesis that classical pathway activation of complement may be involved in the development of vasculitis in GF.