A Case of Interstitial Pneumonitis Induced by Acitretin / 대한내과학회지

Retinoids are effective systemic agents in the treatment of psoriasis. Acitretin, a synthetic aromatic derivative of retinoic acid, has replaced etretinate in retinoid therapy of psoriasis because of its more favorable pharmacokinetic profile, including a significantly shorter half-life. Most of the adverse effects associated with acitretin are teratogenicity, hepatotoxicity, pseudotumor cerebri, pancreatitis, hyperlipidemia, hyperostosis, and mucocutaneous side effects. There are two reports worldwide describing patients who developed acute respiratory distress syndrome associated with acitretin. This suggests the possibility of serious lung complications associated with acitretin. We report a case of a 61-year-old man who developed interstitial pneumonitis that might have been induced by acitretin during the treatment of pustular psoriasis. In these cases, immediate withdrawal of retinoic acid is necessary, and corticosteroid therapy should be considered.

A Case of Impetigo Herpetiformis Treated with Cyclosporine / 대한피부과학회지

Impetigo herpetiformis is a rare, severe dermatologic disorder that is similar to a type of pustular psoriasis that predominantly occurs during the 3rd trimester of pregnancy. This condition usually resolves after delivery and it rarely continues until the postpartum period. Systemic corticosteroids have proven to be the drug of choice for the management of impetigo herpetiformis. In the postpartum period, oral retinoids such as isotretinoin, etretinate and acitretin can be used to control impetigo herpetiformis, but their side effect such as teratogenicity is a big issue to women of child-bearing age. We report here on a case of impetigo herpetiformis in a 28-year-old woman who did not respond to oral corticosteroids, but she was treated successfully with cyclosporine. To the best of our knowledge this is the first case of impetigo herpetiformis treated with cyclosporine in the Korean medical literature.

A Case of Impetigo Herpetiformis Treated with Isotretinoin / 대한피부과학회지

Impetigo herpetiformis is a rare and severe pustular disorder which primarily occurs in pregnancy. Etretinate and acitretin are effective in impetigo herpetiformis with systemic steroid. A major concern with the use of systemic retinoids is their high teratogenic potential. Isotretinoin is another retinoid but its teratogenic potential lasts only for two months. Hence, isotretinoin may be used to treat women of childbearing years with impetigo herpetiformis. We report a case of impetigo herpetiformis in a 26-year-old woman whose condition was improved with isotretinoin.

Three Cases of Porokeratosis of Mibelli Treated with 5% Imiquimod Cream / 대한피부과학회지

Porokeratosis is a disorder of epidermal keratinization that is histologically characterized by the presence of cornoid lamella. Many treatment modalities have been used, such as, surgical excision, cryosurgery, CO2 laser, oral etretinate, topical 5-fluorouracil and keratolytics. However, universal treatment guidelines have never been established. We tried 5% imiquimod cream on three patients who have porokeratosis of Mibelli which are intractable to other previous treatment modalities. Two of them showed a marked and moderate improvement, respectively. But the third patient exhibited only slight response. Herein we report our treatment experience and review the related literature.

A Case of Vesiculobullous Darier' Disease / 대한피부과학회지

Vesiculobullous Darier's disease is a rare variant of Darier's disease which is characterized by vesicles and bullae, usually distributed on the sun-exposed areas of the body. Blistering may be percipitated by factors such as high humidity, ultraviolet radiation, etretinate treatment, surgery or physical stress. Widespread vesiculation or bullous lesions are unusual in the absence of cutaneous bacterial or viral infections. Herein, we report an unusual case of vesiculobullous Darier's disease which developed on the scalp in a 25-year-old man.

A Case of Generalized Pustular Psoriasis Improved with Isotretinoin / 대한피부과학회지

Generalized pustular psoriasis is a lethal variant of psoriasis characterized by the appearance of sterile pustules on psoriatic skin. Etretinate and acitretin are effective in pustular forms of psoriasis, including generalized pustular psoriasis. A major concern with the use of systemic retinoid is their high teratogenic potential. Since etretinate and acitretin are remetabolized into long-lived compounds, isotretinoin may be used to treat women of childbearing years with generalized pustular psoriasis. We report a case of generalized pustular psoriasis in a 28-year-old woman whose condition was improved with isotretinoin. Clinical improvement was noted with the administration of isotretinoin at the dose of 30mg/day.

A Case of Multiple Keratoacanthoma with Intralesional 5-FU and Etretinate Therapy / 대한피부과학회지

Keratoacanthoma is a benign epithelial growth tumor arising from the hair follicle predominantly in elderly patients on sun-exposed skin. Intralesional therapy with 5-fluorouracil is a safe treatment and may be the treatment of choice for multiple keratoacanthoma and solitary lesion which are difficult to remove surgically. We have experienced a case of multiple keratoacanthoma which involved the right nasolabial fold and the left ear. We treated them with oral etretinate and intralesional 5-fluorouracil(5-FU), therefore minimized the scar of the lesion and the possibility of recurrence.

Thost-Unna: presentación de un caso y revisión del tema / Thost-Unna: case presentation and review

Se presenta el caso de una paciente de 26 años de edad que presenta una queratodermia palmoplantar. Aunque las imágenes histopatológicas son comunes a otras queratodermias, las características clínicas y genéticas permiten llegar al diagnóstico de enfermedad de Thost-Unna, considerada por el International Working Group una histodisplasia. La frecuencia de aparición es indeterminada en nuestro país

Lipoidoproteinosis: presentación de un caso y revisión del tema / Lipoid proteinosis: report of a case and revision of the topic

Lipoidoproteinosis o enfermedad de Urbach Wiethe es una rara afección que se hereda de manera autosómica recesiva, que se caracteriza por el depósito progresivo de material de tipo hialino en piel, mucosas y otros órganos internos. Generalmente comienza en la infancia con disfonía. Presentamos el caso de un varón de 11 años portador de lipoidoproteinosis que muestra las características clínicas e histológicas típicas de la enfermedad

Eritroqueratodermia simétrica progresiva: síndrome de Gottron / Symmetric progresive erythrokeratoderma: Gottron syndrome

La eritroqueratodermia simétrica progresiva (Gottron) es una dermatosis infrecuente, autosómica dominante. Se caracteriza por placas eritematosas e hipertqueratósicas de aspecto psoriasiforme, simétricas, que afectan extremidades y nalgas, con un carácter fijo muy típico. No afecta la vida de los pacientes, quienes incluso pueden experimentar la regresión espontánea. Presentamos una niña de cinco años con eritroqueratodermia simétrica progresiva a partir de los 20 días de vida. Las lesiones eritematoescamosas afectan cara, miembros, glúteos, axilas, manos y pies. Se asocia distrofia ungueal y descamación del cuero cabelludo. La respuesta al tratamiento con etretinato ha sido inicialmente favorable

El uso de acitretin en el tratamiento de la eritrodermia ictiosiforme congénita ampollar / Acitretin therapy for congenital bullous ichthyosiform erythroderma

La eritrodermia ictiosiforme congénita ampollar constituye una forma rara de ictiosis de carácter autosómico dominante, que presenta eritema generalizado y ampollas desde el nacimiento, que posteriormente son reemplazadas por escamas gruesas, oscuras y adherentes, predominando en flexuras. El tratamiento de esta condición puede resultar muy difícil. Si bien los retinoides sistémicos constituyen el tratamiento de elección para los trastornos de la queratinización, las dosis elevadas pueden desencadenar fragilidad cutánea y formación de ampollas. Se discute el uso de acitretin y los efectos secundarios en esta forma de ictiosis

Pitiriasis rubra pilaris asociada a artritis reumatoidea. Presentación de 1 caso / Pityriasis rubra pilaris associated with rheumatoid arthritis: a case report

Se presentó 1 caso de pitiriasis rubra pilaris eritrodérmica de 10 años de evolución en 1 paciente de 74 años de edad. Se observó que la enfermedad cutánea se asoció con artritis reumatoidea lo que hizo pensar a los autores en el posible papel etiológico de los factores inmunológicos en la pitiriasis rubra pilaris. Durante su evolución también se presentó un carcinoma papilar de vejiga, queratosis seborreicas múltiples, nódulo laríngeo benigno, carcinomas basales y un queratoacantoma. Se impuso tratamiento con methotrexate, pero no resultó satisfactorio para su PRP. Se obtuvo una buena respuesta con el etretinato

Harlequin icthyosis [HI]

In this text we present two infants born with Harlequin Icthyosis [HI]. The first one died few days after delivery. The second one was successfully treated with Tigason [Etretinate] 1mg/kg/day. There was improvement which began after 10 days from start of treatment and the infant was maintained on treatment till age of 3.5 months, when she expired because of chest infection

A Case of Darier's Disease Improved with Oral Etretinate and Isotretinoin, and CO2 Laser Vaporization / 대한피부과학회지

We report a case of Darier's disease with typical cutaneous and nail lesions in a 50-year-old male patient. He has brownish hyperkeratotic papules on the ant.chest, back, both axilla and groins and verrucous surfaced hypertrophic plaques on the scalp, forehead and both postauricular areas, which were accompanied by a severe itching sensation and malodor. Also he has finger nail lesions such as subungual hyperkeratotic papules, distal wedge shaped notching and multiple longitudinal red lines on the nail plates. Histopathologic features taken from the inguinal area revealed hyperkeratosis, suprabasal clefts and lacuna, protruded villi lined with one layer of basal cells into the cavity and dyskeratotic cells such as corps ronds in the epidermis. Hyperkeratotic papules on the ant.chest, back, both axilla and groins disappeared after systemic treatment withe a low dose of oral etretinate but hypertrophic plaques on the scalp and both postauricular areas still remained clinically and histopathologically. We then the treated postauricular hypertrophic plaques with CO2 laser vaporization and severe malodor with oral isotretinoin and it resulted in a marked improvement.

The Efficacy and Sagety of Re-PUVA Combination Therapy for Psoriasis in Comparison with Retinoid and PUVA Monotherapy / 대한피부과학회지

BACKGROUND: Although retinoid and PUVA monotherapy is effective for psoriasis, both treatment regimens have some limitations because of the risk of long-term toxicity. OBJECTIVE: We performed Re-PUVA combination therapy to evaluate the clinical efficacy and safety in comparison with retinoid and PUVA monotherapy. METHODS: Seventy-eight psoriasis patients received Re-PUVA combination therapy as well as conventional retinoid and PUVA monotherapy. Pretreatment with etretinate for 1 week was followed by PUVA therapy in combination with etretinate. RESULTS: 1. Among patients who received Re-PUVA combination therapy, clearing was shown in 77.3% and improvement in 13.6%, which were higher than retinoid, 27.8%/61.1%, and PUVA monotherapy, 65.8%/21.0%.2. In patients who reached grade 4 with Re-PUVA therapy, the duration and total dose of etretinate were 56.4 days and 1616mg. These were significantly lower than retinoid monotherapy, 107.5 days and 2577.5mg.3. In patients who reached grade 4 with Re-PUVA therapy, the number and total dose of UVA were 13.3 and 82.8 J/cm2, which showed no difference compared with those of PUVA monotherapy, 16.2 and 120.2 J/cm2. However, excluding the cases who did not respond to previous monotherapy, the number and total dose of UVA in Re-PUVA were 10.4 and 49.0J/cm2, which were significantly lower than those of PUVA4. The side effects of Re-PUVA were reported in 54.5%, which were similar to PUVA monotherapy, 52.6%, and lower than retinoid monotherapy, 83.3%. There were some side effects of retinoid, such as cheilitis, dry mouth, elevation of sGOT/sGPT and triglycerides, and side effects of PUVA, such as itching, erythema, anorexia, nausea and vomiting. CONCLUSION: Our study suggests that Re-PUVA combination therapy is more effective and tolerable than retinoid and PUVA monotherapy in the treatment of psoriasis, especially in severe and recalcitrant cases.

Psoríase e pseudo-hipoparatireoidismo / Psoriasis and pseudohypoparathyroidism

Relato de caso clínico de paciente do sexo masculino, de 24 anos de idade, com psoríase pustulosa havia dois meses. Apresentava antecedentes de crises convulsivas e retardo do desenvolvimento neuropsicomotor. Ao exame físico o sinal de Trousseau foi positivo, e a dosagem de cálcio no soro estava acentuadamente diminuída. O diagnóstico foi de psoríase pustulosa generalisada associada a pseudo-hipoparatireoidismo. Inicialmente o paciente foi tratado com etretinato, sem controle do quadro; porém, com detecçäo da hipocalcemia e a reposiçäo do cálcio, houve melhora acentuada das lesöes cutâneas. A remissäo completa foi obtida com a correçäo da hipocalcemia. Esses resultados indicam que a hipocalcemia estava diretamente relacionada com a manutençäo das lesöes de psoríase pustulosa generalizada e pseudo-hipoparatireoidismo

Etretinate in the management of harlequin siblings.

Harlequin fetus is the severe form of congenital ichthyosis. There are very few reports of babies with this condition surviving the first few weeks of life. We treated two siblings who lived for 22 months and six weeks respectively. We started treatment with oral etretinate every early in their lives and achieved good improvement in the skin condition of these babies.

A Case of Confluent and Reticulated Papillomatosis: Treatment with Etretinate / 대한피부과학회지

Confluent and reticulated papillomatosis(CRP) is a rare dermatosis. The lesions are hyperkeratotic, verrucous papules and plaques, resulting in a confluence of lesions centrally and a reticulated pattern at the periphery. It is more common in women and during puberty. The histopathological findings show papillomatosis, hyperkeratosis and a sparse superficial perivascular lymphocytic infiltrate. We report a case of CRP that responded to oral etretinate in an 18-year-old man.

A Case of Generalized Pustular Psoriasis / 대한피부과학회지

We present a case of generalized pustular psoriasis in a 47-year-old female who was treated with cyclosporine and methotrexate. She developed generalized pustular eruptions after irregular therapy of steroid and etretinate for severe psoriasis. Treatment with cyclosporine and a steroid supplement produced much improvement, but during the tapering of cyclosporine there was aggravation with pusutules and fever. She was placed on methotrexate therapy. The pustules and erythema began to remit, and eventual clearing of the skin lesions was achieved in 4 months after introduction of methotrexate.