Alitretinoin as a Treatment Modality for Ichthyosis in Women of Childbearing Age: A Case Series and Review of the Literature.

BACKGROUND: Acitretin, a synthetic vitamin A derivative, is the most studied and widely used oral retinoid for ichthyoses. Its major disadvantage is the need for contraceptive measures during 3 years after discontinuation. An alternative is needed for women of childbearing age. With alitretinoin, another retinoid, pregnancy is considered safe 1 month after discontinuation. OBJECTIVES: The aim of this study was to provide evidence for alitretinoin as an alternative for acitretin for ichthyosis in women of childbearing age. Our experience is shared in a case series combined with an overview of the current literature. METHODS: Nine women of childbearing age (19-31 years, median 21) with different subtypes of ichthyosis (autosomal recessive congenital ichthyosis, (superficial) epidermolytic ichthyosis, erythrokeratoderma variabilis, and epidermolytic epidermal nevi, a mosaic form of epidermolytic ichthyosis) were included and treated with 30 mg alitretinoin during 2-28 months. Severity was measured by Ichthyosis Area Severity Index (IASI) and Investigator Global Assessment (IGA). A literature search in Pubmed using the Mesh terms "alitretinoin," "skin diseases, genetic" and "ichthyosis" was performed. RESULTS: Significant reduction in the mean scores of IGA, IASI-erythema, IASI-scaling, and IASI-total was seen. Seven patients are still being treated, 1 patient stopped to become pregnant, 1 patient discontinued due to financial reasons. Observed side effects were reversible headache (n = 6), asteatotic eczema (n = 1), "not feeling well" temporarily (n = 1), and easier blistering of the feet (n = 1). The literature search resulted in six case reports and case series about alitretinoin in ichthyosis and ichthyosis syndromes with in total 29 patients. The vast majority of articles (21/29) reported significant improvement or even complete remission of skin symptoms. However, validated outcome measures to support these results were lacking. Side effects (n = 16) were relatively mild, except for benign intracranial hypertension (n = 1) and autoimmune hypothyroidism (n = 1). CONCLUSION: Our study shows, with validated outcome measures, that alitretinoin is effective to mitigate the symptoms of ichthyosis in women of childbearing age and a suitable alternative to acitretin.

Scabies in a 14-year-old girl with superficial epidermolytic ichthyosis.

A 14-year-old girl who reported generalized scaling and hyperkeratosis since age 1 year presented with severe pruritus of several months' duration. Scabies mites were detected, and molecular genetic analysis subsequently revealed a rare pathogenic variant in the keratin 2 (KRT2) gene, confirming a diagnosis of superficial epidermolytic ichthyosis. Treatment with oral ivermectin led to complete remission of symptoms. Disorders of keratinization can mimic clinical signs of scabies, leading to a delay in diagnosis.

Bilateral Systematised Epidermolytic Epidermal Nevus: A case report.

Verrucous epidermal nevi (VEN) are benign congenital hamartomas consisting of keratinocytes. Histological examination mostly exhibits hyperkeratosis, acanthosis, papillomatosis and, rarely, the features of epidermolytic hyperkeratosis (EHK). We report a case of a 6-year-old boy who presented at Aga Khan University Hospital, Karachi, Pakistan with bilaterally symmetrical linear epidermal nevi following Blaschko's lines and showing epidermolytic hyperkeratosis on histology. The patient was treated with topical keratolytics and emolients which led to considerable improvement. To the best of the authors' knowledge, this is the first report of VEN from Pakistan.

Eritrodermia ictiosiforme congénita ampollosa: a propósito de un caso / Congenital bullous ichthyosiform erythroderma

La eritrodermia ictiosiforme congénita ampollosa es una entidad poco frecuente, con herencia autosómica dominante, pero un 50% se presenta por mutaciones de novo; se caracteriza en su fase inicial por eritrodermia y aparición espontánea de ampollas. Posteriormente las ampollas disminuyen en frecuencia y aparece una descamación que compromete toda la superficie corporal. No suele haber afectación palmoplantar ni de las mucosas. Su tratamiento de primera línea son los retinoides sistémicos junto a los cuidados de la piel

Congenital bullous ichthyosiform erythroderma is a rare entity, with autosomal dominant inheritance, but in 50% of cases it is presented by de novo mutations; is characterized in its initial phase by erythroderma and spontaneous appearance of blisters. Subsequently the blisters decrease in frequency and appears a scaling that compromises the entire body surface. There is usually no palmoplantar or mucosal involvement. Its first-line treatment is systemic retinoids along with skin care

Uña verde causada por onicomicosis coinfectada por Pseudomonas aeruginosa / Green Nail Caused by Onychomycosis Coinfected With Pseudomonas aeruginosa

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Placa hiperqueratósica asintomática en glande en un varón de mediana edad / Asymptomatic Hyperkeratotic Plaque on the Glans in a Middle-Aged Man

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Eritema difuso e hiperqueratosis acral en un lactante / Diffuse Erythema and Acral Hyperkeratosis in a Newborn

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Long-term safety and efficacy of continuous acitretin monotherapy for three children with different severe hyperkeratotic disorders in China.

Long-term systemic treatment with acitretin for severe hyperkeratotic disorders is needed to maintain quality of life of afflicted patients, but treatment has been limited owing to its potential side-effects including skeletal malformations, particularly for children during their growth and development. A retrospective investigation was conducted with three children afflicted with a severe hyperkeratotic disorder, namely Darier's disease, bullous ichthyosiform erythroderma or lamellar ichthyosis, who were continuously maintained on 0.2-0.3 mg/kg per day acitretin for more than 12 years after an initial period at a larger acitretin dose to bring each disease under control. The patients had good responses to acitretin treatment, which was assessed for safety, skeletal abnormalities, growth retardation and other potential side-effects. Acitretin monotherapy was an effective treatment for these children, and maintenance doses were well tolerated with no skeletal or other observable side-effects during the course of the study.

Otitis externa necrosante causada por Scopulariopsis brevicaulisen un paciente sin factores predisponentes / Necrotising otitis externa due to Scopulariopsis brevicaulis in a patient without predisposing factors

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Paraqueratosis granular, una rara entidad. A propósito de un caso / Granular parakeratosis: a rare entity. A case report

La paraqueratosis granular axilar es una entidad poco frecuente debida a una alteración del proceso de queratinización, lo que produce un engrosamiento de la epidermis a expensas de la capa córnea. La etiología es desconocida, implicándose, entre otros, a agentes irritantes. Presentamos un caso de paraqueratosis granular axilar en una mujer de 31 años, asociada a un posible factor irritante, el desodorante, localizada en ambas axilas, sin mejoría ante el tratamiento farmacológico (corticoides y antifúngico) y con remisión completa tras la retirada del mismo (AU)

Granular parakeratosis is a rare entity that results from a disorder of keratinization, producing an epidermal thickening of the stratum corneum. The etiology is unknown although some factors, such as irritating agents, have been implicated. We present a case of granular parakeratosis associated with an irritating agent (a deodorant) in both axillae of a 31-year-old woman. She did not respond to therapy with corticosteroid and antifungal agents but the GP resolved spontaneously when use of the deodorant was stopped (AU)

Treatment of nail psoriasis with Pulse Dye Laser plus calcipotriol betametasona gel vs. Nd:YAG plus calcipotriol betamethasone gel: an intrapatient left-to-right controlled study / Tratamiento de psoriasis ungueal con Pulse dye laser frente a Nd: YAG, en asociación con gel de betametasona: un estudio con control intrapaciente izquierda-derecha

BACKGROUND: Treatment of nail psoriasis remains a challenging and often disappointing situation. OBJECTIVE: To compare the efficacy, adverse reactions and tolerability of treatment of nail psoriasis with PDL vs. Nd:YAG, in association with betametasona calcipotriol gel. METHODS: An open, prospective intrapatient left-to-right study was designed. The right hand of each patient received treatment with PDL and the left hand with Nd:YAG. Betamethasone calcipotriol gel was applied once a day during the first week after each laser session. A total of four sessions were administered. RESULTS: The clinical efficacy was evaluated according to the NAPSI score. All patients showed improvement in nail bed and nail matrix psoriasis. The global NAPSI mean declined in 15.46 (p < 0.000). There was neither statistical difference between the reduction in nail bed and matrix NAPSI nor in the treatment with PDL vs. Nd:YAG. The administration of Nd:YAG was more painful. No serious adverse effects were documented. Limitations. No random assignment and the small number of patients. CONCLUSIONS: PDL and Nd:YAG have proven to be an effective treatment for nail psoriasis with no serious adverse effect. No statistically significant difference was found between the two treatments

ANTECEDENTES: El tratamiento de la psoriasis ungueal es una situación de difícil manejo y a menudo decepcionante para el dermatólogo. OBJETIVO: Comparar la eficacia, las reacciones adversas y la tolerabilidad del tratamiento de la psoriasis ungueal con PDL vs. Nd: YAG en asociación con gel de betametasona calcipotriol. MÉTODOS: Estudio prospectivo abierto con control intrapaciente izquierda-derecha. La mano derecha de cada paciente recibió tratamiento con PDL y la mano izquierda con Nd: YAG. Se aplicó gel de betametasona calcipotriol una vez al día durante la primera semana después de cada sesión de láser en las 2 manos. Se administraron un total de 4 sesiones. RESULTADOS: La eficacia clínica se evaluó de acuerdo con la escala NAPSI. Todos los pacientes mostraron una mejoría en las lesiones del lecho y de la matriz ungueal. La media global del NAPSI disminuyó en 15,46 (p < 0,000). No hubo diferencia significativa entre la mejoría de las lesiones del lecho y la matriz ni en el tratamiento con el PDL vs. Nd: YAG. La administración de Nd: YAG fue más dolorosa. No se documentaron efectos adversos graves. Limitaciones. Falta de asignación aleatoria y muestra pequeña. CONCLUSIONES: PDL y Nd: YAG han demostrado ser tratamientos eficaces para la psoriasis ungueal sin documentarse efectos adversos graves. No se encontró diferencia estadística significativa entre los 2 tratamientos

Poroqueratosis actínica superficial diseminada / Disseminated superficial actinic porokeratosis

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Manos de mecánico / Mechanic's hands

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Lesiones hiperqueratósicas y prurito en paciente inmunodeprimida / Hyperkeratotic lesions and pruritus in an immunosuppressed patient

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Placas eritematodescamativas en paciente con síndrome de Down / Scaly Erythematous Patches in a Patient With Down Syndrome

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