Penfigoide buloso nodular tratado exitosamente con fototerapia UVB banda angosta / Pemphigoid nodularis successfully treated with narrow band UVB phototherapy

El Penfigoide nodular es una variante clínica poco frecuente de penfigoide buloso. Corresponde a una dermatosis ampollar subepidérmica, crónica, autoinmune, caracterizada por auto anticuerpos contra antígenos específicos de hemidesmosomas en la unión dermo-epidérmica. Su incidencia es desconocida. La etiopatogenia aún no es entendida del todo. Se presenta clínicamente como una superposición de características de pénfigo buloso y prurigo nodular. El diagnóstico se basa en hallazgos clínicos e inmunopatológicos. La histopatología con inmunofluorescencia directa es el gold standard para el diagnóstico. El manejo es difícil, tiene mala respuesta a corticoides potentes locales, siendo necesario el uso de corticoides sistémicos y diferentes inmunosupresores solos o combinados junto a antihistamínicos para el manejo de prurito intenso. Se presenta un caso de pénfigo nodular, donde destaca su buena respuesta a terapia combinada con metotrexato y luz UVB de banda angosta.

Pemphigoid Nodularis is a rare clinical variant of bullous pemphigoid. It is considered an autoimmune, chronic, subepidermal blistering dermatosis, characterized by antibodies against hemidesmosome-specific antigens at the dermo-epidermal junction. Its incidence is unknown and its etiopathogenetic not fully understood. Clinically, it presents with overlapping features of bullous pemphigoid and prurigo nodularis. The diagnosis is based on clinical and immunopathological findings, being the histopathological study with immunofluorescence the gold standard. The management is difficult; since it has a poor response to local potent corticosteroids, requiring the use of systemic corticosteroids and different immunosuppressants alone or combined with antihistamines for the intense pruritus. We present a case of nodularis pemphigoid, highlighting the good response to the combination of methotrexate and phototherapy with narrow band UVB.

Extirpación quirúrgica con cierre primario y uso de Isotretinoína en acné queloídeo de la nuca: serie de 6 casos clínicos / Acne keloidalis nuchae

El Acné queloídeo de la nuca se presenta principalmente en hombres jóvenes de raza negra; su etiología es desconocida, pero se piensa que sería multifactorial. El cuadro clínico corresponde a pápulas y pústulas que tienden a confluir en placas de aspecto queloídeo y que se observan como alopecia cicatricial, produciendo gran repercusión tanto física como psicológica en el paciente. Histológicamente se distingue una etapa temprana y una avanzada. No existe tratamiento curativo para este cuadro, pero se ha experimentado con múltiples opciones de tratamiento. En este trabajo se exponen los resultados de nuestra experiencia terapéutica con cirugía excisional asociada a cierre primario y posterior uso de Isotretinoína oral.

The Acne keloidalis nuchae occurs mainly in young black men; its etiology is unknown but is thought to be multifactorial. Clinically it presents as papules and pustules which tend to coalesce in keloid-like plaques and they are observed as scarring alopecia, producing great impact both physically and psychologically in the patient. An early and an advanced stage can be distinguished with histological techniques. There is no cure for this pathology, even though multiple treatments have been tried. In this paper, we expose the results of our therapeutic experience with excisional surgery associated with primary closure and subsequent use of oral Isotretinoin.

[Laugier-Hunziker syndrome in a patient with Sjögren’s syndrome: Report of one case]. / Posible asociación autoinmune entre síndrome de Laugier-Hunziker y síndrome de Sjögren: reporte de un caso y revisión de la literatura.

Laugier-Hunziker syndrome is a rare benign idiopathic condition characterized by acquired macular pigmentation of lips and buccal mucosa, often accompanied with melanonychia. The main concern with this condition is to rule out other differential diagnosis with systemic repercussions and similar hyperpigmentation patterns, such as Peutz-Jeghers syndrome, adrenal insufficiency and melanoma. We report a 58-year-old female with a 20-year history of Sjögren’s syndrome, presenting with melanonychia and hyperpigmentation in the buccal mucosa. She had no relevant medication history and is a non-smoker. The patient denied any other symptoms. The histopathology confirmed the diagnosis of Laugier-Hunziker syndrome.

Posible asociación autoinmune entre síndrome de Laugier-Hunziker y síndrome de Sjögren: reporte de un caso y revisión de la literatura / Laugier-Hunziker syndrome in a patient with Sjögren’s syndrome: report of one case

Laugier-Hunziker syndrome is a rare benign idiopathic condition characterized by acquired macular pigmentation of lips and buccal mucosa, often accompanied with melanonychia. The main concern with this condition is to rule out other differential diagnosis with systemic repercussions and similar hyperpigmentation patterns, such as Peutz-Jeghers syndrome, adrenal insufficiency and melanoma. We report a 58-year-old female with a 20-year history of Sjögren’s syndrome, presenting with melanonychia and hyperpigmentation in the buccal mucosa. She had no relevant medication history and is a non-smoker. The patient denied any other symptoms. The histopathology confirmed the diagnosis of Laugier-Hunziker syndrome.