Therapeutic Hotline: Topical glycopyrrolate: a successful treatment for craniofacial hyperhidrosis and eccrine hidrocystomas.

Eccrine hidrocystoma is a benign tumor derived from eccrine sudoriparous glands. Most eccrine hidrocystomas are solitary and asymptomatic lesions. Multiple hidrocystomas are unusual and have been associated with Graves' disease, Parkinson's disease, and idiopathic craniofacial hyperhidrosis. This report describes the successful treatment of multiple center facial eccrine hidrocystomas associated with craniofacial hyperhidrosis with 0.5% glycopyrrolate aqueous solution applied locally for 1 week. The present authors obtained a very significant improvement without leaving any trace of clinical examination. Multiple eccrine hidrocystomas are a rare condition and, to date, no effective treatment has been reported. Topical glycopyrrolate is a very good first-line treatment option.

LEOPARD syndrome: what are café noir spots?

Lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retarded growth, and deafness syndrome (multiple lentigines syndrome) is most often characterized by multiple lentigines and cardiac conduction defects. Café noir spot is a term proposed, by analogy to café au lait spots, for the larger and darkly pigmented patches that are frequently observed in patients with this syndrome. Although presumed by some authors to represent lentigines, the histologic features of café noir spots have not been well documented in the literature. Only two previous cases have been reported in which a biopsy of the café noir spots than melanocytic nevi. We describe the histologic characteristics of seven café noir spots in six patients with lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retarded growth, and deafness syndrome. Three lesions represented melanocytic nevi (one with dysplastic features), and four were compatible with lentigo simplex. These findings help our understanding of the histologic spectrum of pigmented lesions in lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retarded growth, and deafness syndrome.

[Angiosarcoma in chronic lymphedema]. / Angiosarcoma sobre linfedema crónico.

Angiosarcoma that develops on a limb with chronic lymphedema is called Stewart-Treves syndrome. This typically appears as a complication of a long course lymphedema located on the arm, after mastectomy and/or radiotherapy due to breast cancer. There are cases of Stewart-Treves syndrome in chronic lymphedema in the upper limb contralateral to the breast treated for cancer and in chronic lymphedema of the leg. We present two cases of this syndrome. The first corresponds to a typical syndrome of Stewart-Treves in an 83-year-old woman who was diagnosed of angiosarcoma in a chronic lymphedema territory secondary to mastectomy and radiotherapy due to breast cancer. The second case is much rarer, since it is a case of diffuse angiosarcoma of the leg in a 42-year-old man with a history of lymphedema. Due to the aggressive nature of this syndrome, knowledge and research on its treatment are necessary.

Angiosarcoma sobre linfedema crónico / Angiosarcoma in chronic lymphedema

El angiosarcoma que se desarrolla sobre una extremidad con linfedema crónico se denomina síndrome de Stewart-Treves. Éste aparece típicamente como una complicación de un linfedema de larga evolución localizado en el brazo, tras mastectomía y/o radioterapia por un cáncer de mama. Existen casos de síndrome de Stewart-Treves sobre linfedema crónico en la extremidad superior contralateral al cáncer de mama tratado y sobre linfedema crónico de pierna. Presentamos dos casos de este síndrome. El primero corresponde a un típico síndrome de Stewart-Treves en una mujer de 83 años, que fue diagnosticada de angiosarcoma en el territorio de un linfedema crónico secundario a mastectomía y radioterapia por un cáncer de mama. El segundo caso es mucho más raro, ya que se trata de un caso de angiosarcoma difuso de pierna, en un hombre de 42 años e historia de linfedema. La naturaleza agresiva de este síndrome precisa de su conocimiento e investigación de tratamientos para prevenirlo

Angiosarcoma that develops on a limb with chronic lymphedema is called Stewart-Treves syndrome. This typically appears as a complication of a long course lymphedema located on the arm, after mastectomy and/or radiotherapy due to breast cancer. There are cases of Stewart-Treves syndrome in chronic lymphedema in the upper limb contralateral to the breast treated for cancer and in chronic lymphedema of the leg. We present two cases of this syndrome. The first corresponds to a typical syndrome of Stewart-Treves in an 83-year-old woman who was diagnosed of angiosarcoma in a chronic lymphedema territory secondary to mastectomy and radiotherapy due to breast cancer. The second case is much rarer, since it is a case of diffuse angiosarcoma of the leg in a 42-year-old man with a history of lymphedema. Due to the aggressive nature of this syndrome, knowledge and research on its treatment are necessary

[Diffuse cutaneous reticulohistiocytosis]. / Reticulohistiocitosis cutánea difusa.

The reticulohistiocytoses make up a heterogeneous group of diseases whose origin lies in an accumulation of cells of histiocytic lineage in different tissues and primarily in the skin. Three main clinical forms have been described (multicentric, solitary, diffuse cutaneous), which present with identical histological, ultrastructural and immunohistochemical characteristics. We present a case of diffuse cutaneous reticulohistiocytosis, which is the least common clinical pattern in the spectrum of this disease.

Reticulohistiocitosis cutánea difusa / Diffuse cutaneous reticulohistiocytosis

Las reticulohistiocitosis constituyen un grupo heterogéneo de enfermedades que tienen su origen en la acumulación de células de estirpe histiocitaria en diferentes tejidos y fundamentalmente en la piel. Se han descrito tres formas clínicas principales (multicéntrica, solitaria, cutánea difusa) que presentan idénticas características histológicas, ultraestructurales e inmunohistoquímicas. Presentamos un caso de reticulohistiocitosis cutánea difusa que constituye el patrón clínico menos común dentro del espectro de esta enfermedad

The reticulohistiocytoses make up a heterogeneous group of diseases whose origin lies in an accumulation of cells of histiocytic lineage in different tissues and primarily in the skin. Three main clinical forms have been described (multicentric, solitary, diffuse cutaneous), which present with identical histological, ultrastructural and immunohistochemical characteristics. We present a case of diffuse cutaneous reticulohistiocytosis, which is the least common clinical pattern in the spectrum of this disease

Hidradenitis ecrina neutrofílica secundaria a tioguanina en paciente neutropénico / Neutrophilic eccrine hidradenitis secondary to thioguanine in a neutropenic patient

La hidradenitis ecrina neutrofílica (HEN) es una dermatosis inflamatoria poco frecuente y autolimitada, caracterizada por un infiltrado neutrofílico alrededor de las glándulas ecrinas y que se presenta clínicamente con diferentes tipos de lesiones. La HEN ocurre con más frecuencia en pacientes tras recibir quimioterapia por neoplasias hematológicas. Se presenta un caso de HEN en un paciente neutropénico varón de 70 años que recibió tioguanina por una leucemia aguda mieloide. Las placas eritematosas desaparecieron en 3-4 semanas. Los hallazgos histológicos fueron compatibles con HEN. Los cultivos cutáneos excluyeron causas infecciosas

Neutrophilic eccrine hidradenitis (NEH) is an infrequent, self-limited inflammatory dermatosis characterized by a neutrophilic infiltrate around the eccrine glands. Clinically, it presents with different types of lesions. NEH occurs most frequently in patients who have undergone chemotherapy for hematologic neoplasms. We present a case of NEH in a 70-year-old neutropenic male who received thioguanine for acute myeloid leukemia. The erythematous plaques disappeared in 3-4 weeks. The histological findings were compatible with NEH. Skin cultures ruled out infectious causes

[Neutrophilic eccrine hidradenitis secondary to thioguanine in a neutropenic patient]. / Hidradenitis ecrina neutrofílica secundaria a tioguanina en paciente neutropénico.

Neutrophilic eccrine hidradenitis (NEH) is an infrequent, self-limited inflammatory dermatosis characterized by a neutrophilic infiltrate around the eccrine glands. Clinically, it presents with different types of lesions. NEH occurs most frequently in patients who have undergone chemotherapy for hematologic neoplasms. We present a case of NEH in a 70-year-old neutropenic male who received thioguanine for acute myeloid leukemia. The erythematous plaques disappeared in 3-4 weeks. The histological findings were compatible with NEH. Skin cultures ruled out infectious causes.