Jorge Angulo

Primary cutaneous cribriform apocrine carcinoma: A clinicopathologic and immunohistochemical study of 26 cases of an under-recognized cutaneous adnexal neoplasm

BACKGROUND Cribriform carcinoma is the histopathologic variant of cutaneous apocrine carcinoma characterized by interconnected solid aggregations of neoplastic cells that are punctuated by small round spaces. OBJECTIVE To describe the histopathologic and immunohistochemical characteristics of this under-recognized cutaneous adnexal neoplasm. METHODS Twenty-six cases of primary cutaneous cribriform apocrine carcinoma were clinically, histopathologically, and immunohistochemically studied. RESULTS Seven neoplasms arose in males and 19 in females. The median age of the patients was 47.8 years. The lower and upper limbs were the most frequent sites. Histopathologically, the neoplasms consisted of well-circumscribed dermal nodules composed of multiple, interconnected, solid aggregations of basophilic epithelial cells that were punctuated by small round spaces. Immunohistochemically, the neoplastic cells expressed immunoreactivity for MNF116, AE1/AE3, Cam 5.2, cytokeratin 7, carcinoembryonic antigen, and epithelial membrane antigen. LIMITATIONS All specimens came from dermatopathology laboratories, and some inherent selection bias may exist. CONCLUSIONS Primary cutaneous cribriform apocrine carcinoma is a distinctive but little-known variant of cutaneous apocrine carcinoma.

Generalized livedo reticularis associated with monoclonal cryoglobulinemia and multiple myeloma

Abstract:  Cryoglobulins are detected in a wide variety of diseases, including malignancies, infections and systemic autoimmune diseases. Classically, monoclonal cryoglobulinemia is associated with hematologic malignancies, whereas mixed cryoglobulinemias are reported in association with hepatitis C virus infections or autoimmune diseases. We present a patient with generalized livedo reticularis as the first manifestation of monoclonal cryoglobulinemia and multiple myeloma. Histopathology demonstrated that nearly all small blood vessels of the upper and deep dermis, as well as the capillaries of the fat lobule, were filled with homogeneous, eosinophilic material that corresponded to monoclonal cryoglobulin deposits within the vascular lumina. Our case of livedo reticularis associated with monoclonal cryoglobulinemia and multiple myeloma was exceptional, because the mottled cyanotic discoloration of the skin with a reticular pattern was generalized, covering most of the skin surface. We have not found previous report of similar cases. Therefore, we recommend that dermatologists be made aware of the significance of this diagnosis.

Late‐onset Ito’s nevus: An uncommon acquired dermal melanocytosis

Dermal melanocytoses comprise a variety of congenital and acquired conditions characterized by a sparse population of intradermal dendritic, variably pigmented, spindle‐shaped melanocytes. While Mongolian spot, Ota’s and Ito’s nevi are usually present at birth or appear around puberty; acquired dermal melanocytoses that appear in adult life are extremely rare. They include the facial lesions of acquired bilateral nevus of Ota‐like macules, also named Hori’s nevus, and the acquired unilateral nevus of Ota, also known as Sun’s nevus. Uncommon extrafacial examples of acquired dermal melanocytoses include lesions involving upper extremities, wrist, back, lower extremities and dorsal aspects of the hands and feet. They are more prevalent among Asian women. In general, dermal melanocytoses are rare lesions in Caucasian patients and acquired variants are exceedingly uncommon. We report a rare example of acquired Ito’s nevus that appeared in a Caucasian elderly woman and review the literature about acquired dermal melanocytoses.

Plaque-like osteoma cutis with transepidermal elimination.

Osteoma cutis is a rare lesion that consists of the presence of bone tissue within the dermis and/or hypodermis. It may be classified as primary osteoma cutis, when bone tissue develops in the skin without any pre‐existing lesion and secondary osteoma cutis, which is more frequent and occurs when osseous tissue develops on a pre‐existing lesion. We present a case of primary plaque‐like osteoma cutis involving the scalp, left forehead and left cheek, which appeared in an adult male. Histopathological study showed several islands of mature osseous tissue involving the full thickness of the dermis. In some areas, there was also transepidermal elimination of bone spicules. We review the literature about previously reported similar cases.

Acroqueratodermia siríngea acuagénica. Presentación de dos casos

Aquagenic syringeal acrokeratoderma is an infrequently described acquired, transitory keratoderma that is exacerbated when the palms and/or soles are immersed in water. It manifests as whitish or yellowish flattened, translucent papules and plaques, located in areas of pressure or trauma on the palms and/or soles. Involvement is usually bilateral. The histologic features are non-specific, showing a slight dilation of the intraepidermal portion of the eccrine sweat duct. The process tends to remit spontaneously. We present the case of two women, aged 20 and 21.

Leukemia cutis presenting as localized cutaneous hyperpigmentation

The usual clinical presentations of leukemia cutis include solitary infiltrated erythematous or violaceous plaques or nodules and multiple localized or generalized papules. On the other hand, cutaneous hyperpigmentation is a frequent finding in patients with malignancies, most of the cases because of chemotherapy or other drugs that the patient is taking. We present a case of cutaneous hyperpigmentation as the presenting sign of leukemia cutis. A 61‐year‐old male presented with cutaneous hyperpigmentation, which had appeared during the last chemotherapy cycle for treatment for biphenotypic leukemia. Cutaneous lesions consisted of bluish to brownish irregular well‐defined discoloration of the skin involving the upper part of the trunk and the temporal regions of the forehead. The patient was asymptomatic and the skin was not infiltrated at all. However, histopathologic study showed nodular infiltrates involving the full‐thickness of the dermis and destroying pre‐existing adnexa. This infiltrate was composed of atypical basophilic cells with large hyperchromatic nuclei and scant cytoplasm. Immunohistochemical studies showed intense immunoexpression for CD43, CD68, CD45RO and myeloperoxidase within these cells. A diagnosis of biphenotypic leukemia cutis was established. In our review of the literature we have not found any report of cutaneous hyperpigmentation as the presenting manifestation of leukemia cutis.

Lipedematous alopecia: an uncommon clinicopathologic variant of nonscarring but permanent alopecia

A 52‐year‐old black woman presented with a 5‐year history of gradual swelling and slowed hair growth involving the vertex and both parietal regions of the scalp. Gradually, the swelling progressed to involve the entire scalp, only sparing a peripheral crown. She reported no history of trauma or medications. Slight pruritus of the involved area was the only accompanying symptom. There was no family history of a similar condition. Her past medical history included surgery for ovarian cysts, 10 years previously, and cholelithiasis.

Squamous cell apocrine hidradenoma

Apocrine hidradenoma is a benign adnexal neoplasm with apocrine differentiation. The neoplasm is composed of four different types of epithelial cells, including pale or clear cells, polygonal cells, mucinous cells and squamous cells, with variable proportions of them from case to case. In most examples of this neoplasm, clear or the polygonal cells are predominant, whereas the other types of neoplastic cells are less abundant. We report two cases of apocrine hidradenoma mostly composed of squamous cells. Histopathologic examination showed that the neoplasms were composed of both solid and cystic areas. The solid aggregations of neoplastic cells were composed of a peripheral layer of basaloid polygonal cells, whereas squamous cells forming the bulk of the aggregations. These squamous cells showed large eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. In one case, small foci of mucinous cells could also be seen in some aggregations of neoplastic cells, mostly around ductal structures. In both the cases, some of the tubular structures lined by epithelial cells showed evidence of decapitation secretion in their luminal border. The neoplastic stroma consisted of sclerotic collagen bundles when compared with adjacent normal dermis, and artefactual clefts separated the neoplasms from the surrounding tissue. The rare cases described in this report are exceptional because most of the neoplastic cells showed squamous appearance and for that reason we think that squamous cell apocrine hidradenoma is the most appropriate name for these neoplasms.

Giant benign lymphangioendothelioma

Benign lymphangioendothelioma is a rare locally infiltrative vascular neoplasm, presenting as a slow‐growing, asymptomatic, reddish‐violaceous plaque. Histopathologically, it is characterized by irregular and thin‐walled vascular spaces, lined by a single and discontinuous layer of flat endothelial cells, dissecting dermal collagen bundles. We present the case of a 75‐year‐old man with a giant benign lymphangioendothelioma, to our knowledge, the largest example described in the literature. The immunohistochemical expression of Wilms tumor 1 gene is useful in vascular lesions to differentiate malformations from proliferative endothelial lesions. In our case, the positivity for WT1 supports the neoplastic nature of this lesion.

Mastocitosis del adulto. Descripción de 9 casos clinicopatológicos

Resumen Introduccion La mastocitosis es un proceso hiperplasico caracterizado por la infiltracion de diversos organos y tejidos por mastocitos maduros. Se trata de una enfermedad que es mas frecuente en la infancia, aunque tambien se dan casos en los adultos. Existen notables diferencias entre las formas de presentacion de la mastocitosis del adulto y de la infancia, asi como en su evolucion y pronostico. Material y metodos En este trabajo describimos los hallazgos clinicopatologicos de 9 pacientes adultos con mastocitosis. Se comparan las caracteristicas clinicas, evolutivas y geneticas de la mastocitosis del adulto con la mastocitosis infantil. Resultados En contraste con la mastocitosis infantil, las lesiones cutaneas de la mastocitosis en adultos son muy monomorfas, y consisten en maculas y papulas de menos de 1 cm de diametro y coloracion pardo-rojiza. La sintomatologia es escasa y lo mas habitual suele ser un discreto prurito. El signo de Darier es con gran frecuencia negativo. Ademas, las lesiones cutaneas no tienden a la regresion espontanea, que es lo habitual en el nino. Por ultimo, la afectacion sistemica en el adulto es practicamente constante, con infiltracion mastocitaria de la medula osea en mas del 90 % de los casos y afectacion osea en mas del 50 % de los casos, mientras que en los ninos la afectacion sistemica es mas rara. De todas formas, a pesar de que exista infiltracion de organos sistemicos en la mastocitosis de adultos, no suele existir sintomatologia acompanante. La mutacion del protooncogen c-kit consistente en la sustitucion de Asp por Val en el codon 816, se encuentra casi constantemente en las mastocitosis del adulto y es menos frecuente en la mastocitosis infantil. Conclusiones Todos estos hallazgos permiten separar la mastocitosis del adulto como una entidad clinicopatologica diferente de las mastocitosis infantiles.