Eruptive syringomas associated with milia-like idiopathic calcinosis cutis.

An 11-year-old boy presented generalized eruptive syringomas (ESs) associated with multiple milia-like whitish palmar papules corresponding to dermal calcium deposits. A relationship between calcium deposits distribution to an underlying eccrine duct was noted on pathology. The observation of dermal calcium deposits and its association with generalized ESs may support a possible sweat duct origin of this uncommon and peculiar form of superficial calcinosis cutis.

Reactive Eccrine Syringofibroadenomatosis Associated With Venous Leg Ulcers: A Case Report and Literature Review.

Eccrine syringofibroadenoma (ESFA) is a rare adnexal tumor deriving from the acrosyringeal portion of the eccrine duct. Five subtypes of ESFA were described including a reactive form. Reactive ESFAs are associated with inflammatory and neoplastic dermatoses. In this article, we report the case of a 90-year-old woman presenting with 3 leg ulcers evolving for 2 years surrounded by large verrucous and eczematous lesions. Multiple skin biopsies showed anastomosing epithelial cords connected to the epidermis consistent with ESFA. We identified 8 cases of ESFA associated with chronic leg ulcers in the literature and reviewed their main clinical and histological features.

Late-onset eccrine syringofibroadenoma of the feet in a patient with hypohidrotic ectodermal dysplasia.

A 56-year-old woman with hypohidrotic ectodermal dysplasia presented with a 10-year history of persisting wart-like skin lesions on her feet. Biopsy revealed changes of eccrine syringofibroadenoma. These lesions are rare, with only nine case reports describing an association with ectodermal dysplasia of hidrotic type (Clouston and Schopf's syndrome). To our knowledge, this is the first case of eccrine syringofibroadenoma developing in the hypohidrotic/anhidrotic subtype of ectodermal dysplasia.

(Reactive) Eccrine Syringofibroadenoma with Foci of Squamous Cell Carcinoma in situ: A Case Report.

We describe a rare case of an eccrine syringofibroadenoma with a foci of squamous cell carcinoma in situ, which has to best of our knowledge been reported only twice in the English literature. An excisional biopsy of an elevated, lobular tumor of the lower leg in an 86-year-old male patient was performed. Histologic examination revealed a tumor consisting of anastomosing strands of epithelial cells originating from the epidermis, occasionally showing ductal eccrine differentiation. Foci of squamous cell carcinoma in situ were observed within the described lesion. The diagnosis of eccrine syringofibroadenoma with squamous cell carcinoma in situ was established. Eccrine syringofibroadenoma is a rare lesion, mostly considered to be a reactive process arising secondarily in association with other cutaneous diseases such as dermatoses or neoplasms, although some researchers do not exclude the possibility that it is a primary neoplasm with a potential for malignant transformation.

Eccrine mucinous nevus: clinical and histopathological description in an adult Nevus mucinoso ecrino en el adulto: descripción clínicohistológica.

Eccrine mucinous nevus is an uncommon hamartoma whose histological diagnosis is simple because of its peculiar morphological features, which include an increase in the number and/or size of the glands and eccrine ducts accompanied by abundant mucin deposits. When it presents with the typical symptom, focal hyperhidrosis, clinical suspicion is possible. El nevus mucinoso ecrino (NME) es un hamartoma escasamente reportado en la literatura cuyo diagnóstico histológico es sencillo debido a sus peculiares rasgos morfológicos, que incluye un aumento del número y/o tamaño de las glándulas ductos ecrinos acompañado de abundante depósito de mucina. Cuando presenta los hallazgos típicos, como la hiperhidrosis focal, es posible su sospecha clínica.

Apocrine adenocarcinoma in the setting of apocrine hidrocystoma of the leg.

Apocrine hidrocystoma is a benign, cystic lesion often presenting in the periorbital region. Apocrine adenocarcinoma is the rare, malignant counterpart occurring mainly in the axilla and anogenital region. There is a paucity of literature on both entities and co-occurrence has been reported in only 5 cases. We present the case of a 48-year-old man with a history of total body irradiation for chronic myelocytic leukemia, diabetes mellitus, and obesity who presented with a calf mass of two years' duration. Epidermal inclusion cyst was presumed and excisional biopsy was carried out. Pathologic analysis revealed apocrine adenocarcinoma in the setting of a precursor apocrine hidrocystoma. Our patient's unique altered immunity and the direct effects of irradiation on the local microenvironment may have resulted in his rare presentation of co-occurrence of apocrine adenocarcinoma within an existing apocrine hidrocystoma. To our knowledge, our patient is the first reported patient with this presentation in the lower extremity.

Scalp porocarcinoma and lichen planopilaris.

Porocarcinoma (PC) is a malignant neoplasm arising from the intraepidermal ductal portion of the sweat gland duct. Lichen planopilaris (LPP) is a not so rare variant of cutaneous lichen planus (LP) with a preferential involvement of hair follicles, consisting of a chronic lymphocytic inflammation, leading to cicatricial alopecia. A 42-year-old woman, recently diagnosed with HIV infection, was referred to our clinic because of an alopecic patch of 6 years' duration. In the upper region of the alopecia a 1.5cm nodule was noticed, which the patient stated had started growing soon after the appearance of the hair loss. Biopsy of the alopecia margin confirmed the diagnosis of LPP, whereas biopsy of the nodule revealed an infiltrating tumor consistent with the diagnosis of PC. We present a scalp PC emerging in a background of LPP in an HIV patient. We do not know the role, if any, HIV infection and LPP played in this particular case. Immunosuppression and HIV have been implicated in the etiology of PC. However, her HIV diagnosis was made after the appearance of the scalp nodule. We did not find any association between LPP and PC in the literature. Even though an association by chance cannot be excluded, this deserves further investigation.

Primary cutaneous cribriform apocrine carcinoma: Case report and literature review.

Primary cutaneous cribriform apocrine carcinoma is a rare but distinct variant of primary cutaneous apocrine carcinoma and it is considered a low grade malignancy. We herein present a case of primary cutaneous cribriform apocrine carcinoma at the neck of a 26-year-old female. The tumor features a relatively well-circumscribed border and multiple aggregations of mildly pleomorphic epithelial cells with large ovoid nuclei, small nucleoli and abundant eosinophilic cytoplasms. Cribriform and tubular structures are the major architectural patterns. The primary differential diagnosis is cutaneous metastasis from a cribriform visceral carcinoma; others include primary secretory carcinoma of the skin, adenoid cystic basal cell carcinoma and primary cutaneous adenoid cystic carcinoma.

Giant eyelid eccrine hidrocystoma-induced progressive ptosis in childhood.

An upper lid eccrine hidrocystoma presenting as early childhood progressive ptosis is very rare. We present a 9-year-old female child with droopy right upper lid since birth and progressive increase in symptoms. She had right upper lid ptosis (marginal reflex distance 1 of -1 mm) with fair levator function (8 mm) and abnormal cystic change on the conjunctival side. Computerized tomography imaging delineated the well-defined cystic lesion with homogeneous cavity with no contrast enhancement. Following the cyst excision, a giant eccrine hidrocystoma measuring 25 mm × 15 mm was removed, the largest reported in pediatric eyes. The case demonstrates the possibility of giant lid eccrine hidrocystomas presenting as progressive ptosis at a pediatric age and the need for early surgical intervention to prevent amblyopia.

Eccrine Syringofibroadenoma in Association With Acquired Epidermodysplasia Verruciformis.

A 75-year-old man with human immunodeficiency virus infection and numerous biopsy-proven warts for 10 years, refractory to cryosurgery, cimetidine, and topical imiquimod, presented with numerous pink to hypopigmented verrucous papules and plaques involving the face, trunk, buttocks, and groin. Laboratory evaluation revealed a CD4 T-cell count of 62 cells per microliter and human immunodeficiency virus viral load of <117 copies per milliliter. Biopsy of a plaque groin lesion was performed. Histopathology revealed vertically oriented anastomosing strands of basaloid epithelium arising from multiple points along the epidermis in a background fibrovascular stroma. Ductal differentiation was identified. Areas of epidermis showed compact orthokeratosis, coarse hypergranulosis, and keratinocytes with abundant steel-blue-gray cytoplasm, indicative of viral cytopathic changes. Cytologic atypia was not identified. Human papillomavirus (HPV) genotyping of this lesion was positive for types 5 and 14. Overall, the findings were consistent with epidermodysplasia verruciformis in association with eccrine syringofibroadenoma (ESFA). The patient was subsequently treated with acitretin and showed clinical improvement. ESFA is an uncommon benign adnexal tumor with unknown pathogenesis. Although its association with HPV has rarely been reported, ESFA in the setting of acquired epidermodysplasia verruciformis has not been described. The development of ESFA in this case may be the result of HPV-induced cellular transformation.

Apocrine Metaplasia Found at MR Biopsy: Is There Something to be Learned?

The purpose of this study was to determine (a) the frequency of apocrine metaplasia (ApoM) found on MR core biopsy of suspicious findings, and (b) to determine if there are specific MR imaging features that might obviate the need for biopsy. This HIPAA-compliant retrospective study was performed under IRB exemption for quality assurance studies. Patient demographics, MR imaging features, and pathology were reviewed. Breast lesions which underwent MR-guided biopsy, yielding ApoM on pathology analysis were included. Retrospective review of MR imaging features of these lesions was performed by two radiologists blinded to pathology results except for the presence of ApoM. Imaging features on MR assessed included location, size, morphology, T1 and T2 signals, and enhancement kinetics. Full pathology results were subsequently reviewed during data analysis. The pathology slides and imaging was subsequently reviewed by two fellowship trained radiologists and a breast pathologist to categorize the finding of ApoM into target lesion (imaging corresponds to size of lesion on pathology) versus incidental lesion. Target lesion characteristics were assessed to determine specific MRI features of ApoM. Between January 2011 to November 2012, 155 distinct breast lesions suspicious for malignancy successfully underwent MR-guided biopsy. Of the 155 lesions biopsied, 123 (79%) were benign and 32 (21%) were malignant. Of the 123 benign biopsies, ApoM was found in 57 (46%), of which 35 (61%) had no associated atypia and 22 (39%) had associated atypia. Of the 32 malignant biopsies, three (9%) had associated ApoM (DCIS in two cases and DCIS/LCIS in one case). Of the 60 cases with ApoM, only 11 (18.3%) were target lesions and 49 were incidental lesions (81.7%). Of the 60 cases with ApoM, 35 (58%) were masses (average size 0.8 cm for both with or without atypia) and 25 (42%) were nonmass enhancement (NME) (average size 2.1 cm with and 1.0 cm without atypia). Only five (14%) of 35 masses demonstrated spiculated margins, of which four were associated with atypia (80%). Of 22 lesions with atypia or other high-risk lesion, 14 (64%) were masses, most commonly with irregular margins (64%). Of the 12 T2 hyperintense lesions, only two (1.7)% had associated atypia or high-risk lesion, and none were associated with malignancy. Of the 11 target lesions, seven were T2 hyperintense. Enhancement kinetics were variable: 30 (50%) showed mixed persistent and plateau kinetics, eight (13%) persistent delayed enhancement, 10 (17%) plateau kinetics, four (7%) washout kinetics, and eight (13%) were below threshold for kinetic analysis. ApoM is a common benign pathologic result at MR-guided core biopsy for both masses and NME accounting for 39% of all biopsy results in this series. Although there is considerable variability in imaging characteristics on MR, our results suggest biopsy may be safely obviated for lesions that are subcentimeter T2 hyperintense areas of NME and short term follow-up imaging may be a reasonable alternative for these lesions.

Porocarcinoma ecrino: a propósito de un caso / Eccrine porocarcinoma: a case report

El porocarcinoma ecrino es un tumor cutáneo, maligno, infrecuente, de crecimiento lento, que deriva de las glándulas sudoríparas ecrinas. Se describe la evolución de una paciente femenina de 46 años, que presenta un tumor cutáneo raro localizado en la región inguinal. Se realiza una resección amplia con vaciamiento inguinopélvico y colgajo de avance del muslo, y se indica radioterapia adyuvante, la cual se negó a recibir; a los seis meses de operada, sufrió una recaída ganglionar pélvica irresecable. En la consulta multidisciplinaria, se le planteó realizar hemipelvectomía clásica, con la que no estuvo de acuerdo. Falleció a consecuencia de metástasis pulmonares, 10 meses después de operada(AU)