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.

The story of a deformed leprous foot.

Eccrine syringofibroadenoma (ESFA) is a rare adnexal tumour of eccrine ductal proliferation. A 50 year old treated case of leprosy presented with a chronic non healing ulcer of 5 years duration on the deformity laden right foot. Multiple verrucous papules and plaques were seen surrounding the ulcer which showed histopathological findings consistent with ESFA. Although ESFA constitutes a rare association with leprosy, considering the load of treated cases in our country and elsewhere, it may represent an under-reported entity which requires more attention in the post elimination era.

Linear syringocystadenoma papilliferum on the retroauricular area associated with nevus sebaceus.

Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. We report a child with linear retroauricular distribution of syringocystadenoma papilliferum. A background nevus sebaceus was shown histologically. Total excision was curative with no recurrence. An association between the linear variant of syringocystadenoma papilliferum and nevus sebaceus has not been reported previously.

Lymphangiectatic Variant of Eccrine Spiradenoma - A Diagnostic Challenge.

Conventional eccrine spiradenoma is a benign, slow growing and painful tumor of the skin. While the tumor does not usually present a diagnostic dilemma, a rare variant with marked stromal lymphedema can be a challenge to interpret. We present a case of lymphangiectatic variant of eccrine spiradenoma in an 82-year-old white male who presented with a persistent left flank lesion for several months. The patient was initially asymptomatic and subsequently developed a suspected abscess that was excised to reveal a 6.5 cm subcutaneous mass. Microscopic examination reveals strands and cords of dark, epithelial, round to oval cells with inconspicuous nucleoli streaming between prominently dilated and congested vascular spaces. Within the cystic component there are small ductular structures. Additionally, prominent stromal lymphedema is present. To the best of our knowledge, there is only one reported case of this entity in the English literature. This case represents a diagnostic challenge and the purpose of reporting it is to alert surgical pathologists, dermatopathologists and dermatologists of the existence of this unusual variant of eccrine spiradenoma.

Eccrine syringofibroadenomatosis of the reactive subtype occurring in chronic poorly-controlled psoriasis.

Eccrine syringofibroadenomatosis (ESFA) is a rare adnexal tumor with acrosyringeal differentiation. Clinically, it can be mistaken for granulomatous infections or malignancies such as squamous cell carcinoma. Despite the rarity of the condition, we recently encountered two cases of the reactive subtype, which occurred in patients with poorly controlled chronic psoriasis. Both patients presented with long-standing, thick verrucous lesions on the lower legs. The diagnosis was made after histological examination and exclusion of infectious and neoplastic disorders. As this is a reactive disorder, management is focused on treating the underlying condition. Unfortunately, psoriasis was difficult to manage in both our patients and they defaulted further treatment. It is important to recognize ESFA as it can be confused with infectious or malignant disorders.

Syringocystadenoma.

We present a case of a 35-year-old woman with a yellow, verrucous, and itchy plaque on her scalp. Within this plaque, there was an erythematous, bleeding papule. Histopathologic findings were compatible with a diagnosis of syringocystadenoma papilliferum within a nevus sebaceous. We present a brief review of the natural history of nevus sebaceus, its pathogenesis, and management.

Syringocystoadenoma papilliferum associated with apocrine hidrocystoma and verruca.

Syringocystoadenoma papilliferum is a benign adnexal tumor usually located in head and neck that occurs during childhood or adolescence. A case of a syringocystoadema papilliferum associated with apocrine hydrocystoma and verruca is presented. It is unusual to see the occurrence of three histopathologic types of tumors coexisting in one cutaneous lesion.

Adenocarcinoma in situ arising in vulvar papillary hidradenoma: report of 2 cases.

Adenocarcinoma in situ rarely occurs in vulvar papillary hidradenoma. We encountered 2 cases of adenocarcinoma in situ arising in a papillary hidradenoma of the vulva. Both patients were asymptomatic women, aged 83 and 92 years, who presented with nodules (1.0 and 2.0 cm) on the vulva. Macroscopically, the lesions seemed tan-pink, fleshy, and well circumscribed. One tumor ulcerated the overlying epidermis. On microscopic examination, the tumors showed focal features of benign hidradenoma at the periphery with transitions into areas of increasing cytologic atypia that fulfilled criteria for adenocarcinoma in situ similar to that seen in the breast. One tumor showed a predominant cribriform pattern with moderate atypia and many mitoses; the other showed a mixture of cribriform and micropapillary patterns, mild atypia, and fewer mitotic figures. There was no evidence of destructive invasion or desmoplasia in either tumor. Both lesions show areas strongly immunoreactive for mammaglobin and gross cystic disease fluid protein 15 as well as estrogen and progesterone receptor protein in 1 case. There was no evidence of benign ectopic breast tissue within or adjacent to the neoplasms. Both patients underwent local excision with no evidence of tumor recurrence at 15 and 32 months of follow-up. The fact that these tumors displayed morphologic and immunohistochemical features that resembled ductal carcinoma in situ of the breast demonstrates the close homology between papillary hidradenoma and breast epithelium. In the absence of invasion, our experience suggests that these tumors can be cured by local excision.

[Malignant nodular hidradenoma and multiple nodular hidradenomas in a hypogonadic patient]. / Hidradénome nodulaire malin et hidradénomes nodulaires multiples chez un malade hypogonadique.

BACKGROUND: We report a case of multiple benign nodular hidradenomas associated with malignant nodular hidradenoma in a hypogonadic patient. CASE-REPORT: A 49 year-old man presented 11 benign nodular hidradenomas in the pectoral region. A malignant nodular hidradenoma had recently appeared in this region some distance from the pre-existing benign modular hidradenomas. This patient had peripheral hypogonadism associated with congenital bilateral cryptorchidia responsible for gynecomastia due to "relative hyperestrogenism". Large numbers of estrogen receptors were demonstrated in each of the benign nodular hidradenomas. The malignant nodular hidradenoma mass was unavailable and screening for these receptors was not possible. DISCUSSION: Cases of multiple nodular hidradenoma are extremely rare: we have seen only two. To the best of our knowledge, there have been no reports to date of combined malignant nodular hidradenoma and multiple benign nodular hidradenoma. However, there is histological evidence of transition forms. The coexistence of endocrine anomalies associated with this type of tumour has never been reported. However, the presence of estrogen receptors has occasionally been demonstrated in benign nodular hidradenomas. Our patient's endocrine disease may have played a role in the presentation of multiple hidradenomas, but this cannot be demonstrated.

[Pigmented apocrine hidradenoma]. / Hidroadenoma apocrino pigmentado.

Apocrine hidradenoma is a benign adnexal neoplasm. It has no specific site predilection, and usually affects middle-aged people. The same as other tumors of the sweat glands, there is a pigmented variety. We present the case of a 92-year-old male who consulted his physician for a slow-growing asymptomatic lesion in the right groin which had been developing for 2 years. After the histopathological study, the diagnosis was established as pigmented apocrine hidradenoma.

Sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum arising within a nevus sebaceus.

BACKGROUND: Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. OBJECTIVE: The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. METHODS: A 45-year-old woman presented with a nevus sebaceus that contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. RESULTS: Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. CONCLUSION: This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus.

Malignant eccrine hidradenoma of neck causing acute heart failure.

Tumours of eccrine sweat glands are uncommon, with complex classification and different terms used even for the same tumour. Therefore, for practical purposes, it may be sufficient to differentiate between benign and malignant. Malignant eccrine hidradenoma has a predilection for head and neck, with high incidence of recurrence following surgical excision and also regional and distant metastases. We describe a case, which presented as a medical emergency with symptoms of severe anaemia and acute heart failure secondary to intermittent bleeding from a huge ulcerative neck lesion, which was subsequently diagnosed as eccrine hidradenocarcinoma. The tumour was successfully treated with complete surgical excision and reconstruction followed by radiotherapy. Two years postoperatively, the patient is very well with no sign of recurrence.

Eccrine syringofibroadenoma arising in peristomal skin: a report of two cases.

Eccrine syringofibroadenoma (ESFA) is a benign neoplasm arising from the intraepidermal portion of eccrine ducts. It is characterized by a distinctive histologic pattern of epithelial cells arranged in anastomosing cords surrounded by a fibrovascular stroma. Approximately 50 cases of ESFA have been reported, and in recent years the lesion has been described occurring in association with other skin conditions. We report 2 cases of ESFA arising in abdominal skin adjacent to enterostomy sites.