Reactive Eccrine Syringofibroadenoma (ESFA) in Association With a Merkel Cell Carcinoma Excision Scar.

ABSTRACT: Eccrine syringofibroadenoma (ESFA) is a rare benign skin adnexal lesion of the acrosyringium of eccrine sweat ducts. Reactive ESFA, a subtype of ESFA, is usually associated with non-neoplastic cutaneous dermatoses or neoplastic skin tumors. Clinically, the lesions can be solitary or multiple, pink, or skin-colored coalescing papules or nodules of variable sizes. Histopathologically, this tumor is composed of numerous anastomosing cords of monomorphic cuboidal epithelial cells with eccrine duct formation. The association of reactive ESFA with benign conditions, such as psoriasis, diabetic polyneuropathy, scars, and leprosy, has been reported. However, the association of reactive ESFA with malignant tumors is extremely rare, with very few cases reported in the literature. We present a case of a 72-year-old woman who developed reactive ESFA associated with Merkel cell carcinoma excision scar. The ESFA tumors developed in the area of the surgical graft 10 months after the Merkel cell carcinoma had been excised. New ESFA tumors have continued to appear in the scar on a yearly basis while, so far, has been no recurrence of the original tumor. However, the presence of new tumor growths in the area suggested the possibility of recurrence of the Merkel cell carcinoma. That possibility was enhanced by the fact that PET scans revealed hypermetabolic activity in the ESFA papules.

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.

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.

Eccrine spiradenoma with chondroid syringoma in Blaschkoid distribution.

Eccrine spiradenoma (ES) very rarely presents in a linear or zosteriform distribution. It may be associated with foci of various other appendageal tumors. We report a 14-year-old boy who presented to us with multiple nodules in a linear distribution in the posterior aspect of the right lower limb since 2 years of age. The lesions became significantly painful for the past 2 years. Histopathology revealed dermal lobules of ES with smaller foci of chondroid syringoma.

Characterizing the nature of eccrine syringofibroadenoma: illustration with a case showing spontaneous involution.

Eccrine syringofibroadenoma (ESFA) is a histological entity. It has been widely debated as to whether ESFA is a true neoplasm, a hamartoma or a form of reactive hyperplasia. ESFA should not be considered a homogenous condition and there are five clinical subtypes: (i) multiple ESFA associated with ectodermal dysplasia, (ii) multiple ESFA without cutaneous features (both hamartomatous), (iii) unilateral linear ESFA (naevoid), (iv) solitary ESFA (neoplastic) and (v) reactive ESFA, which seems to start off as pathologically hyperplastic and then become hamartomatous. We report a patient with reactive ESFA secondary to lepromatous leprosy, showing spontaneous involution in an area of her lesions. Reactive ESFA represents a hyperplastic and hamartomatous process, and may be more appropriately known as reactive eccrine syringofibroadenomatosis. The risk of malignancy is low in reactive ESFA, and monitoring instead of excision is a reasonable option.

Leprosy-associated eccrine syringofibroadenoma of Mascaro.

Eccrine syringofibroadenoma of Mascaro is a rare benign tumour. There are five subtypes, one of which is known to occur in reaction to inflammatory and neoplastic dermatoses. We describe a patient with previous lepromatous leprosy presenting with multiple flesh-coloured verrucous plaques over the right foot. Histology was consistent with eccrine syringofibroadenoma. To our knowledge, this is the first reported case of multiple eccrine syringofibroadenomata occurring in association with leprosy. The tumours in this case are most likely reactive in nature, subsequent to multiple traumatic events with tissue remodelling in an insensate foot affected by leprosy. It is less probable that the tumours are a result of scarring from recurrent infections. We also raise the possibility of a neuroeccrine interaction, with sympathetic neuropathy in leprosy as a contributing factor in the pathogenesis.