Eccrine syringofibroadenomatosis associated with hidrotic ectodermal dysplasia

Abstract

Eccrine syringofibroadenoma (ES) is generally regarded as a solitary benign adnexal neoplasm of the skin. Recently, the epithelial and stromal changes of this lesion have been recognized as being a reaction pattern in certain settings. We describe two individuals in whom this process was a manifestation of hidrotic ectodermal dysplasia. In both cases, the diagnosis had been missed for many years and the process had been thought to be a manifestation of an inflammatory dermatosis of the palms and soles. In both instances, other family members were found to be affected. Thus, ES may be manifested clinically as erythematous erosive plaques, especially of the palms and soles. It is important that clinicians be familiar with this disorder and be aware that it may exhibit unusual cutaneous manifestations.

Reactive eccrine syringofibroadenoma arising in peristomal skin: An unusual presentation of a rare lesion

“…9,13 ESFA has been associated with numerous entities/conditions, which are summarized in Table I. These include nevus sebaceus, 29 hidrotic ectodermal dysplasia, 28 and diabetic polyneuropathy. 30 In addition, a reactive subtype of ESFA 6,12,31,32 has been reported in association with burn scar ulcers, 33 bullous pemphigoid, 34,35 and erosive palmoplantar lichen planus.…”

“…3,7 Together, the features raise a differential diagnosis that includes fibroepithelial tumor of Pinkus, tumor of the follicular infundibulum, pseudoepitheliomatous hyperplasia, papillary eccrine adenoma, seborrheic keratosis, squamous cell carcinoma, and artifacts of histologic processing. 22,28 Immunohistochemical studies show that the lesional cells are positive for keratin 6, keratin 19, and filaggrin. [9][10][11][12]14,23 In addition, electron microscopic studies show that the ducts have a keratinization pattern similar to acrosyringeal cells.…”

Metastatic Melanoma in a Patient with Clouston Syndrome Successfully Treated with Isolated Hyperthermic Limb Perfusion

“…syringofibroadenoma, a benign adnexal neoplasm of the skin presenting as erythematous erosive plaque, has been documented in two patients with Clouston syndrome. 5 Although melanoma has also been associated with some forms of ectodermal dysplasia, such as ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome, 9 the incidence of melanoma in patients with Clouston syndrome is unknown. Thus far, to our knowledge, this is the first case report of melanoma arising in a patient with Clouston syndrome.…”

“…3 A wide variety of unusual skin manifestations have been described; however, the incidence of melanoma in patients with Clouston syndrome is unknown. 4,5 Subungual melanoma is an infrequent presentation of cutaneous melanoma that develops in only 2-3% of Caucasian patients but in a higher proportion of dark-skinned patients. [6][7][8] Here we report a case of a patient with Clouston syndrome with recurrent in-transit melanoma from a subungual primary melanoma who was successfully treated with hyperthermic isolated limb perfusion (IHLP) using melphalan.…”

Dedifferentiated Salivary Hybrid Carcinoma of the Maxillary Sinus with Pagetoid Spread to the Overlying Lining Mucosa

“…Hybrid carcinoma of salivary glands is a very rare subtype of malignant salivary gland neoplasm with a little more than 30 cases documented in the literature [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] (See Table 1). Most cases of hybrid carcinoma of salivary glands have been reported in the parotid gland and palate.…”