Reactive eccrine syringofibroadenomatosis secondary to primary cutaneous amyloidosis: a novel association

Abstract

We report the unprecedented case of reactive eccrine syringofibroadenoma (ESFA) secondary to primary cutaneous amyloidosis. A 62-year-old woman of Asian ethnicity presented with a pruritic rash on the back of long-standing duration. Physical examination revealed diffuse hyperpigmentation localized to the interscapular region; there were a multitude of hyperpigmented macules merged in a rippled pattern intermixed with scattered papules and cobblestone-like areas. A punch biopsy from a papule was taken. Histopathological examination revealed a network of epithelial strands and cords hanging from the epidermis and harboring foci of ductal differentiation. Eosinophilic collections of amorphous material were found between the epithelial strands, obscuring the superficial dermis. The microscopic picture was consistent with primary cutaneous amyloidosis associated with reactive ESFA. Results of histochemical and immunohistochemical staining confirmed the diagnosis. We speculate that pathogenetic mechanisms intrinsic to primary cutaneous amyloidosis, in addition to unknown genetic factors, resulted in clinical changes of lichen amyloidosus associated with an abnormal hyperplastic epithelial response with histopathological features of ESFA rather than the common epidermal change of acanthosis and hyperkeratosis.

Syringocystadenocarcinoma Papilliferum In Situ–Like Changes in Extramammary Paget Disease: A Report of 11 Cases

“…This lesion is considered by many to be a benign eccrine neoplasm but an alternative view is that it represents a reactive process involving the epidermis and eccrine ducts. 20,21 The latter conclusion is supported by the microscopic heterogeneity of cases reported as ESFA and their wide clinical associations, including burns, 22 erosive palmoplantar lichen planus, 23 stoma, 29,30 podoconiosis, 31 amyloidosis, 32 and other conditions. 33 Some lesions have clearly been induced by irritation from urine, feces, mechanical friction, or trauma.…”

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

“…3,8 ESFA associated with hidrotic ectodermal dysplasia (Clouston syndrome and Schopf's syndrome) is genetically mediated and falls in the non-reactive category. 5 Either the genes responsible for ESFA and HED are closely linked or ESFA manifests in fully expressed HED syndrome. 9 The variety of clinical phenotypes reflects heterogeneity in the gene defects.…”

“…4 No significant differences were detected between the immunostaining (IHC) properties of primary and reactive ESFA, supporting the view of ESFA as a homogeneous histopathological reaction pattern. 5 ESFA may resolve spontaneously, or persist and grow slowly for a variable period of time. Solitary ESFA is treated with surgical excision.…”

Reactive Eccrine Syringofibroadenoma Associated with Neuropathy, Venous Stasis, and Diabetic Foot Ulcer

“…ESFA is categorized into 5 subtypes according to its clinical presentation: (1) solitary ESFA, a solitary verrucous nodule in elderly adults; (2) multiple ESFA associated with Clouston syndrome and Schöpf-Schulz-Passarge syndrome; (3) multiple ESFA without associated cutaneous findings, also referred to as syringofibroadenomatosis; (4) nonfamilial unilateral linear ESFA (nevoid ESFA), and (5) reactive ESFA associated with inflammatory or neoplastic dermatoses (e.g., erosive lichen planus, bullous pemphigoid, epidermolysis bullosa, primary cutaneous amyloidosis, burn scar, venous stasis, chronic diabetic foot ulcer, neuropathy, nevus sebaceous, and squamous cell carcinoma) [ 2 , 4 5 6 7 8 9 10 11 ].…”