Folliculocystic and Collagen Hamartoma: A Subset of Fibrous Cephalic Plaque.

Anita S Savell,Kyle Norton,Michael R Heaphy

doi:10.7759/cureus.14987

Abstract

Tuberous sclerosis complex is known to cause a variety of cutaneous hamartomas, most commonly hypomelanotic macules, angiofibromas, shagreen patches, and fibrous cephalic plaques. In recent years, a new cutaneous hamartoma that bears physical and histological resemblance to fibrous cephalic plaque has been proposed called folliculocystic and collagen hamartoma. The primary difference between the two diagnoses is the histologic presence of infundibular cysts in the latter. However, some authors have called into question if the two diagnoses are truly distinct. In this case report, we present a patient with tuberous sclerosis complex and fibrous cephalic plaque with infundibular cysts and propose that the presence of cysts should be incorporated into the possible histologic features of fibrous cephalic plaque.

Highlights

Many people with tuberous sclerosis complex (TSC) have cutaneous manifestations: hypomelanotic macules (97.2%), facial angiofibromas (74.5%), shagreen patches (48%), fibrous cephalic plaques (FCPs) (18.9%), and periungual fibromas (15.1%) [1]
Tuberous sclerosis complex is known to cause a variety of cutaneous hamartomas, most commonly hypomelanotic macules, angiofibromas, shagreen patches, and fibrous cephalic plaques
We present a patient with tuberous sclerosis complex and fibrous cephalic plaque with infundibular cysts and propose that the presence of cysts should be incorporated into the possible histologic features of fibrous cephalic plaque

Summary

Introduction

Many people with tuberous sclerosis complex (TSC) have cutaneous manifestations: hypomelanotic macules (97.2%), facial angiofibromas (74.5%), shagreen patches (48%), fibrous cephalic plaques (FCPs) (18.9%), and periungual fibromas (15.1%) [1]. FCP typically presents as large, smooth-to-bumpy, firm-to-rubbery plaques on the forehead, face, scalp, or neck of patients with TSC [2]. It is histologically characterized by thick collagen deposition with perifollicular fibrosis and may demonstrate increased numbers of dilated vessels [2]. A 17-year-old male with a known history of TSC and epilepsy presented to the dermatology clinic with multiple angiofibromas on the face and a large, painless exophytic mass on his central forehead. He requested excision due to cosmetic concerns (Figure 1). Nodular lesions previously excised from the patient's scalp (white arrow)

Discussion

Conclusions

Findings

Disclosures

Hake S