The First Case Report of Multiple Familial Trichoepitheliomas Responding Successfully to Topical Benzoyl Peroxide: A Possible Therapeutic Action Underlying Structural Turnover and Antiinflammation.

Sir,

Multiple familial trichoepitheliomas (MFT) is an autosomal dominant genodermatosis of skin appendage tumor, and may present in syndromic conditions, including Brooke–Spiegler syndrome, Rombo syndrome, or Bazex syndrome. It mostly affects the face during puberty and tends to become obviously toward adolescent ages, thus causing significant cosmetic morbidity. Moreover, updated evidence series explored the premalignant potential of long-standing MFT.[12] Currently, no established treatments are available for the disease. We present, to our knowledge, the first documented case of MFT responded satisfactorily to topical benzoyl peroxide (BPO), a free-radical generating compound that has been well-recognized for the treatment of acne.

An otherwise healthy 18-year-old Japanese female presented with multiple, skin-colored papules on her mid-face, which had developed since childhood [Figure 1a]. Under the provisional diagnosis of acne vulgaris or angiofibroma associated with tuberous sclerosis complex, physicians have introduced topical and systemic antibiotics, which were all unhelpful. Her mother and uncle have the same skin manifestation. Dermoscopy of the skin lesion showed milia-like cysts and telangiectasis [Figure 1b]. Histopathological examination showed comedones lined by irregularly shaped basaloid cell nests which invaded downwards. The nests were surrounded by fibrous stroma and lymphocytic infiltrates [Figure 1c, d]. The clinicopathological features and family history suggest the diagnosis of MFT.

Figure 1

Clinical presentation and skin pathology. (a) Multiple, skin-colored papules scattering over her mid-face. (b) Dermoscopy showing milia-like cysts and telangiectasis. (c, d) Histopathology showing comedones lined by irregularly shaped basaloid cell nests that were surrounded by fibrous stroma and lymphocytic infiltrates: (c, H & E ×40; d, ×100)

She denied receiving any conservative therapies, such as cryotherapy, laser therapy, and electrosurgery, because of anxiety concerning post-treatment pigmentation or scaring. Therefore, we challenged to use a topical BPO (BEPIO gel 2.5%; Maruho, Osaka, Japan). By three months of a BPO monotherapy, her facial papules gradually became flatter and smaller in size [Figure 2a]. Seven months later, the remaining papules became more inconspicuous, concomitant with faded telangiectasia [Figure 2b]. There were no adverse events. The skin lesions have never flared and remained a favorable clinical control at least during 2.5 years of the treatment follow-up. We recommended the topical BPO to her mother and uncle, but they refused.

Figure 2

Clinical course after BPO monotherapy. (a) After three months of BPO monotherapy, facial papules became relatively flatter and smaller in size. (b) Seven months later, the remaining papules became more inconspicuous

MFT poses a treatment dilemma, particularly in young female. Surgical excision, carbon dioxide laser, and cryotherapy by liquid nitrogen are often selected, although these may confer an increased risk of local scarring and pigmentation, with a high frequency of relapse. Topical imiquimod, tretinoin, and sirolimus raise a single-case efficacy but remain a therapeutic challenge.[1] Moreover, long-standing MFT lesions may develop malignancy, such as trichoblastic carcinoma, basal cell carcinoma, or squamous cell carcinoma.[12]

BPO is a potent oxidizer that has been widely accepted as the first-line therapy of acne. It harbors variable pharmacological action; i) comedolytic activity loosening intercellular spaces of follicular plugs, ii) stimulation of keratinocyte proliferation/differentiation, and iii) anti-inflammatory effects through inhibition of proinflammatory cytokines, including tumor necrosis factor-α, IL-1α, and IL-8, released from the follicular epithelium and surrounding inflammatory infiltrates.[34] In addition, BPO enhances peroxidative modification of microstructural lipids, including cell membrane lipids and intra-corneal ceramide, contributing to accelerate the corneo-epidermal and follicular turnover.[5] A complex of these might reorganize the overall follicular/perifollicular circumstances of trichoepithelioma and bring a favorable clinical outcome in our case. Because of its safety and ease of administration, a topical BPO can be worth challenging for MFT.

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Conflicts of interest

There are no conflicts of interest.