Solitary skin-colored nodule on a child’s face

Sir, A healthy, six-year-old boy presented with a slowly grown dome-shaped nodule on the mandibular angle region present for two years. The patient’s past medical and family history were unremarkable. A physical examination revealed a solitary, 1.3 × 1 cm, firm, painless, flesh-colored tumor (Fig. 1). Dermoscopy showed branching, serpentine vessels on a pink background (Fig. 2a). These features disappeared when slight pressure was exerted on the dermoscope and the tumor exhibited a central, white, structureless area (Fig. 2b). An excisional biopsy was performed. A microscopic examination showed a well-circumscribed, paucicellular dermal tumor composed of eosinophilic collagen bundles separated by clefts and forming a storiform pattern. Scattered fibroblasts were found among the collagen bundles. The overlying epidermis was slightly flattened (Fig. 3). The diagnosis of solitary sclerotic fibroma was established. Sclerotic fibroma (SF), also known as storiform collagenoma, is a rare benign skin tumor. It usually manifests itself as an asymptomatic, slowly growing, white-to-skin-colored papule or nodule [1]. It was first described in patients with Cowden’s disease, yet may also occur sporadically [2]. There were no mucocutaneous features of Cowden’s disease (tricholemmomas, oral fibromas, acral keratoses, palmar pits, and gingival and palatal papules) in the patient and her family members. Dermatofibroma, the main differential diagnosis of SF, usually exhibits hyperplastic changes of the epidermis instead of atrophy, and the boundaries of the lesion are unclear [2]. Only two papers have been published describing the dermoscopic findings of SF, consisting of a white background with peripheral arborizing vessels [3]. A white background may be related to an increased dermal collagen density. It is also described in dermatofibroma, typically with a peripheral pigmentation network. Although dermoscopy may improve the clinical diagnosis of SF, histopathological analysis is required.

Sclerotic Fibroma: A Differential Diagnosis of Spontaneous Keloid

CASE DESCRIPTIONSclerotic fibroma (SF) is a rare neoplasm first described in 1972 by Weary et al in association with Cowden syndrome.1 This rare syndrome typically manifests with mucocutaneous findings (trichilemmomas, verrucous papules in the oral mucosa, acral keratosis and sclerotic fibromas) and an increased risk for several neoplasms.1 Later, Rapini e Golitz coined the term solitary SF in the absence of Cowden syndrome.2 It is debatable whether SF is distinct lesion or a sclerotic evolutionary end-point of other conditions.3 SF manifests clinically as an asymptomatic, well-demarcated skin-colored to erythematous papule or nodule with no site or sex predilection.2,3 The authors present a healthy 56-year-old male with a pink, linear plaque located on the left shoulder slowly growing for about 20 years. He was previously treated with intralesional corticosteroids for a suspected spontaneous keloid but the patient abandoned treatment by his own initiative. [...] REFERENCES1. Weary PE, Gorlin RJ, Gentry WC, Jr., Comer JE, Greer KE. Multiple hamartoma syndrome (Cowden’s disease). Arch Dermatol. 1972;106:682-90.2. Rapini RP, Golitz LE. Sclerotic fibromas of the skin. J Am Acad Dermatol. 1989;20:266-71.3. High WA, Stewart D, Essary LR, Kageyama NP, Hoang MP, Cockerell CJ. Sclerotic fibroma-like change in various neoplastic and inflammatory skin lesions: is sclerotic fibroma a distinct entity? J Cutan Pathol. 2004;31:373-8. Received: 22/09/2017 - Accepted: 30/11/2017

Do you know this syndrome?

Cowden's disease or multiple hamartoma syndrome is an autosomal dominant inherited disease and the main dermatological features are facial trichilemmomas (hamartomas of the follicular infundibula), oral fibroma and benign acral keratoses. The importance of this disease lays in the increased susceptibility to malignization of some lesions, especially breast, thyroid and genitourinary tract. Despite its varied phenotypic expression, this disease is generally unknown. Consequently, many cases are undiagnosed or diagnosis comes at a late stage, which reinforces the importance of an early investigation of the disease so the patient may have periodic check-ups to discover and treat malignancies.