Keratoacanthoma centrifugum marginatum after photodynamic therapy with good response to oral retinoids and topical 5‐fluorouracil

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma (KA), characterized by progressive peripheral growth, and usually devoid of deep invasion. Different systemic (oral retinoids) or topical treatments have been reported, but there is not a well‐defined therapeutic protocol. We report the case of a KCM developing after photodynamic therapy (PDT) on the right leg of a 64‐year‐old woman. It was treated successfully with oral acitretin combined with topical 5‐Fluorouracil + salicylic acid for 5 months. This is the first case of KCM developing after PDT and successfully treated with oral retinoid combined with topical treatment.     

Keratoacanthoma centrifugum marginatum associated with mechanical trauma: Response to acitretin—A case report and review of the literature

Keratoacanthoma centrifugum marginatum (KCM) is a very rare variant of keratoacanthoma, characterized with progressive centrifugal growth, central healing, and atrophy. Due to its rarity and lack of distinctive histopathological features, KCM often raises diagnostic and therapeutic challenge. We present a case of a 76‐year‐old Caucasian woman with a single large tumor on her right shin that responded to oral retinoids. The patient presented history of local trauma. The tumor developed over the course of 20 months from a scar. To the best of our knowledge, this is the fifth case of KCM associated with mechanical trauma as a possible triggering factor.     

Ineffectiveness of radiotherapy for the treatment of keratoacanthoma centrifugum marginatum: A case report

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacantoma (KA). Although KCM shares histological and clinical features with KA, KCM has no tendency of spontaneous regression, and presents with progressive peripheral expansion with a bank‐shaped outer wall and concurrent central healing. As such, early diagnosis and proper treatment of the patient are required. However, because of its rarity, previous reports are insufficient to evaluate which treatment should be selected. Here, we report a case of KCM that responded to radiotherapy, but relapsed 6 months later.     

Extramammary Paget disease

We report the case of a 60-year-old man with penile-scrotal extramammary Paget disease (EMPD). The patient initially underwent Mohs micrographic surgery, but the margins remained positive after several sections; multiple scouting punch biopsies used to define the extent of the tumor were also positive. Because of concerns about functional impairment and cosmesis associated with wide local excision, the patient instead chose treatment with topical 5 percent imiquimod cream as a cytoreductive and margin-defining treatment. Owing to the association between EMPD and underlying malignant conditions, a thorough metastatic evaluation is necessary, particularly to rule out genitourinary cancer in the setting of penile-scrotal EMPD. Management of EMPD is complicated by the multifocal, non-contiguous nature of the disease and the presence of clinically occult extensions. As a result, recurrence rates after surgery are high. Several non-surgical modalities have been used to treat EMPD, which include radiotherapy, topical imiquimod, topical 5-fluorouracil, topical bleomycin, photodynamic therapy, CO2 laser ablation, and topical retinoids. Systemic chemotherapy also has been used to treat advanced EMPD. However, because EMPD is so uncommon, clinical trials comparing the various methods of treatment are lacking. Regardless of the mode of treatment, long-term follow up is essential, given the high rate of recurrence.     

Treatment of angiolymphoid hyperplasia with eosinophilia with the carbon dioxide laser

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, idiopathic condition that presents with isolated or grouped plaques or nodules in the periauricular region, forehead, or scalp. ALHE is marked by a proliferation of blood vessels with distinctive large endothelial cells accompanied by a characteristic inflammatory infiltrate that includes eosinophils. The lesion is benign but may be persistent and difficult to eradicate. Various therapeutic modalities that have been tried for its treatment include intralesional and oral corticosteroids, cryotherapy, oral retinoids, vinblastine, surgical excision, laser therapy, and INFalpha2a. We report two cases with this rare condition: one patient, treated with cryotherapy, did not improve, while the second patient was successfully treated with the CO(2) laser.     

Vitamin D Deficiency After Oral Retinoid Therapy for Ichthyosis

Oral retinoids are being increasingly used to treat ichthyotic disorders in children. We report on two children with ichthyotic disorders who developed unusual manifestations after they were started on oral retinoids. The first case is a 10‐year‐old girl with nonbullous ichthyosiform erythroderma and the second is a 2‐year‐old girl with lamellar ichthyosis. The child with ichthyosiform erythroderma developed features of rickets within months of initiation of systemic retinoids. Her baseline examination before initiation of oral retinoids was normal. The second patient with lamellar ichthyosis was found to have low vitamin D levels after 6 months of retinoid therapy, and prompt supplementation reversed the levels in 2 months. These cases are being reported to bring attention to the probable need for initiation of vitamin D supplementation with systemic retinoid therapy in ichthyotic disorders in children.     

口腔黏膜色素痣4例临床及组织病理分析

目的报道4例口腔黏膜内痣病例,并探讨口腔黏膜色素痣的临床病理学特征、诊断及鉴别诊断要点。方法收集4例发生在口腔黏膜的色素痣患者临床资料,进行光学显微镜观察及随访,并结合文献复习。结果 4例患者临床表现均为无痛性斑片或斑块,无明显不适,3例位于牙龈,1例位于颊部黏膜,均行手术完整彻底切除,镜下形态与皮肤皮内痣相似。随访4个月～5年,均未见复发或恶变。结论口腔黏膜色素痣是一种罕见部位的黑色素细胞痣,手术彻底切除预后好。     

Treatment of severe recalcitrant hidradenitis suppurativa with adalimumab

Hidradenitis suppurativa is a chronic inflammatory skin disease featuring inflammatory nodules, fistulas and discharge of secretions in the intertriginous regions. Without therapy the disease is chronic and progressive. The most effective treatment is the radical wide excision of the affected areas. Systemic antibiotics, immunosuppressants, oral retinoids or antiandrogens have limited beneficial effects. TNF‐α antagonists may represent a new therapeutic approach for patients suffering from severe hidradenitis suppurativa offering promising positive influence on the disease outcome. In contrast to infliximab and etanercept, there are only few reports describing the use of adalimumab in severe hidradenitis suppurativa. Here we report a case of a patient with severe recalcitrant hidradenitis suppurativa successfully treated with adalimumab.     

Squamous epithelial carcinoma at the site of lichen ruber hypertrophicus of the glans penis

Reports of cancer development in lichen planus mostly concern the oral erosive form. The reported frequency of malignant transformation ranges between less than 1% and 10%. There have been a few isolated observations of malignant transformation of cutaneous lichen planus. In these cases, the hypertrophic type located on the lower limbs predominated. We report an unusual case of squamous cell carcinoma developing on the glans penis in the presence of hypertrophic lichen planus. Systemic treatment of the lichen planus with dapsone, retinoids, corticosteroids and topical cyclosporin A failed to prevent this development.     

Late diagnosis of ectodermal dysplasia syndrome

This case study reports the clinical, skin biopsy and molecular findings in a 56‐year‐old Filipino man with the autosomal recessive ectodermal dysplasia disorder, Schöpf‐Schulz‐Passarge syndrome, the precise nature of which was established only after reading of a similar case in this journal. In addition to the late diagnosis, successful clinical management of his acral hyperkeratosis and ulceration has been difficult, with oral retinoids exacerbating the skin fragility.     

Poroid hidradenoma in the hand: A case report and systematic review

Poroid hidradenoma (PH) is a rare benign neoplasm of the skin appendages. There are currently no guidelines for its management, and little information available regarding the natural history, treatment options, or outcomes. Systematic literature review identified 19 cases of isolated PH. Mean age at presentation was 54 years. Male to female ratio was 3:1, and the majority of cases were in the head and neck region. One‐third of lesions were painful or tender. Sizes varied from 0.11 × 0.9 × 0.2 cm (digit) to 6 cm (presternal region). Twelve cases were treated by surgical excision, while the remainder were biopsy specimens. Follow‐up was reported in five cases, with a median follow‐up of 1 year. No cases of local invasion or spread have been reported, although there was one case of possible recurrence identified 8 years after surgical excision. The presented case is the first to identify a PH on the hand, represents the smallest lesion to date, and was successfully treated by excision. We suggest that PHs be treated by excision and followed up for 6 to 12 months. Given one case of potential (but unconfirmed) recurrence, and no report of malignant transformation, we suggest that narrow margins may be appropriate.     

Keratoacanthoma centrifugum marginatum—A rare variant of keratoacanthoma: Case report and literature review

Keratoacanthoma centrifugum marginatum (KCM) is a rare variant of keratoacanthoma and is characterized by progressive peripheral growth with accompanying central healing. Here, we report a case of multiple KCM. A 53-year-old man presented with multiple erythematous papulonodules on both upper limbs and neck for >2 years. His skin lesions enlarged in an annular manner with central residual cribriform scarring that eventually formed confluent plaques (2–8 cm in diameter) with elevated hyperkeratotic borders. Skin biopsy of a developed matured nodule on the right forearm was consistent with that of classical keratoacanthoma. KCM was diagnosed on the basis of clinical and pathological presentation. Low-dose acitretin (0.7 mg/kg/day) was administered and the skin lesions improved significantly within 3 months after the treatment. In this case, we present the clinical and histological features of KCM and discuss the different effective treatment modalities.     

Spontaneous resolution of keratoacanthoma centrifugum marginatum

Keratoacantnoma centrifugum marginatum (KCM) is a rare variant of keratoacantnoma, with > 40 cases reported world wide. Spontaneous resolution of KCM is very rare. To our knowledge, this is the first case of KCM with spontaneous resolution as documented by serial photographs.     

Large plaque‐type blue naevus with subcutaneous cellular nodules

We report a new case of ‘large plaque‐type blue naevus (PTBN) with subcutaneous cellular nodules’ involving the breast. A 25‐year‐old‐woman presented with a large plaque, 140 × 100 mm in size, on the right chest wall involving the right breast, associated with recent subcutaneous nodules. Histopathological examination revealed features of cellular and common blue naevus. Dermatoscopy was performed, and the diagnosis of PTBN was made. PTBN is a rare condition with an imprecise prognosis. In cases of PBTN lesions on the head, clinicians must keep in mind the risk of malignant transformation of PTBN or an association with cutaneous neurocristic hamartoma. For this new variant of PTBN appearing on the trunk, we propose close monitoring of the patient if surgical excision is impossible.     

Successful treatment with topical imiquimod of anal Buschke–Löwenstein tumor in a child

Buschke–Lowenstein tumor (BLT) is a large exophytic lesion of the anogenital region with a clinically malignant aspect but a benign histology. Because of its low incidence, this pathology is exceptional in childhood. Surgical excision is the main therapeutic approach. Herein, we report a case of BLT successfully treated with imiquimod in a 2‐year old boy. Young children with BLT should be offered a chance of medical treatment before extensive surgical destruction is used.     

Post‐transplant plasmablastic lymphoma of the skin

Plasmablastic lymphoma (PBL) is a recently described rare variant of diffuse large B‐cell lymphoma characterised by its aggressive nature and plasmacytic differentiation. It most frequently arises in the oral cavity of human immunodeficiency virus (HIV)‐infected patients. However extra‐oral involvement is becoming increasingly recognised, particularly in HIV‐negative patients. We report a case of PBL presenting as multiple violaceous nodules and plaques on the leg of a HIV‐negative patient, 13‐years post‐renal transplant. To date, 20 cases of PBL presenting in the skin have been reported. We review and compare the clinico‐pathological features of these cases.     

Keratoacanthoma centrifugum marginatum

Keratoacanthoma centrifugum marginatum (KCM) is a rare distinct variant of keratoacanthoma. Based on three personal observations and a review of the literature, the authors describe the clinical and histological features of this neoplasm. Clinically KCM is characterized by the lack of a tendency for spontaneous remission and by continuous centrifugal spread. Histologically there is a subclinical, iceberg-like growth pattern. Like keratoacanthoma, KCM is a highly differentiated, biologically benign, non-metastasizing tumour. The treatment of choice is early excision of the tumour.     

Presternal Dermoid Cyst Mimicking Lymphatic Malformation: a Case Report and Review of the Literature

Abstract: We describe an 11‐month‐old boy with an unusually large presternal mass present since birth. The large size, fluctuant properties, transillumination, compressibility, and imaging of this lesion were characteristic of a lymphatic malformation. Although four treatments with sclerotherapy markedly reduced its size, it was not until definitive treatment with surgical excision and the final pathology report that we arrived at the ultimate diagnosis of dermoid cyst. Dermoid cysts, although appearing along embryologic lines of closure, are rarely presternal. They are usually small, thick walled, and filled with sebaceous or keratinous fluid, which typically allows for clinical diagnosis, and show characteristic features on magnetic resonance imaging (MRI) and ultrasound. However, this case illustrates that dermoid cysts can appear in somewhat atypical locations, and imaging is not always diagnostic, so dermoid cyst should remain a part of the differential diagnosis for any lesion presenting midsternally, regardless of the size and imaging characteristics.     

A Case of Trichilemmal Carcinoma Treated with Mohs Micrographic Surgery

Trichilemmal carcinoma is a cutaneous adnexal tumor originating from the outer root sheath of hair follicle, and it was first described by Headington in 1976. Clinically, it usually occurs as an asymptomatic solitary papule, nodule or mass on the face or scalp. This neoplasm is a malignant counterpart of trichilemmoma, and it has been reported in the literature as trichilemmal carcinoma, tricholemmal carcinoma, malignant trichilemmoma, and tricholemmocarcinoma. Although histologically, trichilemmal carcinoma frequently has maliganant features, it has a relatively benign clinical behavior. We think Mohs micrographic surgery is a useful treatment modality in trichilemmal carcinoma because the final skin defect is smaller than a wide excision. We report a case of primary trichilemmal carcinoma which had developed on the face, treated with Mohs micrographic surgery.     

Keratoacanthoma centrifugum marginatum arising in vitiligo: a case report

Keratoacanthoma centrifugam marginatum (KCM) is a rare variant of the Keratoacanthoma (KA) form of squamous cell carcinoma with only 30 cases reported to date. We report a case of KCM arising in a long-standing vitiligo lesion chronically exposed to sunlight. Over years, the vitiligo lesions gradually evolved into sclerotic plaques and subsequently KCM developed in one of the plaques.