Combination treatment of bilateral periocular sebaceous carcinomas with microsatellite instability with neoadjuvant pembrolizumab and Mohs surgery.

This case highlights the successful use of pembrolizumab for neoadjuvant treatment of MMR-deficient sebaceous carcinoma of bilateral eyelids to reduce tumour burden allowing smaller defect post-Mohs surgery and better reconstructive outcome. Microsatellite stability, tumour mutational burden and PD-L1 expression are important prognostic factors to be considered for the use of neoadjuvant pembrolizumab. Further studies are needed to determine if neoadjuvant pembrolizumab consistently improves surgical and cosmetic outcomes and reduces local recurrence and metastasis.

Long pulsed dye laser with a back-to-back double-pulse technique and compression for the treatment of epidermal pigmented lesions.

BACKGROUND: In the recent past, long pulsed dye lasers (LPDL) have been investigated for the treatment of epidermal pigmented lesions (EPLs). Using a pigmented lesion compression headpiece, blood is pushed laterally out of the laser field focusing laser energy on melanin. Recent studies have demonstrated excellent responses using a single-pulse at the following settings: 9-12 J/cm2 , 1.5 milliseconds, 7-10 mm spot size with compression. However, the majority of these studies report patients requiring up to four treatments for lesion resolution. Herein, we describe our experiences utilizing a back-to-back double-pulse technique to decrease the total number of treatments needed for EPL clearance. METHODS: Thirty-six patients (27 females, 9 males; skin types I-IV) with benign facial EPLs were included. Each lesion received two back-to-back pulses (fluence of 9-12 J/cm2 , 1.5 milliseconds duration, and 7 mm spot size). If needed, a second treatment was delivered 4-8 weeks later. Pre- and post-photos after the first treatment were evaluated by two independent board-certified dermatologists. RESULTS: Lesion clearance after the first treatment was graded on a Likert scale as: 1 = poor (<25% clearance); 2 = fair (25-50%); 3 = good (51-75%); and 4 = excellent (>75%). Of the 36 participants, 23 had excellent clearing, 10 with good, 2 with fair and 1 with poor clearance. There was only one case of post-inflammatory hyperpigmentation that subsequently resolved, and no cases or scarring or hypopigmentation. CONCLUSION: LPDL with compression continues to be a safe and effective modality for treatment of EPLs. Double-pulsing decreases the total number of treatments needed for lesion resolution, while maintaining safety and potentially decreasing cost. Lasers Surg. Med. 51:136-140, 2019. © 2018 Wiley Periodicals, Inc.

Micropapular Cutaneous Sarcoidosis Reviewed.

BACKGROUND: Micropapular cutaneous sarcoidosis (MPCS) is a rare variant of sarcoidosis. Herein we review the literature and include a recent case of MPCS discussing pathogenesis, diagnosis, treatment, and prognosis. METHOD: A review was conducted using the terms "micropapular sarcoidosis" and "micropapular sarcoid." A recent case of a 50-year-old male patient with biopsy-identified MPCS was also included in the review. RESULTS: In total, 12 cases with an aggregate of 18 patients were included in the review. Presentation among all patients was consistent, with scattered, occasionally pruritic, faintly erythematous shiny white papules. Skin biopsy demonstrated noncaseating granulomas. Systemic prednisone, oxytetracycline, and hydroxychloroquine, as well as topical betamethasone, were used for therapy. CONCLUSION: In our review there does not seem to be a clear link as to the definite cause of the MPCS. While the relationships to tuberculosis and autoimmunity seem to be often emphasized, there was no clear association with either etiology.

Ewing-Like Sarcoma: A Case of a Primary Cutaneous Primitive Neuroectodermal Tumor With Atypical Immunohistochemistry and Lack of Fusion Oncogene Detection.

We present a 25-year-old male patient with a primary cutaneous primitive neuroectodermal tumor (cPNET) with unusual immunohistochemistry and lack of fusion oncogene generation. The lesion expressed CD99 and WT-1, and the histological features were consistent with cPNET. Differential diagnoses such as rhabdomyosarcoma, desmoplastic small round blue cell tumor, hematolymphoid neoplasm, neuroblastoma, and CIC-DUX round cell sarcoma were ruled out based on immunohistochemistry, genetic studies, and histology. Previous cPNET cases have been published detailing abnormal immunochemistry and genetic expression. However, to our knowledge, fusion oncogene negativity in cPNET tumors has only been reported in one other published case series. These reports, including this study, reinforce the fact that a high index of suspicion should be used when diagnosing these tumors, regardless of immunohistochemical and genetic variability. This case highlights that the typical genetic and immunohistochemical features of cPNET may be more variable than previously thought. Future studies are needed to better understand these variations of cPNET.

Angiomyxoma of the nasal dorsum treated by Mohs surgery.

Cutaneous angiomyxomas are myofibroblastic neoplasms with locally aggressive behaviour and a high risk of recurrence. We describe a case of a solitary cutaneous angiomyxoma presenting on the nasal dorsum of a 28-year-old man, excised with Mohs surgery using permanent section control and repaired with an advancement flap. Histology showed myxoid nests of bland CD34-positive and vimentin-positive stellate and spindled cells in the deep dermis and abundant thin-walled blood vessels. An echocardiogram, performed to rule out the possibility of a cardiac myxoma with cutaneous embolisation, was normal.

Mohs moat: peripheral cutaneous margin clearance in a collaborative approach for aggressive and deeply invasive basal cell carcinoma.

Although Mohs micrographic surgery is the standard of care for large, aggressive or recurrent non-melanoma skin cancers of the head and neck, tumours that involve deep underlying structures (including bone, parotid gland and named nerves) are impractical for extirpation under local anaesthesia. Such cases are often referred to a head and neck surgeon, who typically relies on intraoperative frozen section analysis of the peripheral cutaneous margin. Here we describe the use of the Mohs moat technique as part of a collaborative approach for the treatment of aggressive and deeply invasive basal cell carcinoma that allows an analysis of the complete peripheral cutaneous margin and results in decreased operating room and general anaesthesia time.

Acral peeling skin syndrome resulting from a homozygous nonsense mutation in the CSTA gene encoding cystatin A.

Acral peeling skin syndrome (APSS) is a clinically and genetically heterogeneous disorder. We used whole-exome sequencing to identify the molecular basis of APSS in a consanguineous Jordanian-American pedigree. We identified a homozygous nonsense mutation (p.Lys22X) in the CSTA gene, encoding cystatin A, that was confirmed using Sanger sequencing. Cystatin A is a protease inhibitor found in the cornified cell envelope, and loss-of-function mutations have previously been reported in two cases of exfoliative ichthyosis. Our study expands the molecular pathology of APSS and demonstrates the value of next-generation sequencing in the genetic characterization of inherited skin diseases.

Acral peeling skin syndrome: a clinically and genetically heterogeneous disorder.

Acral peeling skin syndrome (APSS) is a rare, autosomal, recessive genodermatosis characterized by painless spontaneous exfoliation of the skin of the hands and feet at a subcorneal or intracorneal level. It usually presents at birth or appears later in childhood or early adulthood. Some cases result from mutations in the TGM5 gene that encodes transglutaminase 5, which has an important role in cross-linking cornified cell envelope proteins. We report a new APSS pedigree from Jordan that contains at least 10 affected family members, although sequencing of the TGM5 gene failed to disclose any pathogenic mutation(s). On the basis of probable consanguinity, we performed homozygosity mapping and identified areas of homozygosity on chromosomes 1, 6, 10, 13, and 16, although none of the intervals contained genes of clear relevance to cornification. APSS is a clinically and genetically heterogeneous disorder, and this Jordanian pedigree underscores the likelihood of still further heterogeneity.

Postoperative Candida Infection following Complex Periocular Reconstruction.

Postoperative candida infection is a rarely reported complication in cutaneous surgery, although it may develop more often in particular clinical settings. We present a 59-year-old woman with a well-controlled human immunodeficiency virus infection. She developed a bright red eruption with satellite pustules 2 weeks after excision of recurrent lentigo maligna melanoma of the left lower eyelid and periocular region. Due to defect size and complexity of the reconstruction (glabellar transposition flap, Hughes flap, composite graft from upper contralateral eyelid, and full-thickness skin graft from ipsilateral retroauricular region), she was placed on prophylactic oral amoxicillin-clavulanic acid and topical bacitracin and polymyxin. Immediate postoperative course was unremarkable, and sutures were removed after 7 days. Three days later, she developed bright red erythema and pustules within the surgical site and complained of burning. Empirically she was switched to topical gentamicin and oral ciprofloxacin, and later to linezolid, due to inadequate response. Wound culture grew Candida albicans sensitive to fluconazole and voriconazole. After oral fluconazole and topical clotrimazole initiation, the patient rapidly improved. The graft remained viable and apart from small partial dehiscence on the cheek, the healing was unremarkable. Apart from the case presentation, we also discuss different factors associated with postoperative candida infection, including immunocompromised status, surgical procedure location, and postoperative antibiotic use. Early recognition and treatment of postoperative candida infections are crucial to prevent delayed healing and associated morbidity.

Multiple clustered dermatofibroma: case report and review of the literature.

The presence of multiple dermatofibromas is rare and is defined as more than 15 lesions. Multiple clustered dermatofibroma (MCDF) is a distinct entity with only 12 reported cases in the literature. MCDF occurs in healthy individuals of both sexes in the first to third decades on the lower half of the body and portends an excellent prognosis. On histology, MCDF is consistent with benign dermatofibromas. We report a 31-year-old healthy Hispanic woman with a 14-year history of slowly progressive MCDF located on her right hip initially misdiagnosed as dermatofibrosarcoma protuberans. We believe this case represents the 13th report of MCDF in the literature and the second from North America.