Gardner-Diamond Syndrome: A Psychodermatological Condition in the Setting of Immunodeficiency.

Gardner-Diamond syndrome (GDS) is a psychological and dermatologic syndrome involving painful, ecchymotic, purpuric lesions that typically appear after a period of stress or minor trauma. This syndrome most commonly occurs in young women, though it has also been less commonly reported in men and adolescents. It is an uncommon condition and appropriate diagnosis is important to properly manage symptoms and minimize risks to patients. Here report a case of GDS presenting in a woman with common variable immunodeficiency (CVID), which, to the best of our knowledge, is not a comorbid condition that has previously been reported in correlation with GDS.

Erythroderma: A Rare Complication of Dyshidrotic Eczema.

A 47-year-old man with a history of dyshidrotic eczema presented to the emergency department with diffuse erythema, chills and pruritus of three weeks' duration. The patient had received two injections of methotrexate in the preceding two weeks, both of which had failed to improve his whole-body erythema and pruritus. In the emergency department, the patient was evaluated for infection and admitted for the dermatology consultation. After being seen on the general medical floor by the dermatology service the diagnosis of erythroderma was made and the patient was treated with intravenous (IV) cyclosporine therapy, with which his rash dramatically improved over three days. This case report summarizes the presentation and differential of erythroderma, and highlights the importance of having a high index of suspicion for this potentially fatal disease.

Clinical features and management of schwannoma affecting the upper and lower lips.

Head and neck schwannomas comprise 25-40% of all schwannomas, with presentation on the lips as the rarest and most surgically complicated site for perioral tumors. A systematic literature review was conducted to include 21 cases of patients with schwannoma of the upper or lower lips. The majority of patients presented with a single, painless, well-encapsulated nodule on the upper or lower lips. The nodules were consistently slow-growing, with an average 29.3 months from symptom onset to clinical presentation. Most cases were complicated by profound cosmetic disfigurement as well as dysphagia, dysarthria, snoring, and/or sleep apnea. Overall, histological analysis was consistent with classic schwannoma, and all cases were treated via complete surgical excision, and for malignant tumors, additional therapy was utilized. All but four cases achieved full remission by final follow-up. Recurrence rate for benign lip schwannomas was 5.3%, which is remarkably different from the standard recurrence rate of 8-24% for benign peripheral schwannomas. Additionally, the proportion of malignant tumors was greater for lip schwannomas than other schwannomas. Lip schwannomas demonstrate different characteristics than schwannomas from other locations on the body, and these remarkable differences highlight significant implications for clinical practice. Complete excision is the primary mode of treatment with overall excellent postoperative prognosis and rare instances of recurrence. Given the rarity of this tumor, this review of available cases serves to comprehensively describe clinical presentation and surgical treatment approaches to upper and lower lip schwannomas.

Clinical Features and Treatment of Fibrous Histiocytomas of the Tongue: A Systematic Review.

Introduction Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Objectives This study systematically reviews the available literature discussing the clinical and pathological features of malignant and benign fibrous histiocytomas. Data Synthesis A total of 20 cases were included in this review. Patient-level data were extracted from cases to include clinical presentation, workup, treatment, and outcome. Conclusion Benign fibrous histiocytomas are consistent in clinical and histopathologic presentation. Surgical treatment provides excellent outcome, with no recurrence in all excised cases. Malignant tumors have a more aggressive clinical and pathological presentation. Surgical treatment with possible adjuvant radiotherapy resulted in recurrence in 40% of cases (follow-up of 24 months), and death due to disease in 47% of patients (follow-up of 19 months).

Clinical Features and Treatment of Fibrous Histiocytomas of the Tongue: A Systematic Review

Abstract Introduction Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Objectives This study systematically reviews the available literature discussing the clinical and pathological features of malignant and benign fibrous histiocytomas. Data Synthesis A total of 20 cases were included in this review. Patient-level data were extracted from cases to include clinical presentation, workup, treatment, and outcome. Conclusion Benign fibrous histiocytomas are consistent in clinical and histopathologic presentation. Surgical treatment provides excellent outcome, with no recurrence in all excised cases. Malignant tumors have a more aggressive clinical and pathological presentation. Surgical treatment with possible adjuvant radiotherapy resulted in recurrence in 40% of cases (follow-up of 24 months), and death due to disease in 47% of patients (follow-up of 19 months).

Clinical Features and Surgical Treatment of Schwannoma Affecting the Base of the Tongue: A Systematic Review.

Introduction Schwannomas of the head and neck account for 25-40% of all cases, with presentation at the base of the tongue as the most frequent site for intraoral tumors. Objectives Here, a systematic review was conducted to include 15 cases of patients with schwannoma of the base of the tongue. Data Synthesis Most patients presented with a single, painless, well-encapsulated nodule at the base of the tongue. These nodules were slow-growing, with an average of 13.3 months from onset to presentation. Most cases were accompanied by airway obstruction, indicated by symptoms of dysphagia, dysarthria, snoring, and sleep apnea. Overall, the histological studies were consistent with a benign schwannoma with a palisading Antoni A and Antoni B pattern without malignant changes in cell morphology. These tumors were treated via complete surgical excision, and all cases achieved full remission by final follow-up. Conclusion Surgical removal is the primary mode of treatment with excellent postoperative prognosis and rare instances of recurrence. Given the rarity of this tumor, this review of available case studies serves to comprehensively describe clinical presentation and surgical treatment approaches to tongue base schwannoma.

Clinical Features and Surgical Treatment of Schwannoma Affecting the Base of the Tongue: A Systematic Review

Abstract Introduction Schwannomas of the head and neck account for 25-40% of all cases, with presentation at the base of the tongue as the most frequent site for intraoral tumors. Objectives Here, a systematic review was conducted to include 15 cases of patients with schwannoma of the base of the tongue. Data Synthesis Most patients presented with a single, painless, well-encapsulated nodule at the base of the tongue. These nodules were slow-growing, with an average of 13.3 months from onset to presentation. Most cases were accompanied by airway obstruction, indicated by symptoms of dysphagia, dysarthria, snoring, and sleep apnea. Overall, the histological studies were consistent with a benign schwannoma with a palisading Antoni A and Antoni B pattern without malignant changes in cell morphology. These tumors were treated via complete surgical excision, and all cases achieved full remission by final follow-up. Conclusion Surgical removal is the primary mode of treatment with excellent postoperative prognosis and rare instances of recurrence. Given the rarity of this tumor, this review of available case studies serves to comprehensively describe clinical presentation and surgical treatment approaches to tongue base schwannoma.

Approach to management of giant basal cell carcinomas.

Basal cell carcinoma (BCC) is a common nonmelanoma skin cancer with increasing incidence in the United States and worldwide. It is strongly linked to UV radiation exposure and typically is slow growing. Malignancy in BCCs is due to local growth and invasion rather than metastasis, and the prognosis is generally favorable. We report the case of a 60-year-old man with a large, locally destructive giant BCC on the right side of the upper back that was successfully treated via complete surgical excision (SE) and did not recur in the subsequent 36 months. We review the indications, evidence, advantages, and disadvantages associated with multiple surgical and nonsurgical treatment modalities available for the management of giant BCCs.

Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It typically presents as a dermal plaque or nodule on the trunk, limbs, or head and neck region. Vulvar DFSP has also been described, although it is less common. OBJECTIVE: To review the available literature and discuss the clinical course of DFSP affecting the vulva. MATERIALS AND METHODS: We reviewed the existing English-language literature on DFSP of the vulva with respect to clinical presentation, diagnosis, treatment, and outcome. RESULTS: Thirty three case reports and series were included (n = 54 patients). Vulvar DFSP most commonly presents as a slowly enlarging tender or asymptomatic mass on the labia majora, with histological findings of classic DFSP. Most patients were treated with wide local excision. Three patients were treated with Mohs micrographic surgery, which may decrease local recurrence and seems well suited for use in vulvar DFSP. CONCLUSION: This literature review comprehensively reviews and describes the clinical presentation of vulvar DFSP and the treatment options for this rare vulvar neoplasm.

Clinical features and treatment of vulvar Merkel cell carcinoma: a systematic review.

BACKGROUND: Merkel cell carcinoma is a rare and aggressive neoplasm originating from mechanoreceptor Merkel cells of the stratum basale of the epidermis. Cases affecting the vulva are exceedingly rare, with the currently available literature primarily in case report form. BODY: Systematic review of the PubMed database returned 17 cases of Merkel cell carcinoma affecting the vulva. Patients presented at a mean age of 59.6 years with a firm, mobile vulvar mass. Symptoms of pain, erythema, pruritus, edema, and ulceration have been reported. Tumor histology is consistent with that of neuroendocrine tumors and typical Merkel cell carcinomas. Neuroendocrine and cytokeratin immunostains are frequently utilized in histopathological workup. Surgical management was the unanimous first-line therapy with adjuvant radiation in most cases. Recurrence occurred in 70.6% of patients at a mean follow-up of 6.3 months. Mortality was at 47.0% at a mean of 7.8 months after initial operation. CONCLUSION: Merkel cell carcinoma affecting the vulva is an extremely rare and highly aggressive neoplasm. The present review of published cases serves to comprehensively describe the clinical course and treatment approaches for vulvar Merkel cell carcinoma.

Light and laser treatment modalities for disseminated superficial actinic porokeratosis: a systematic review.

Treatment of disseminated superficial actinic porokeratosis (DSAP) is poorly standardized. The present review seeks to comprehensively discuss the potential for laser and light modalities in the treatment of DSAP. A systematic review of light and laser treatment modalities was conducted to include 26 cases of patients with DSAP. Systematic review resulted in 14 articles to be included. Photodynamic therapy (PDT) overall was the least successful treatment modality, with clinical improvement seen in a minority of patients (MAL-PDT: N = 9 patients, 33.3% showed improvement; ALA-PDT: N = 3 patients, 0% improvement; hypericin-PDT: N = 2 patients, 0% improvement) after numerous post-procedural side effects of hyperpigmentation, inflammation, erythema, and discomfort. Overall, in the available reports, PDT demonstrates poor outcomes with greater incidence of side effects. The response rates of DSAP lesions treated with lasers were as follows: (Q-switched ruby lasers: N = 2, 100%; CO2 laser: N = 1, 100%; PDT and CO2 combination therapy: N = 2, 0-50%; erbium and neodymium YAG lasers: N = 2, 100%; fractional 1927-nm thulium fiber lasers: N = 2, 100%; Grenz rays: N = 1, 100%; and fractional photothermolysis: N = 2, 100%). The side effects of laser therapy were minimal and included mild erythema, slight hyperpigmentation, and moderate edema. Laser therapy is a promising treatment option for DSAP with an excellent side effect profile. However, higher power studies are required to determine optimal guidelines for laser treatment of DSAP.

Systematic review of laser therapy in xanthelasma palpebrarum.

Xanthelasma palpebrarum is a benign periorbital xanthoma with substantial cosmetic and psychosocial burden for patients. Treatment modalities should be considered based on efficacy as well as cosmetic outcome. Laser modalities in the treatment of xanthelasma palpebrarum have not been comprehensively reviewed and discussed. Accordingly, this study seeks to systematically and critically review the available literature discussing laser treatment of xanthelasma palpebrarum. PubMed was systematically reviewed for reports on laser therapy in the treatment of xanthelasma palpebrarum. A total of 21 studies were included in this review discussing laser treatment of xanthelasma palpebrarum. Laser types included carbon dioxide, yttrium aluminum garnet, pulsed dye, argon, and a 1450 nm diode laser. The carbon dioxide laser was the most commonly reported modality followed by yttrium aluminum garnet laser. All of the laser modalities offered moderate to excellent clearance rates with minimal side effect profiles. Further large scale studies comparing different laser modalities are required to determine the best laser modality. However, laser modalities as a whole offer a treatment option for xanthelasma palpebrarum, that is, cosmetically excellent with a reasonable side-effect profile.

Has Vitamin D Had Its "Time In The Sun" For Melanoma?

Growing evidence suggests a pivotal role for vitamin D in cancers, particularly melanoma. The broad immunologic effects of vitamin D and its signaling axis make for a complex interplay between tumor cells and the immune system. Preclinical evidence suggests vitamin D to have protective effects in melanoma oncogenesis and progression, creating a potential role for vitamin D supplementation in cancer prevention and/or adjuvant therapy. In this commentary, the authors highlight studies of vitamin D in melanoma with clinical implications and call for large clinical trials to definitively determine the role of supplementation in patients to prevent and help treat melanoma.

Indurated vascular plaques on the legs of an 85-year-old woman.

This is a case of classic type Kaposi sarcoma occurring in an 85-year-old woman who presented with indurated vascular plaques on both legs below the knee that has been present for two years. A brief review of the literature on Kaposi sarcoma is included.

Rapidly growing hemorrhagic papule on the cheek of a 54-year-old man.

A 54-year-old man sought medical attention for a growth on his right cheek that had been present for three months. The growth began as a small, brown "pimple" that gradually increased in size over time. Physical examination revealed a 9 mm well-circumscribed erythematous nodule with a hemorrhagic crust. On dermoscopy, the lesion was completely vascular appearing, with no pigment visualized. A clinical diagnosis of pyogenic granuloma was made. The lesion was biopsied and histopathologic examination revealed a 2.8 mm thick, Clark level IV, ulcerated, amelanotic nodular melanoma. Because the literature contains reports of nodular melanoma mimicking the presentation of a pyogenic granuloma, all such lesions should be biopsied for histopathologic diagnosis.

Erythema ab igne: a case report and review of the literature.

Erythema ab igne (EAI) is a rare condition since the advent of central heating. Its incidence has been rising as heating sources are being used to treat chronic pain. Multiple activities that chronically expose patients to heating sources also have been documented with this condition. We present a case of EAI induced by the use of an electric blanket.

A man with an umbilicated papule of the hand: what is your diagnosis?

Introduction. Ecthyma contagiosum is a zoonotic disease caused by the parapoxvirus that causes "sore mouth" in sheep and goats and orf in human. Case Presentation. A 61-year-old sheep farmer presented with a painful non-pruritic lesion on the left hand that had been present for approximately 5 weeks. Physical examination demonstrated a 1 cm pearly, umbilicated papule with raised borders. A biopsy showed an asymmetrical nodule with parakeratotic crust and acanthosis with thin epidermal strands extending deeply in the underlying dermis. Marked edema, capillary proliferation and extensive lymphocytic infiltration was also present. One red intranuclear inclusion was identified in an epidermal keratinocyte. A diagnosis of human orf (ecthyma contagiosum) was made. Conclusion. Infected sheep and freshly vaccinated sheep or goats are the reservoir for human infection. After an incubation period of 3-7 days, parapoxvirus infections produce 1-3 painful lesions measuring 1-2 cm in diameter. The natural history of the disease is complete resolution and no treatment is indicated. Prevention of echthyma contagiosum in ruminants through vaccination is thought to be the best way to control infection.

Poorly differentiated squamous cell carcinoma arising in tattooed skin.

Introduction. Tattoos have increasingly become accepted by mainstream Western society. As a result, the incidence of tattoo-associated dermatoses is on the rise. The presence of a poorly differentiated squamous cell carcinoma in an old tattooed skin is of interest as it has not been previously documented. Case Presentation. A 79-year-old white homeless man of European descent presented to the dermatology clinic with a painless raised nodule on his left forearm arising in a tattooed area. A biopsy of the lesion revealed a poorly differentiated squamous cell carcinoma infiltrating into a tattoo. The lesion was completely excised and the patient remains disease-free one year later. Conclusion. All previous reports of squamous cell carcinomas arising in tattoos have been well-differentiated low-grade type or keratoacanthoma-type and are considered to be coincidental rather than related to any carcinogenic effect of the tattoo pigments. Tattoo-associated poorly differentiated invasive carcinoma appears to be extremely rare.