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.

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.

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 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.

Purpura annularis telangiectodes of Majocchi: case report and review of the literature.

BACKGROUND: Purpura annularis telangiectodes of Majocchi is an uncommon form of pigmented purpuric dermatosis which may present a therapeutic challenge. Given the rare nature of this condition, there is limited anecdotal information available regarding optimal therapy. Although pigmented purpuric dermatoses are generally innocuous, in some cases they may cause patients significant distress, and there is a need to exclude cutaneous T-cell lymphoma. METHODS: We reviewed the literature on the treatment of pigmented purpuric dermatoses and managed a 69-year-old woman who presented with purpuric annular patches on the legs. RESULTS: Three separate biopsies demonstrated an interstitial to perivascular lymphocytic infiltrate with erythrocyte extravasation, consistent with pigmented purpuric dermatosis. The patient's condition proved refractory to many of the previously reported modes of management, but markedly improved with methotrexate. Treatment alternatives for pigmented purpuric dermatosis are reviewed, and a treatment algorithm is proposed. CONCLUSION: This is the first reported case regarding the successful use of methotrexate for pigmented purpuric dermatosis. Methotrexate may offer a therapeutic alternative to patients with highly symptomatic pigmented purpuric dermatosis refractory to other, more conservative, treatment modalities.

Do melanoidins induced by topical 9% dihydroxyacetone sunless tanning spray inhibit vitamin d production? A pilot study.

We report here preliminary pilot study data of the effect of sunless tanning spray with 9% [Correction added after online publication (August 24th, 2009): The concentration of Dihydroxyacetone used in the study was 9% and not 3% as previously stated] dihydroxyacetone (DHA) on 25-hydroxyvitamin D [25(OH)D] serum levels in subjects exposed to controlled amounts of UV-B radiation during April/May in Omaha, NE, 41 degrees N latitude. We found that DHA-induced melanoidins in skin act as a topical sunscreen attenuating the formation of 25(OH)D.

Desmoplastic trichoepithelioma: report of a case illustrating its natural history.

First described more than 30 years ago, desmoplastic trichoepithelioma is a rare but benign adnexal neoplasm. Most often identified in middle-aged individuals and females, desmoplastic trichoepithelioma usually is a solitary annular plaque. Though the tumors are benign, the possibility of malignant neoplasm may spark both clinical and histologic concern. A full-thickness skin biopsy is advisable when desmoplastic trichoepithelioma is suspected. A patient's clinical history may provide some clues to help guide diagnosis, as the tumors may be present for years and slow growth is commonly reported. We present a patient with desmoplastic trichoepithelioma that uniquely documents and supports the typical natural history of this tumor, as demonstrated by annual school photographs.

Manifestations of cutaneous diabetic microangiopathy.

The etiologies of a variety of skin conditions associated with diabetes have not been fully explained. One possible etiological factor is diabetic microangiopathy, which is known to affect the eyes and kidneys in patients with diabetes. There are many mechanisms by which diabetes may cause microangiopathy. These include excess sorbitol formation, increased glycation end products, oxidative damage, and protein kinase C overactivity. All of these processes occur in the skin, and the existence of a cutaneous diabetic microangiopathy has been well demonstrated. These microangiopathic changes are associated with abnormalities of skin perfusion. Because the skin plays a thermoregulatory role, there is significant capillary redundancy in normal skin. In diabetic patients, loss of capillaries is associated with a decrease in perfusion reserve. This lost reserve is demonstrable under stressed conditions, such as thermal stimulation. The associated failure of microvascular perfusion to meet the requirements of skin metabolism may result in diverse skin lesions in patients with diabetes. Many skin conditions peculiar to diabetes are fairly rare. Necrobiosis lipoidica diabeticorum (NLD) and diabetic bullae occur very infrequently as compared with diabetic retinopathy and nephropathy. Conversely, there is a correlation between diabetic microvascular disease and NLD. This correlation also exists with more common skin conditions, such as diabetic dermopathy. This relationship suggests that diabetic microangiopathy may contribute to these conditions even if it is not primarily causal. Clinically, the major significance of diabetic cutaneous microangiopathy is seen in skin ulceration which is very common and has a major impact on diabetic patients. Many factors contribute to the development of diabetic foot ulcers. Neuropathy, decreased large vessel perfusion, increased susceptibility to infection, and altered biomechanics all play a role, but there is no doubt that inadequate small blood vessel perfusion is a major cause of the inability to heal small wounds that eventually results in ulcer formation. The accessibility of skin capillaries makes cutaneous diabetic microangiopathy an attractive model for research on the evolution of microvascular disease in diabetic patients.

A case of human orf contracted from a deer.

Orf, or contagious ecthyma, is a rare viral dermatosis caused by a member of the genus Parapoxvirus. The typical lesion consists of solitary or multiple papules that progress through a series of stages, terminating in complete resolution. This zoonotic disease is most commonly transmitted to humans from infected sheep or goats. We report a case of human orf, likely contracted from exposure to deer carcasses.