Dermatology Online Journal

Toxic epidermal necrolysis (TEN) is a severe life-threatening adverse drug reaction that predominantly involve the skin and mucous membranes, and is associated with high mortality (25-35% or even higher) and with various long term sequelae. There is no universally accepted treatment for TEN, but key elements of management include rapid diagnosis, identification and interruption of the culprit drug, evaluation of the prognosis using SCORTEN, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents. Cyclosporine has recently emerged as a promising immunomodulating agent in the management of TEN and we have performed a brief review of evidence highlighting its role in TEN management.

Literature is limited regarding the medical and cosmetic dermatologic issues pertinent to transgender patients and the reasons why 19 transgender individuals seek care from dermatologists. Clinical management of this population has historically been limited to 20 mental health providers, endocrinologists, and select surgeons with expertise in sex reassignment surgery. The impact of hormonal 21 therapy on transgender skin has been well documented in endocrinology journals, but is underrepresented in dermatology 22 literature. Hormonal therapy leads to drastic skin alterations, impacting sebum production, hair growth, and acne, all of which may 23 become a dermatologic concern for the transgender patient. Dermatologists may also be consulted regarding issues such as 24 permanent hair removal, androgenic alopecia, or scar revision following breast reduction surgery or genital reassignment surgery. 25 The purpose of this review is to provide relevant information for use by all dermatology providers who care for transgender 26 patients or patients undergoing transition.

We report several scenarios of compromise in patient safety owing to the re-use of mis-assigned patient’s surgical instruments in Mohs micrographic surgery.

We discuss the breaks in universal protocols that others may experience in their practices and describe corrective measures that our institutions employed to avoid such future events.

There is a lack of publication in the literature on the topic of mis-assigned instrument use in Mohs surgery. We believe that the  practice of re-using instruments is cost-effective and therefore common. Based on our humbling experience, this publication may initiate important discussion among dermatologist regarding safety protocols at their respective institutions.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon intermediate-grade soft tissue sarcoma. The tumor is found preferentially on the trunk, whereas the head and neck region are affected in only 10% to 15% of cases. We report the case of a 44-year-old woman with a 5-month history of an asymptomatic, firm, yellow nodule in the mucosa of the right cheek measuring 2.5 cm. The clinical diagnosis was lipoma and an excisional biopsy was obtained. Histopathological analysis revealed a proliferation of spindle-shaped mesenchymal cells arranged in interlacing fascicles amidst fibrous stroma. Low mitotic activity (2 mitoses per field) and deep cell infiltration into adipose tissue were observed. These features led to the histopathological diagnosis of a malignant low-grade mesenchymal cell tumor. Immunohistochemical analysis revealed negative staining for α-SMA, S-100, and Bcl-2, but positive staining for Ki-67 (18%) and strong and diffuse staining for CD34. These findings were compatible with the diagnosis of DFSP. Only two cases of DFSP in the oral cavity, both involving the cheek mucosa, have been described so far, highlighting the rarity of the present case.

Background

CD30+ lymphoproliferative disorders are rare and may feature a wide variety of presentations that mimic other conditions.

Purpose

A man with metastatic papillary thyroid carcinoma to skin who subsequently developed cutaneous anaplastic large cell lymphoma is described.

Methods

The PubMed medical database was used to search the following terms separately and in combination: ALCL, anaplastic large cell lymphoma ALCL, cutaneous anaplastic large cell lymphoma CALCL, cutaneous t-cell lymphoma CTCL, large t-cell lymphoma LTCL, lymphoproliferative, lymphomatoid papulosis LyP, mimic, papillary, thyroid cancer.

Results

CD30+ cutaneous anaplastic large cell lymphoma was diagnosed in a man with metastatic papillary thyroid carcinoma based on the temporal, histologic, and immunochemical features of an enlarging lesion. To the best of our knowledge, this is the initial description of a CD30+ lymphoproliferative disorder occurring in a patient with primary carcinoma of the thyroid.

Conclusion

Cutaneous lesions may present with various morphologies. Our patient had a previous history of metastatic papillary thyroid carcinoma to skin. His new chest lesion was originally suspected to be either an infection or a cutaneous metastasis. Multiple biopsies, not only for microscopic evaluation but also cultures for infectious organisms, were performed. Unexpectedly, a CD30+ lymphoproliferative disorder was diagnosed; subsequently the tumor spontaneously resolved. Therefore, when skin lesions appear that have more than one clinical presentation, it may be prudent for the clinician to collect representative samples of each distinct morphology to assure that an accurate diagnosis is established.

Leukonychia refers to a white discoloration of the nails. Although several conditions may cause white nails, a rare, isolated, congenital form of the disease is hypothesized to stem from disordered keratinization of the nail plate. Herein, we report a case of a 41-year-old woman with congenital leukonychia and review prior cases.

Cutaneous Crohn disease (CD) affecting the vulva, perineum, and perianal skin, is a rare entity, which may accompany or precede gastrointestinal CD. Vulvar involvement, if untreated, may ultimately require extensive surgery including vulvectomy to gain control of the disease. Both gastrointestinal and cutaneous CD respond to biologics, which block TNF. In addition, ustekinumab, which targets both IL-12 and IL-23 cytokines, is effective in patients with gastrointestinal CD who fail TNF blockade. However, it is unclear if ustekinumab is effective for cutaneous CD. Herein we present a patient with cutaneous CD affecting the vulva and perianal skin, which, at seven months, had a marked response to ustekinumab administered at higher doses than typically used for psoriasis.

Superficial angiomyxoma is a distinctive cutaneous soft tissue neoplasm that is clinically variable, infrequent, and benign. However, this tumor has a high propensity for local recurrence. There is a known association of angiomyxomas with Carney complex. We report a case of superficial angiomyxoma in a 28-year-old woman who presented with an erythematous, mul-tilobulated nodule on the nipple. This tumor has a predilection for the trunk, head and neck, extremities, and genitalia. The present case, hence, is unusual, because the lesion developed on the nipple. The appearance of a solitary cutaneous angiomyx-oma warrants an examination to rule out the presence of Carney complex. After clinical examination, echocardiogram and en-docrine analyses, Carney complex was ruled out in our patient.

Tuberculosis affects one-third of the world’s population. The incidence of extrapulmonary tuberculosis including cutaneous and skeletal manifestations has increased in the last decades. The authors report a clinical case of concomitant metastatic cutaneous abscesses and Pott disease in an immunocompetent patient, a rare presentation of tuberculosis.

We report the case of a 44-year-old woman with a history of Crohn disease treated with infliximab who presented with erythematous papules and plaques on the upper extremities accompanied by fevers. She was subsequently diagnosed with palisaded neutrophilic and granulomatous dermatitis (PNGD). Whereas immune-complex mediated diseases such as rheumatoid arthritis and systemic lupus erythematosus are most commonly associated, inflammatory bowel disease deserves increased consideration as one of the systemic diseases that can present with PNGD. Additionally, PNGD should remain in the differential diagnosis of cutaneous eruptions that develop in the setting of tumor necrosis factor (TNF) antagonist therapy.

Glomus tumor is a rare benign vascular hamartoma derived from the modified smooth muscle cells of the glomus body. Paroxysmal pain is the leading symptom. A 78-year-old woman presented at our clinic with a 50-year history of a slow growing painful purple blue tender nodule under the right fourth finger nail. Dermoscopy and magnetic ressonance confirmed the presence of a large subungual tumor and total surgical excision was performed after partial nail plate avulsion. Histologic examination confirmed a glomus tumor. After 1 year of follow-up, the nail grew with normal shape and neither complications nor recurrence were observed.

Alternaria species are a type of dematiaceous fungi that cause a wide spectrum of disease with cutaneous infections being the most common. These infections are most common in immunocompromised individuals. However, only a few cases of primary cutaneous alternariosis have been reported in immunocompetent patients. We present a case of an otherwise healthy 13-year-old male who presented with primary cutaneous alternariosis on his left ankle that responded to the use of topical econazole and oral itraconazole. We also provide a review of previously described cases of primary cutaneous alternariosis in the literature.

Gout is the most common cause of inflammatory arthritis in men over 40 [1]. The prevalence of gout in the US is approximately 3.9%. Tophus is a characteristic sign of gout and results when monosodium urate deposits at the joint, skin, or cartilage. Tophi develop in 12-35% of patients who are diagnosed with gout [2]. We report a case of a 70-year-old man who was diagnosed with squamous cell carcinoma of the helix via shave biopsy. During Mohs micrographic surgery, a vigorous foreign-body reaction was noted. Review of the initial biopsy slides identified crystals with pseudoepitheliomatous hyperplasia (PEH) rendering a diagnosis of gout. This case emphasizes the importance of recognizing causes of PEH and the predilection for tophi to form on the ear.

Differential diagnoses of pigmented lesions of the nipple include melanocytic nevus, melanosis of the nipple, seborrheic keratosis, pigmented basal cell carcinoma, melanoma and Paget disease. The histologic exam with appropriate immunohistochemistry is a fundamental tool to achieve a correct diagnosis. We present a patient with a pigmented lesion of her right nipple revealing mammary Paget disease and elucidate diagnostic obstacles and prognostic importance of early breast cancer detection.

Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytosis, which commonly manifests as a solitary papule or nodule in infants and young children. However, other less common clinical presentations have been reported including clustered, agminated or plaque-like. It is important to recognize these variants, as diagnosis is often clinical. We highlight a case of juvenile xanthogranuloma presenting as nodules on the trunk of an infant that gradually coalesced to form a larger plaque.

Melanoma occurrence during treatment with anti-tumor necrosis factor is considered an incidental event, although very recent studies suggest a risk. Etanercept is a fusion protein that binds the tumor necrosis factor receptor and is included among TNF inhibitors, approved for the treatment of several autoimmune diseases, such as psoriasis.We described a 79-year-old man with psoriasis, being treated with etanercept, who presented with a new brown to black macule on his right shoulder; this was immediately surgically excised. Histology showed a superficial spreading melanoma, 1.2 mm Breslow thickness, one mitosis/hpf, with no vascular or neural invasion (stage T2b). Sentinel lymph node biopsy was negative. There were no apparent melanoma risk factors: normal total nevus count, photo type IV, no childhood sunburns, no family history of melanoma, and no previous immune suppressive drugs and/or phototherapies. Etanercept 50 mg/week had been administered continuously for 5 years before the melanoma occurrence. After etanercept withdrawal his psoriasis slowly, but progressively relapsed.

Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by a triad of relapsing or persistent orofacial edema, recurrent lower motor neuron facial nerve palsy and fissured tongue. Acute, painless, non-erythematosus orofacial edema is observed in all patients. We report a case of a 13-year-old girl who presented with a 2-year history of swelling of the upper lip, facial paralysis, and fissured tongue; she was treated successfully with dapsone.

Hypothyroidism is a common disease, and there may be a link between hypothyroidism and inflammatory skin disease. The purpose of this study is to assess whether hypothyroidism is more prevalent in psoriasis or rosacea patients. We utilized a large claims-based database to analyze rates of hypothyroidism in patients with psoriasis and rosacea compared to other patients with skin diseases. Participants were patients between 20-64 years of age with ICD-9 diagnosis codes for psoriasis, rosacea, and hypothyroidism. We found that rates of hypothyroidism in rosacea and psoriasis patients were similar to rates of hypothyroidism in those without rosacea or psoriasis.

We report a 41-year-old woman presented with a 4-year history of an exuberant, erythematous tumor, with necrotic appearance of the right distal toe. The histopathology examination showed a nodular-ulcerative melanoma, with bone infiltration. Widespread metastases including the right ovary, peritoneum, and right breast were present. The patient was misdiagnosed for almost 4 years as having onychomycosis, ingrown nail and pyogenic granuloma.

There have been reports of the use of silicone oils in injectable form for cosmetic purposes since the 1940s. With the popularization of the use of this technique in the mid-60s, there have been a wide range of adverse effects reported. We report a 32-year-old woman, who had intramuscular injections of an unknown substance, which led to serious complications.

Hyperhidrosis, or abnormally increased sweating, is a condition that may have a primary or secondary cause. Usually medication- induced secondary hyperhidrosis manifests with generalized, rather than focal sweating. We report a 32-year-old woman with a history of palmoplantar hyperhidrosis for 15 years who presented for treatment and was prescribed oral glycopyrrolate. One month later, the palmoplantar hyperhidrosis had resolved, but she developed new persistent craniofacial sweating. After an unsuccessful trial of clonidine, oxybutynin resolved the craniofacial hyperhidrosis. To our knowledge, this is the first case of compensatory hyperhidrosis secondary to glycopyrrolate reported in the literature. The case highlights the importance of reviewing medication changes that correlate with new onset or changing hyperhidrosis. It also demonstrates a rare drug adverse effect with successful treatment.

The University of California Davis, School of Medicine, Department of Dermatology is recruiting for a full-time position at the Associate or Full Professor level in the Clinical X Series or Health Sciences Clinical Professor (HSCP) Series. The successful candidate is nominated to be the holder of the Frederick G. Novy, Jr. M.D Endowed Professorship. Appointees to the ClinX series are expected to conduct independent research. Both series require significant participation in teaching and University/public service.The holder of the Frederick G. Novy, Jr. M.D. Endowed Professorship will be a nationally recognized scholar and clinician whose role would emphasize clinical education and, potentially, clinical research. A history of lecturing at national forums would be expected of the Novy Professorship holder. He /She will serve in a leadership capacity for educational issues in the department including a strong involvement with the dermatology residency program and medical student curriculum in the area of dermatology.Administrative responsibility within the Dermatology Clinic, which sees over 32,000 patient visits a year, is possible, based on departmental need. The Novy Professor will have oversight of the Volunteer Clinical Faculty Program, Grand Rounds, Novy Lecture Series, and community interactions. This person would undertake clinical, educational, scholarly, and publication commitments and would play an active role in the Department, the Medical School, UC Davis Health System, and the community.Required candidate qualifications include: an M.D. or D.O. degree, board certification or eligibility in Dermatology, eligibility for medical licensure in the state of California, an established track record of original research publications, and a record of teaching and patient care in the area of skin diseases. Demonstrated leadership experience and abilities, the ability to adhere to policies and procedures are required. The ability to work cooperatively and collegially in a diverse environment and credentials sufficient to be appointed to the full or associate level in the Professor of Clinical Dermatology series and/or Health Sciences Professor series. Individuals with an academic interest and background in clinical education and/or research is required.This recruitment is conducted at the Associate/Full Professor rank. The resulting hire will be at the Associate/Full Professor rank.For full consideration applications must be received by September 15, 2016, the position will remain open until filled through January 30, 2017. The following information is required: current curriculum vitae, cover letter and 3-5 references (contact information only) and Statement of Contributions of Diversity. Please visit http://academicaffairs.ucdavis.edu/diversity/equity_inclusion/index.html for information about why diversity statements are requested and guidelines for writing a diversity statement.Please apply online line to: https://recruit.ucdavis.edu/apply/JPF01087The University of California, Davis is an affirmative action/equal opportunity employer with a strong institution commitment to the achievement of diversity amount its faculty, staff and students.