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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 22, Issue 5, 2016

Commentary

Hormones and clocks: do they disrupt the locks? Fluctuating estrogen levels during menopausal transition may influence clock genes and trigger chronic telogen effluvium

Chronic telogen effluvium describes the clinical condition noted mostly in middle-aged women of increased, diffuse scalp hair shedding that is prolonged and often presents with a fluctuating course that may continue for years but does not lead to visible hair thinning. Despite its description almost 20 years ago, the underlying pathologic cause of CTE is yet to be identified. However the culmination of research in the field of hair biology and the burgeoning field of chronobiology may lead to exciting breakthroughs in our understanding of CTE. In this paper the current literature on CTE is reviewed and a hypothesis is put forth that CTE may be triggered by hormonal fluctuations and alterations in circadian control genes.

Original

Tumor stage mycosis fungoides: a single-center study on clinicopathologic features, treatments, and patient outcome

Background: Tumor stage mycosis fungoides (MF) is a subtype of cutaneous T-cell lymphoma (CTCL). Tumor stage MF is rarely curable. Treatment is aimed towards controlling the disease and minimizing side effects from therapy.

Objective: To characterize clinicopathologic features of tumor stage MF and the impact of clinical characteristics and treatment modalities on patient outcome.

Methods: A retrospective chart review was conducted on 39 patients with tumor stage MF followed at Vanderbilt University between July 1995 and July 2010.

Results: The median age of diagnosis was 61 years (IQR: 54-70). Sixty-nine percent of the patients were male (27/39). The median follow-up time was 13.6 months (IQR: 5.5-35.9). Among the patients younger than 60 years at the time of initial diagnosis (n = 19), median overall survival (OS) was 7.0 years (95% CI: 2.1-17.9), compared with 3.3 years (95% CI: 2.4-9.3) in patients who were 60 years or older at initial diagnosis. Ten patients with T1/T2 stage at diagnosis had median OS of 5.0 years (95% CI 3.2-7.0). Twenty-eight patients with T3 stage at diagnosis had median OS of 5.8 years (95% CI 2.4-14.2). Median OS for patients with large cell transformation (LCT) and without LCT was 3.3 and 7.7 years, respectively.

Limitations: This is a retrospective study with the bias of a tertiary-care referral center.

Conclusion: Although LCT and older age at diagnosis were not statistically significant negative prognostic indicators of OS, there was a trend towards statistical significance for LCT. Clinical stage at diagnosis may not affect OS in patients who develop tumor stage MF

Case Report

Goodness, gracious, great balls of fire: A case of transient lingual papillitis following consumption of an Atomic Fireball

Transient lingual papillitis is a benign condition characterized by the inflammation of one or more fungiform papillae on the dorsolateral tongue. Although it is a common condition that affects more than half of the population, few cases have been reported in the dermatological literature. Therefore, it is a condition uncommonly recognized by dermatologists though it has a distinct clinical presentation that may be easily diagnosed by clinicians familiar with the entity. We report an interesting case of transient lingual papillitis in a 27 year-old healthy woman following the consumption of the hard candy, Atomic Fireball. We describe treatment and resolution of the condition, and its recurrence following re-exposure to the identified culprit. This report further reviews the literature to illustrate the clinical manifestations, etiology, differential diagnosis, course, and treatment of this condition.

Lhermitte-Duclos Disease in association with Cowden Syndrome

Cowden syndrome (CS) is a rare genetic disorder with autosomal dominant inheritance, linked to germline mutations in the PTEN tumor suppressor gene on chromosome 10. Cowden syndrome often co-exists with Lhermitte–Duclos disease (LDD), with LDD included as a major criterion in CD diagnosis. This case involves a woman presenting with many of the classic diagnostic criterions and associations of CD, as well as with several comorbidities and unique objective findings.

Case Presentation

Intravascular papillary endothelial hyperplasia of the vulva: report of a patient with Masson tumor of the vulva and literature review

Background: Intravascular papillary endothelial hyperplasia -- also known as Masson tumor -- is a rare, benign vascular condition that manifests on the skin as a firm, blue-black colored nodule or papule. Lesions range in size from 0.25 to 5 centimeters in diameter and may be tender or painless. In some individuals, nodules appear red colored, mimicking hemangioma or pyogenic granuloma. Histologically, intravascular papillary endothelial hyperplasia is characterized by the presence of an organizing thrombus in the vascular lumen with accompanying hyperplastic endothelial cell proliferation. Common sites of presentation include the head, neck, and extremities. However, albeit rarely, lesions may also appear in the genital region.

Purpose: We describe the clinical and pathologic findings of a woman who developed intravascular papillary endothelial hyperplasia of the vulva. We also review the characteristics of other patients with intravascular papillary endothelial hyperplasia of the vulva and summarize the differential diagnosis and treatment options for this condition.

Materials and methods: The features of a woman with intravascular papillary endothelial hyperplasia of the vulva are presented. Using PubMed, the following terms were searched and relevant citations assessed: intravascular papillary endothelial hyperplasia, IPEH, labia majora, Masson hemangioma, Masson pseudoangiosarcoma, Masson tumor, and vulva. In addition, the literature on intravascular papillary endothelial hyperplasia is reviewed.

Results: A 32-year-old woman presented with a 3 x 2 millimeter painless, black colored submucosal papule on her left labia majora. The lesion was removed by excisional biopsy. Microscopic examination revealed a re-canalizing thrombus and a proliferation of erythrocytes within a dilated vascular structure. Based on correlation of the clinical presentation and histopathologic findings, a diagnosis of intravascular papillary endothelial hyperplasia was established. The patient applied mupirocin 2% ointment to the biopsy site, which subsequently healed without complication or recurrence.

Conclusion: Intravascular papillary endothelial hyperplasia -- also known as Masson tumor -- is a rare, benign vascular eruption. Cutaneous lesions typically present as red colored or blue-black colored nodules ranging in size from 0.25 to 5 centimeters in diameter. The most common sites of presentation include the head, neck, and extremities. However, albeit rarely, lesions may also appear in the genital region. The histologic hallmark of intravascular papillary endothelial hyperplasia is an organizing thrombus in the vascular lumen with associated hyperplastic endothelial cell proliferation. Lesions often appears similar to other neoplastic and non-neoplastic cutaneous tumors, including hemangiomas, pyogenic granulomas, and cutaneous angiosarcomas. Therefore, pathologic examination is required to confirm the suspected diagnosis and exclude malignancy. The condition can usually be treated with simple local excision.

Plasmablastic lymphoma: an atypical cutaneous presentation of a rare entity

Plasmablastic lymphoma is a very rare B-cell lymphoma typically associated with immunosuppression: It occurs primarily in the oral cavity, although some cases were reported in other organs and tissues.To date, only 10 cases of primary cutaneous plasmablastic lymphoma have been described. Clinically, primary cutaneous plasmablastic lymphoma presents as non-specific cutaneous lesions (purple nodules, erythematous infiltrated plaques). In previously described cases, as in this case, histology and immunohistochemistry are required to make the diagnosis. Owing to the rarity of this entity, there is no established therapy, which makes its management an individualized, patient-based decision.

Successful treatment of pityriasis lichenoides chronica with narrow-band ultraviolet B therapy in a patient with Keratitis-Ichthyosis-Deafness syndrome: a case report

Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis causing ichthyosis-like skin lesions, keratitis, and deafness. Herein, we report a patient with this rare syndrome in association with pityriasis lichenoides chronica, which was succesfully treated with narrow-band ultraviolet B phototherapy despite our concerns regarding the increased risk of squamous cell carcinoma, hyperpyrexia, and keratitis.

Pigmented hidrocystoma of nasal epithelium (PHONE): report of a man with a pigmented hidrocystoma of his nose and literature review

Background: Hidrocystomas are benign tumors of apocrine or eccrine epithelium. They most commonly occur on the head and neck, especially periorbitally. Albeit rare, these adnexal tumors may present as pigmented lesions.

Purpose: To describe a patient with a pigmented eccrine hidrocystoma of his nose and to review the features of other individuals with pigmented hidrocystoma of the nasal epithelium.

Material and Methods: PubMed was used to search the follow terms: hidrocystoma and pigmented. All papers were reviewed and relevant manuscripts, along with their reference citations were evaluated.

Results: A 52-year-old man who presented with a pigmented eccrine hidrocystoma on his nasal bridge was described. The features of three previously described patients with pigmented hidrocystoma of the nose were evaluated. The tumors presented as single or multiple, less than 2mm, blue papules. Our patient’s tumor would intermittently bleed, which prompted consideration of a possible basal cell carcinoma. Biopsy established the diagnosis showing a cystic lesion lined by eccrine epithelium with pigmented secretion within the cyst’s lumen. The cyst content stained positive with Fontana-Masson stain. Our patient’s excisional biopsy resulted in excellent cosmetic appearance and complete removal of the benign adnexal tumor.

Conclusion: Pigmented hidrocystomas may be mistaken for other skin lesions, such as a pigmented basal cell carcinoma and melanoma. A biopsy readily establishes the diagnosis. We respectfully suggest that a hidrocystoma located on the nose that is pigmented be referred to as a PHONE: pigmented hidrocystoma of the nasal epithelium.

Milia-like idiopathic calcinosis cutis in a child with Down syndrome

Idiopathic calcinosis cutis refers to progressive deposition of crystals of calcium phosphate in the skin and other areas of the body, in the absence of any inciting factor. Idiopathic calcinosis cutis may sometimes take the form of small, milia-like lesions. Most commonly, such milia like lesions are seen in the setting of Down syndrome. Herein, we report a 5-year-old girl with multiple asymptomatic discrete milia-like firm papules distributed over the face and extremities. A diagnosis of milia-like idiopathic calcinosis cutis associated with Down Syndrome was provisionally made and was confirmed by histopathology and karyotyping.

Cutaneous Richter Syndrome mimicking a lower limb cellulitis infection - a case report and review of the literature

Richter syndrome (RS) is characterized by the development of a high-grade lymphoma in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Herein, we present the case of an 85-year-old woman with a 3-year history of stable asymptomatic CLL that developed a cutaneous RS. The patient presented with painless inflammation in the left leg and foot that was initially diagnosed as a cellulitis infection. She was treated accordingly with ceftriaxone and clindamycin. However, after completing the antibiotic regimen, not only did the inflammation persist, but also superimposed painless nodules gradually appeared on the left leg and foot over the course of four months. The histopathological examination of the nodules revealed a large B-cell cutaneous lymphoma. The patient underwent chemotherapy with CVP, followed by R-CHOP, resulting in a reduction of size of the nodules and remission of the inflammation. The patient died five months after the diagnosis owing to a bacterial pneumonia. We identified in previous reports a total of fifteen cases of cutaneous RS. Most cases presented with rapidly growing tumors or multiple erythematous nodules, similar to our case. This case of a cutaneous RS mimicking a cellulitis infection underlines the importance of a low threshold for performing biopsies of suspicious skin lesions in patients with CLL/SLL.

Photo Vignette

Criptococosis cutánea primaria en paciente inmunocompetente

La criptococosis cutánea es una micosis propia de pacientes inmunodeprimidos, sobre todo aquellos con infección por el virusde la inmunodeficiencia humana (VIH). Sin embargo, existen casos infrecuentes de criptococosis cutánea en pacientes inmunocompetentes, que suelen simular otras dermatosis, lo que retrasa su diagnóstico y tratamiento. Presentamos el caso de un varón pluripatológico de 79 años, con úlceras dolorosas en dorso de mano derecha que no respondían a tratamientos tópicos. A través del estudio histopatológico y micológico se alcanzó el diagnóstico de criptococosis cutánea primaria, lográndose la remisión de las lesiones tras 6 meses de tratamiento con fluconazol.

Penicillamine-associated cutis laxa and milia en plaque - case report and review of cutaneous changes associated with penicillamine

Penicillamine-induced skin changes are rare and include: hypersensitivity reactions, autoimmune reactions, and cutaneous elastoses. We report a case of a 73-year-old man with cystinuria taking penicillamine for over 50 years who presented with penicillamine-induced cutis laxa and milia en plaque. A brief review of penicillamine induced skin changes, specifically cutis laxa and milia en plaque, is presented.

Infantile myofibroma: a firm, round plaque in an infant

Infantile myofibroma is a rare fibromatous tumor that is variable in presentation and is frequently mistaken for hemangioma or rhabdomyosarcoma. We describe a 14-month-old boy who presented with multiple, enlarging, firm lesions on the shoulder. Biopsy revealed a proliferation of small spindle cells with myxoid and hyalinized stroma infiltrating into the superficial adipose tissue. We provide a brief review of the clinical presentation, histopathologic features, management, and recent advances in our understanding of this rare condition.

Letter

Micosis fungoide e inhibidores del TNFα: ¿riesgo o beneficio?

The growing use of anti-TNF drugs during the last years has reopened the discussion about the possible increased risk of developing non-Hodgkin lymphoma in patients with such type of treatments. We present our clinical experience and critical opinion about the current situation of such issue regarding cutaneous T-cell lymphomas.El creciente uso de fármacos anti-TNF durante los últimos años ha reabierto el debate sobre el posible aumento de riesgo de linfomas no Hodgkin en los pacientes con este tipo de tratamientos. Presentamos nuestra experiencia clínica y opinión critica sobre la situación actual de este tema en relación a los linfomas cutáneos de células T.

Hypotrichosis with juvenile macular dystrophy: Portuguese case

Hypotrichosis with juvenile macular dystrophy is a rare congenital disease mainly found in the Druze population of Northern Israel. This disorder is caused by the CDH3 mutation encoding P-cadherin, which is expressed in retinal pigment epithelium and hair follicles. An 11-year-old girl who was born to related Portuguese parents, had hypotrichosis since birth and macular dystrophy diagnosed at age 5. Fundus examination and fluorescein angiography revealed located macular pigmentary abnormalities. No molecular analysis was done. A fundus examination should be considered mandatory in the assessment of congenital hypotrichosis.

Increasing adherence to topical therapy in psoriasis through use of solution medication

Patient outcomes and clinical improvement are closely related to topical medication adherence, and is especially important in chronic dermatological diseases such as psoriasis. About one-fifth of patients undergoing topical treatment were dissatisfied with its convenience for various reasons. Providers can help increase adherence through selecting the correct medication vehicle, involving family members or friends in the patient’s mediation application, and explaining likely side effects to the patient prior to use of the medication. Increased inherence will lead to better psoriasis disease control.

Angioqueratomas de fordyce múltiplos de vulva associados à cirrose hepática

Fordyce angiokeratoma is a benign skin neoplasm, characterized by erythematous-violet keratotic papules with mamillated surface, which follows a chronic and minimally symptomatic course. However, symptoms can be related such as itching, burning, bleeding, dyspareunia; aesthetic and social concerns may prompt request for treatment. The condition mainly affects men and usually occurs in the scrotum, but rarely may occur in women. A 30-year-old woman with liver cirrhosis and portal hypertension is described. Over a period of two years she developed multiple angiokeratomas of the vulva, complicated by frequent bleeding. Histopathologic analysis of the lesion confirmed the diagnosis of angiokeratoma and all lesions were surgically removed. The results of the treatment were very satisfactory, with no relapses or complications.

Angioqueratoma de Fordyce é uma dermatose incomum que compõe o grupo das neoplasias cutâneas benignas, caracterizando-se pelo aparecimento de lesões papuloceratósicas, eritemato-violáceas de superfície mamilonada, que apresentam curso crônico e oligossintomático. No entanto, outros sintomas podem estar associados, como prurido, ardência, sangramento e dispareunia, além de problemas estéticos e de ordem social. Afeta principalmente homens, acometendo a região escrotal, sendo raro seu surgimento em mulheres. Relata-se caso de uma paciente com 30 anos de idade, portadora de cirrose hepática e hipertensão portal evoluindo há 2 anos com múltiplos angioqueratomas na vulva de sangramento frequente. A análise histopatológica confirmou o diagnóstico de angioqueratoma e a paciente foi submetida à exérese cirúrgica das lesões. Os resultados terapêuticos foram satisfatórios, sem recidivas ou complicações.