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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 20, Issue 5, 2014

Review

A Review of phosphodiesterase-inhibition and the potential role for phosphodiesterase 4-inhibitors in clinical dermatology

Background: Phosphodiesterase inhibitors are commonly used drugs.  Specific phosphodiesterase inhibitors with anti-inflammatory properties are being assessed as dermatological treatments.

Purpose:  To describe important aspects of phosphodiesterase inhibition and the safety and efficacy of 2 phosphodiesterase- 4 inhibitors being studied for the treatment of dermatologic diseases

Methods: We did a non-systematic analysis of literature on phosphodiesterase inhibition followed by a review of published information on apremilast and topical AN2728 and their use for psoriasis and atopic dermatitis.

Findings: Apremilast and topical AN2728 have modest efficacy in treatment of psoriasis. Apremilast achieved PASI-75 scores ranging from 24-33%. In phase 2 studies, AN2728 had modest efficacy for psoriasis (40% of patients achieved a ≥ 2 grade improvement as assessed by the Overall target Plaque Severity Score).   In phase 2 studies of AN2728 use in atopic dermatitis, subjects achieved a 71% improvement from baseline Atopic Dermatitis Severity Index. In all studies, most adverse effects were minimal. The limitations of this paper are the limited number of published studies, the lack of long-term data, and the lack of head -to - head trials directly comparing phosphodiesterase inhibitors with other treatments.

Conclusion: Phosphodiesterase inhibitors constitute a widely used class of drugs that may see growing use for inflammatory dermatologic diseases.

Dissecting cellulitis (Perifolliculitis Capitis Abscedens et Suffodiens): a comprehensive review focusing on new treatments and findings of the last decade with commentary comparing the therapies and causes of dissecting cellulitis to hidradenitis suppura

Dissecting cellulitis (DC) also referred to as to as perifolliculitis capitis abscedens et suffodiens (Hoffman) manifests with perifollicular pustules, nodules, abscesses and sinuses that evolve into scarring alopecia. In the U.S., it predominantly occurs in African American men between 20-40 years of age.  DC also occurs in other races and women more rarely.  DC has been reported worldwide.  Older therapies reported effective include: low dose oral zinc, isotretinoin, minocycline, sulfa drugs, tetracycline, prednisone, intralesional triamcinolone, incision and drainage, dapsone, antiandrogens (in women), topical clindamycin, topical isotretinoin, X-ray epilation and ablation, ablative C02 lasers, hair removal lasers (800nm and 694nm), and surgical excision.  Newer treatments reported include tumor necrosis factor blockers (TNFB), quinolones, macrolide antibiotics, rifampin, alitretinoin, metronidazole, and high dose zinc sulphate (135-220 mg TID).  Isotretinoin seems to provide the best chance at remission, but the number of reports is small, dosing schedules variable, and the long term follow up beyond a year is negligible; treatment failures have been reported.  TNFB can succeed when isotretinoin fails, either as monotherapy, or as a bridge to aggressive surgical treatment, but long term data is lacking.  Non-medical therapies noted in the last decade include: the 1064 nm laser, ALA-PDT, and modern external beam radiation therapy.  Studies that span more than 1 year are lacking. Newer pathologic hair findings include: pigmented casts, black dots, and "3D" yellow dots.  Newer associations include: keratitis-ichthyosis-deafness syndrome, Crohn disease and pyoderma gangrenosum.  Older associations include arthritis and keratitis.  DC is likely a reaction pattern, as is shown by its varied therapeutic successes and failures.  The etiology of DC remains enigmatic and DC is distinct from hidradenitis suppurativa, which is shown by their varied responses to therapies and their histologic differences. Like HS, DC likely involves both follicular dysfunction and an aberrant cutaneous immune response to commensal bacteria, such as coagulase negative staphylococci. The incidence of DC is likely under-reported. The literature suggests that now most cases of DC can be treated effectively.  However, the lack of clinical studies regarding DC prevents full understanding of the disease and limits the ability to define a consensus treatment algorithm.

Original

Common reasons why acne patients call the office

Background: Communication between physicians and patients is essential to providing proper medical care. At times, patients leave visits with insufficiently addressed questions. These questions prompt patients to call the clinic for additional information, which disrupts the flow of care, delays proper treatment, and reduces patient satisfaction.

Purpose: We aim to examine acne patients’ post-visit questions to develop interventions to improve patient education and reduce call backs.

Methods: A retrospective electronic medical record chart review was performed involving Wake Forest Baptist Health Dermatology clinic visits between October 1, 2012 and October 31, 2012. We identified acne patients using clinic visit notes and recorded their telephone calls to the clinic between October 1, 2012 and March 29, 2013.

Results: Of 315 acne patients, 31 (9.8%) called the clinic. Isotretinoin was the subject of 66.7% of the calls, half of which involved questions about potential side effects. Other calls addressed topical medications, acne symptoms, and pharmacy requests.

Limitations: The study involved one center and email and fax correspondence was not captured.

Conclusions: We found gaps in communication sufficient to require patients to call in for support, specifically regarding oral isotretinoin treatment. Interventions to address these questions have the potential to improve quality of care.

Case Report

Multinucleate cell angiohistiocytoma: a case report and review of the literature.

Multinucleate cell angiohistiocytoma (MCAH) is a rare, benign vascular proliferation.  Fewer than 80 cases have been reported to date, which may relate to under-recognition of this entity. Lesions are commonly asymptomatic and appear as erythematous to violaceous papules on the lower extremities and dorsal hands of middle-aged to elderly women. The characteristic histopathologic and immunohistochemical features of MCAH are essential for definitive diagnosis of MCAH. Multinucleate cell angiohistiocytoma follows a slowly progressive course, although spontaneous regression has been reported in rare cases. We present a case of MCAH to increase awareness and elucidate the characteristic clinical and histopathologic features of this disorder.

Cutis laxa adquirido asociado a gammapatía monoclonal y enfermedad por depósito de cadenas ligeras lambda

Cutis laxa is a rare entity characterized clinically by redundant skin that gives an appearance of premature aging. The appearance relates to a loss of elasticity because of the destruction of elastic fibers that affects the skin and other organs. It may be associated with inflammatory conditions or diseases, such as plasma cell dyscrasias. We report the case of a 54-year-old man with acquired cutis laxa, which preceded the development of IgG-lambda monoclonal gammopathy with lambda light chain deposits in the kidney. The patient had a fatal outcome owing to severe and rapidly progressive renal failure. We emphasize the importance of recognizing a plasma cell dyscrasia in a patient with cutis laxa, although this association is rare.

Case Presentation

Multiple clustered and focally atrophic dermatofibromas (DF)

Multiple clustered dermatofibromas describes a confluence of dermatofibromas in one anatomic location. We describe a 32-year-old man who presented for evaluation owing to skin papules and plaques and concerns about malignancy. Repeat histopathological evaluation found no evidence for dermatofibrosarcoma protuberans. Our case presents this relatively rare condition and discusses observation along with potential treatment options.

Cetirizine-induced Acute Generalized Exanthematous Pustulosis: a serious reaction to a commonly used drug

Acute generalized exanthematous pustulosis (AGEP) is an abrupt cutaneous adverse reaction usually in response to medications. It is generally a self-limiting disease if diagnosed promptly and the offending agent discontinued. Cetirizine, a commonly used anti-histamine medication for the treatment of allergic diseases has few reported side effects and is normally well-tolerated and effective. Herein, the first reported case of cetirizine induced AGEP is presented, followed by a discussion of the clinical and pathological aspects of this adverse cutaneous reaction to a widely used drug. Awareness of this reaction is vital owing to the extensive use of cetirizine and the importance of drug cessation once the reaction is identified. Lastly, other pustular cutaneous reactions may present similarly and therefore accurate identification of this disease can prevent unnecessary diagnostic testing.

Regression of cutaneous invasive squamous cell carcinoma in a patient with chronic cutaneous graft versus host disease

Numerous complications can be observed in the post-transplant period among recipients of hematopoietic stem cells including graft-versus-host disease (GVHD), which is associated with significant morbidity and mortality. On the other hand, graft versus tumor (GVT) effect is a well-described phenomenon in patients with hematologic malignancies and has also been reported in renal cell cancer, ovarian cancer, breast carcinoma, and melanoma. We describe spontaneous regression of a cutaneous invasive squamous cell carcinoma and multifocal atypical intraepidermal proliferations in a patient with chronic graft-versus-host disease following initiation of extracorporeal photopheresis (ECP). This observation raises questions regarding the GVT in cutaneous neoplasms and potential immunomodulatory effects of ECP.

Adenocarcinoma de colo uterino com disseminação linfática apresentando-se como carcinoma en cuirasse da vulva: relato de caso

We report the case of a patient with carcinoma “en cuirasse” of the vulva.

Case report:  A female patient presented complaining of inguinal lymphadenopathy. Lymph node excision, immunohistochemistry analyses, and further exams showed the presence of cervical adenocarcinoma. The cancer was surgically removed and the patient was treated with radiotherapy and chemotherapy with a good initial response. Some months later she presented with intense edema of the lower limbs, hardening and thickening of the labia majora, and pelvic and genital ulceration. A cutaneous biopsy with subsequent immunohistochemical staining showed lymphatic dissemination of adenocarcinoma to the vulva.

Discussion:  Carcinoma “en cuirasse” is a rare presentation of cutaneous metastasis in which the affected skin shows hardening and induration, acquiring a sclerodermoid appearance.  This is, to the best of our knowledge, the first report in Brazil of  carcinoma “en cuirasse” of the vulva associated with cervical adenocarcinoma.

Disseminated cutaneous mycobacterium avium complex in a person with AIDS

A cutaneous Mycobacterium avium complex (MAC) infection is a rare infection that can occur through direct inoculation or via dissemination to the skin.  We report an interesting case of disseminated cutaneous MAC infection in a 45-year-old man with acquired immunodeficiency syndrome (AIDS), currently treated with highly active antiretroviral therapy (HAART) and MAC prophylaxis.

Improvement in severe psoriasis associated with isoniazid treatment

A 67 year-old man was diagnosed 10 years ago with severe plaque psoriasis and was hospitalized for re-evaluation of therapy. He was treated previously with topical corticosteroids, methotrexate, and phototherapy. As part of the assessment protocol for initiation of biological treatment, a tuberculin skin test was done, which was positive without signs of active tuberculosis. Isoniazid therapy was started with marked improvement of psoriatic lesions before biologic treatment could be initiated.

Photo Vignette

Liquen plano pigmentoso sobreimpuesto

Lichen planus pigmentosus is an uncommon variant of lichen planus that is characterized by the insidious onset of dark brown macules in sun-exposed areas and flexural folds.

Superimposed linear lichen planus is an exceedingly rare disorder, but it has been found in both lichen planopilaris and lichen planus types. A 39-year-old woman is presented showing a segmental and linear lichen planus associated with non-segmental lesions meeting all criteria for the diagnosis of superimposed linear planus pigmentosus. The segmental lesions were always more pronounced.

Cervicofacial actinomycosis: a long forgotten infectious complication of immunosuppression - report of a case and review of the literature

Actinomycosis is a rare chronic granulomatous infection caused by Gram-positive, non-acid-fast, anaerobic to microaerophilic bacteria.We report a case of cervicofacial actinomycosis in an 86-year-old woman undergoing immunosuppressive therapy with azathioprine and prednisone for rheumatoid arthritis. She underwent a dental treatment several months earlier. The diagnosis of culture-negative actinomycosis was based on histolopathology findings and the isolation of companion bacteria. The patient was treated with amoxicillin-clavulanic acid for 3 months, which produced complete clearance of her cervicofacial actinomycosis.Our case points out the pitfalls of diagnostic procedures in actinomycosis and the ability of this rare disease to mimic other medical conditions.

Omphalolith: a rare entity but important to recognize

Omphalolith (umbolith) is an uncommon entity under normal circumstances. However, in a deeply retracted umbilicus in an obese individual, the accumulation of sebum and keratin may lead to the formation of a stone. This calculus may remain undiagnosed for many years until revealed by secondary infection or ulceration. We report an interesting case of an omphalolith in a young woman.  In our literature search, very few cases of omphalolith have been reported.

Letter

Dermatology on Tumblr

Tumblr broke into the social media scene in 2007 as a micro-blogging platform that hosts 169 million blogs, 75 billion total posts, and 111 million posts daily to date. This study aimed to determine the presence of dermatology-focused journals, organizations, and groups on Tumblr. These were entered into the Tumblr search query to identify affiliated Tumblr blogs and ‘dermatology Tumblr’ was searched on Google to determine the first four distinct results that were active within the last six months. None of the dermatology-focused journals, organizations, or groups maintained a Tumblr blog and three of the first four blogs on Google were maintained by private practices. In conclusion, Tumblr remains a social media domain that lacks a strong presence from dermatology journals and organizations, remaining an untapped resource for information dissemination and interaction with the public.

Nodular goiter with thyroid antibodies in chronic idiopathic urticaria

We report a case of chronic idiopathic urticaria associated with nodular goiter and Graves disease. The urticaria resolved with normalization of the thyroid function.

Citation classics and top-cited authors of psoriasis in five high-impact general medical journals, 1970-2012

Psoriasis is a relevant topic for publication in general medical journals. We conducted a search of the Thomson Reuters’ Science Citation using the search term of “psoriasis” in five high-impact general medical journals. All citation classics from 1970 to 2012 were included and each author’s total number of citations was summated. A total of 51 citation classics were collected. The most common topic of publication was psoriasis treatment (37), especially biologic agents (16). A total of 1037 authors of psoriasis articles were identified in our study and the top 25 most-cited authors were compiled. We hope our citation analysis provides a historical perspective and highlights the work of our colleagues and predecessors.

Bilateral suborbital rash: A dermatologic manifestation of neuropsychiatric disease in a pediatric patient

A 10-year-old girl presented with a new onset bilateral suborbital rash. Dermatologic examination revealed violaceous, non-tender, well-demarcated patches with an atypical distribution and pigmentation. After further investigation, a diagnosis of Munchausen syndrome was made and the patient was referred to her primary care provider for further management.