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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 9, 2014

Case Report

Toxic epidermal necrolysis due to voriconazole: case report and review

Toxic epidermal necrolysis is an uncommon but potentially life-threatening adverse cutaneous drug reaction characterized by variable degrees of epidermal necrosis and detachment leading to morbidity and risk of mortality. We describe a 67-year-old woman who underwent allogeneic peripheral blood stem cell transplantation as treatment for chronic lymphocytic leukemia. She developed toxic epidermal necrolysis after she was transitioned to voriconazole, which was a component of her post-transplant regimen. The diagnosis of toxic epidermal necrolysis in our patient was made clinically and confirmed histologically. Based on the temporal initiation of voriconazole therapy and the development of her adverse cutaneous reaction, we concluded that voriconazole was the offending agent. There are limited reported cases of voriconazole-induced toxic epidermal necrolysis; we report this case to increase awareness of this potential life-threatening complication.

Acitretin for the management of generalized cutaneous lichen planus

Background: Lichen planus is an inflammatory disease that affects the skin, the oral mucosa, or both.  Generalized cutaneous lichen planus may pose a therapeutic challenge for clinicians if the condition persists or flares after topical or systemic corticosteroid therapy.

Purpose: Acitretin, a systemic retinoid, can be considered a potential second-line treatment for patients with generalized cutaneous lichen planus.  Herein, we describe a postmenopausal woman with generalized cutaneous lichen planus who was successfully treated with acitretin.

Methods: A 58-year-old woman presented with generalized cutaneous lichen planus involving her upper and lower extremities as well as her lower back.  After failing corticosteroid therapy, she was started on acitretin 20 mg/day, which was later increased to 30 mg/day.  To review the literature on the use of acitretin in cutaneous lichen planus, we used the PubMed search engine and searched for the terms “acitretin” and “cutaneous lichen planus.”

Results: Our patient had complete resolution of pruritus within one week of initiating acitretin 20 mg/day.  After an increase in dose to 30 mg/day, the cutaneous lesions completely resolved over a 3-month period.  There was no recurrence of disease as acitretin was tapered and discontinued.

Conclusion: Generalized cutaneous lichen planus may pose a therapeutic challenge for the symptomatic relief of skin lesions.  Topical and systemic corticosteroids are first-line treatments.  In patients who fail corticosteroids, relapse after corticosteroid therapy, or have contraindications to corticosteroids, acitretin may be considered a potential second-line therapy.

Metastatic laryngeal carcinoma mimicking eruptive keratoacanthomas: report of keratoacanthoma-like cutaneous metastases in a radiation port

Metastatic skin lesions from a primary squamous cell carcinoma of the head and neck have only been reported in 1%–2% of these patients. Hence, skin metastases from laryngeal carcinoma are uncommon. Also, cutaneous metastases clinically presenting as a keratoacanthoma are rare. We describe cutaneous metastases in a radiation port clinically mimicking eruptive keratoacanthomas. Using the PubMed database, an extensive literature search was performed using the keywords cancer, carcinoma, keratoacanthoma, laryngeal, metastases, metastasis, metastatic, mimicking, port, radiation, radiotherapy, radiation, skin, visceral. We were able to summarize the features of patients with keratoacanthoma-like cutaneous metastases and discuss radiation port cutaneous metastases. Cutaneous metastases can be the initial manifestation of a previously undiagnosed malignancy or can present in a patient with an established diagnosis of cancer. Our patient not only developed skin metastases that mimicked eruptive keratoacanthomas, but to the best of our knowledge, is the first individual to develop radiation port cutaneous metastasis from a primary laryngeal carcinoma. The development of cutaneous metastases in an area of skin that has been treated with radiation therapy may result from the treatment altering and/or injuring the site, thereby making it more susceptible to another condition, such as metastatic skin tumors. In patients with an established diagnosis of visceral malignancy, the appearance of new keratoacanthoma-like lesions should prompt the clinician to consider a biopsy in order to establish or exclude the possibility of a cutaneous metastasis.

Oral lichen sclerosus expressing extracellular matrix proteins and IgG4-positive plasma cells

Lichen sclerosus (LS) is a mucocutaneous disease with uncommon oral involvement. The etiology is not yet well understood, but LS has been associated with autoimmune, genetic, and immunological factors. We report a 47-year-old man with LS that exhibited an asymptomatic white plaque with red patches on the maxillary alveolar mucosa extending to the labial mucosa. He had no other skin disease. Positive immunostaining for tenascin and scarcity of fibronectin suggested extracellular matrix reorganization. Elastin immunostaining indicated a reduction of elastic fibers. Immunoexpression of collagen IV in blood vessels and its absence in the epithelial basement membrane, together with diffuse MMP-9 immunoexpression, suggested altered proteolytic activity. Mast cell staining bordering areas of sclerosis indicated a possible role in the synthesis of collagen. IgG4 positivity in plasma cells suggested a role in the fibrogenesis. This is an unusual presentation of oral LS and we discuss immunohistochemical findings regarding cellular and extracellular matrix components.

Case Presentation

Incidental angiofibromas prompt a diagnosis of multiple endocrine neoplasia type-1 (MEN-1)

Importance: We believe this to be the first documented report of multiple endocrine neoplasia type-1 (MEN-1) in which the diagnosis was suspected based purely on cutaneous findings. The patient was initially referred to the dermatology department for cosmetic concerns and had no overt symptoms, laboratory abnormalities, or known family history of MEN-1.Observations: The patient is a 28-year-old man who was referred to the dermatology department for evaluation and removal of skins lesions, later confirmed by biopsy to be facial angiofibromas and a truncal collagenoma. This combination of cutaneous findings was suspicious for a genodermatosis and genetic testing subsequently confirmed the diagnosis of MEN-1. The patient was referred for appropriate follow up and surveillance.Conclusions and Relevance: This case highlights the importance of vigilance on the part of dermatologists to be aware of subtle skin findings that may be characteristic of rare disorders and may have gone unrecognized by other providers and the patients themselves. In this respect, dermatologists are in a unique position given their specialized training in the recognition of inherited skin disorders. An early diagnosis of an inherited disorder, especially one with increased risk of malignancy, can allow for appropriate surveillance and potentially alter the course of the disease.

Disseminated Herpes Simplex Virus presenting as crusted papules on the palms and soles of an immunosuppressed patient

Disseminated Herpes Simplex Virus (HSV) may occur in immunosuppressed patients, most commonly causing visceral organ involvement and rarely presenting solely with cutaneous lesions.  We report an interesting case of disseminated HSV in a 77-year-old immunocompromised man, presenting as necrotic papules and pustules with no associated systemic involvement.

Porokeratotic eccrine ostial and dermal duct nevus - revisited

We hereby report a rare case of a 14-year-old girl presenting with asymptomatic pitted papules over the flexor aspect of her right 4th and 5th digits. This was histopathologically proven to be porokeratotic eccrine ostial and dermal duct nevus (PEODDN).

Alopecia areata with white hair regrowth: case report and review of poliosis

Alopecia areata is thought to be a T-cell mediated and cytokine mediated autoimmune disease that results in non-scarring hair loss. Poliosis has been described as a localized depigmentation of hair caused by a deficiency of melanin in hair follicles. A 57-year-old man with a history of alopecia areata developed white hair regrowth in areas of previous hair loss. We retrospectively reviewed the medical literature using PubMed, searching: (1) alopecia areata and (2) poliosis. Poliosis may be associated with autoimmune diseases including alopecia areata, as described in our case. However, it is also reported in patients who have cutaneous lesions, genetic syndromes, infections, medication use, and trauma. Hair regrowth following alopecia areata may be associated with poliosis. We hypothesize that the incidence of poliosis in areas of previous alopecia areata-related hair loss may be greater than reflected in the published literature.

Bullous systemic lupus erythematosus in a patient with human immunodeficiency virus infection: a paradox of autoimmunity and immunodeficiency

Bullous lupus erythematosus is a rare variant of systemic lupus erythematosus (SLE) and is characterized by autoantibodies to type VII collagen.  Co-existence of SLE and human immunodeficiency virus (HIV) infection is extremely rare; the development of bullous lupus in the setting of HIV has been, to our knowledge, reported in the literature only once.

We describe a 26-year-old man with an 8-year history of HIV infection who developed bullous SLE.  The patient presented with widespread, tense bullae as well as oral ulcerations.  Clinical, laboratory, histological, and cutaneous immunofluorescence findings confirmed the diagnosis of bullous SLE.

Given the immunological consequence of HIV infection, the co-occurrence of these two diseases would, theoretically, be unusual. Theories pertaining to the interplay of immunologic mechanisms of the seemingly paradoxical occurrence of autoimmunity in the setting of HIV infection are discussed.

Dyskeratosis Congenita

Importance: Dyskeratosis congenita is a rare disorder that often leads to early death owing to a variety of complications and associated disorders.  Early diagnosis and intervention is important in care for patients affected by this disease.

Observations: We describe a patient with dyskeratosis congenita (DC) in a child. Our patient had a 3-year history of transaminitis that was felt to be the result of biopsy proven progressive fibrosis of the liver beginning at age 3. He was referred to the dermatology department because of a chronic, evolving eruption with the hope of establishing a unifying diagnosis.  Further examination revealed dystrophic nails, numerous dental caries, and blepharitis.  Chromosomal analysis on leukocytes showed significant telomere shortening consistent with DC.

Conclusions and Relevance: Early recognition and long term care is important in patients with DC because of their propensity to develop malignancy, hematologic abnormalities, and infection.  Better understanding of this disease may lead to insights into other disorders associated with abnormal telomere maintenance.

Commentary

Future perspective of probiotics in dermatology: an old wine in new bottle

Probiotics are live microbial food supplements that are beneficial to the host health when administered in adequate amounts. Probiotics do have an exciting concept in digestive functions, but these live microbes have wider applicability as evidenced by gut-brain-skin axis theory given 80years back. However, the details regarding use of probiotics for dermatological indications ranging from atopic dermatitis to acne and sexually transmitted infections is dispersed in the literature, herein we have tried to focus all under one heading. Overall, probiotics seem to be promising and safe therapeutic modality, but the evidence as of now, from the available published data is low. This review will stimulate readers to carry out well designed, larger population based trials, so as to validate its use in dermatology practice.

Increasing use of non-traditional vehicles for psoriasis and other inflammatory skin conditions

Background: Although topical corticosteroid ointments were once viewed as the best vehicle for treating inflammatory skin diseases, the recognition of the problem of poor compliance and patients’ preferences for other vehicles has led to the development of corticosteroid products in alternative formulations.Objective: To describe patterns of use of newer vehicle formulations including foams, shampoos, sprays, and lotions for the treatment of psoriasis and other dermatoses.Methods: The use of non-traditional vehicles was identified using visit with diagnoses for psoriasis and other dermatoses from the National Ambulatory Medical Care Survey 2000-2010 data. Trends in corticosteroid vehicles mentions were evaluated over the study period to determine how the use of non-traditional vehicles has changed. The odds ratios of being prescribed a nontraditional vehicle were reported for patient and office-based characteristics of visits.Results: Approximately 2.3% and 1.9% of visits mentioned foam and other non-traditional vehicles (shampoo, lotion, spray), respectively. The use of corticosteroids in shampoo, lotion, or spray preparations increased by 0.5% annually (p=0.008) but did not significantly change for corticosteroids in a foam preparation (p=0.10). Psoriasis and seborrheic dermatitis were the leading diagnoses at visits prescribed corticosteroids in nontraditional vehicles. Dermatologists were more likely than non-dermatologists to prescribe foam products [OR: 8.4 (3.6, 19.9)] or clobetasol in another non-traditional vehicle [OR: 49.7 (10.3, 240.5)].Limitations: Product vehicle was not specified for all corticosteroids.Conclusion: Although there was increasing use of non-traditional vehicles, the rate of use has remained low. Dermatologists appear to have greater familiarity with the use of these newer vehicle formulations than do physicians in other specialties.

Photo Vignette

Myxoid stroma and delicate vasculature of a superficial angiomyxoma give rise to the red planet sign

Superficial angiomyxomas are uncommon benign mesenchymal tumors. They often recur locally if partially removed. This case report demonstrates not only the characteristic pathological findings of a superficial angiomyxoma in a 33- year-old man, but also shows a unique dermatoscopic image, which in our estimation resembles a celestial red planet such as the blood moon seen during a lunar eclipse. We propose to call this the “red planet” sign for a superficial angiomyxoma on dermoscopic examination.

Trichobacteriosis: contribution of dermoscopy

We report a case of axillary trichobacteriosis in a 34-year-old patient whose complaints were bromhidrosis and hyperhidrosis axillaris. The condition can be diagnosed clinically; dermoscopy may be useful and reveal typical signs.

Endometriosis primaria: un diagnóstico diferencial dentro de las lesiones nodulares umbilicales

La endometriosis umbilical primaria se caracteriza por la aparición de lesiones nodulares a nivel umbilical sin la presencia de intervenciones quirúrgicas abdominales previas ni presencia de endometriosis conocida en otras localizaciones. Presentamos el caso de una mujer de 43 años que presenta dicha entidad. Con este caso queremos destacar la importancia del diagnóstico diferencial de las lesiones nodulares a nivel umbilical sobre todo en mujeres en edad fértil.The onset of umbilical nodules with no previous abdominal surgeries or known endometriosis in other locations characterizes primary umbilical endometriosis. We present a 43-year-old woman with a painful umbilical nodule for several months. We report this case to emphasize the importance of the differential diagnosis of umbilical nodules, especially in women during the reproductive period.

Letter

Understanding the influence of social media in medicine: lesson learned from facebook

Atopic dermatitis is a very common chronic skin disease. With increasing number of patients searching social media outlets such as Facebook for medical information, social media can be used by physicians as a powerful educational tool. We analyzed the unmoderated Q&A series on Facebook begun by members of National Eczema Association Scientific Advisory Committee. Four respondents accounted for more than 50% of all responses and the most common were negative posts about topical steroids (61%). Possible strategies to accomplish the safe dissemination of information in a public forum may include a moderator role for physicians.

Not French manicure but onycholysis

French manicure, a simple and elegant manicure style, caught the public’s attention by the 19th century in French nail salons. Now this style has spread all over the world and is the first choice of 80% of women in the USA and Europe. It consists of a natural, pink, beige, or nude base tipped with pure white at the distal end.Onycholysis is a disease of the nails characterized by separation of the nail plate from the nail bed, with variable causes. We present 2 cases of onycholysis that resemble the beautiful and elegant French manicure.