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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 2, 2016

Review

Proximal nailfold microhemorrhage events are manifested as distal cuticular (eponychial) hemosiderin-containing deposits (CEHD) (syn. Maricq sign) and can aid in the diagnosis of dermatomyositis and systemic sclerosis

Importance: Many patients present with cutaneous signs and symptoms that suggest a diagnosis on the autoimmune disease spectrum. During the "acute phase" of disease activity, patients with systemic sclerosis (SSc) and dermatomyositis (DM) have characteristic nailfold findings, including dilated capillaries, microhemorrhages, and hemosiderin deposits.

Objective: To review the literature on the presentation of microhemorrhages and to highlight the differences (in terms of terminology, characterization, and clinical relevance) between proximal microhemorrhage events and the distal products, often thought of as “hemosiderin deposits” located in the cuticle (eponychium). Because we found no studies directly showing these cuticular products are in fact “hemosiderin-containing,” we conducted a direct staining experiment in vivo using Prussian blue in order to increase our confidence that these products are indeed hemosiderin-containing and that the terminology is accurate for further use.

Evidence Review: In July-December 2014, the MeSH function in PubMed was used to identify approximately 165 articles relating to capillaroscopy. We reviewed these articles for mention of microhemorrhages and hemosiderin deposits. In addition, we used PubMed and Google Scholar searches for “hemosiderin + nail”, “Prussian Blue + nail”, and “hemosiderin deposit.” We found no papers reporting the use of Prussian Blue directly on nailfolds of patients with SSc and DM in vivo.

Findings: In our literature review, “microhemorrhages” and “hemosiderin deposits” were often used synonymously, yet they are clearly distinct entities. We present a case in which the presence of these deposits supported a diagnosis of amyopathic DM. We used Prussian blue staining solution to visualize the cuticular (eponychial) hemosoderin-containing deposits (CEHD) – distal cuticular products that reflect previous proximal nailfold microhemorrhage events. CEHD can serve as an indicator of active autoimmune disease, particularly in SSc and DM.

Conclusions and Relevance: CEHD are in fact hemosiderin-containing deposits that can reflect the active inflammatory phase of microvascular injury occuring in autoimmune disorders such as DM and SSc. CEHD can be visualized and documented at the bedside with tools commonly available to any dermatologist (portable dermatoscope and compact digital camera).

Update on treatment of photodermatosis

Photodermatoses are a group of skin conditions associated with an abnormal reaction to ultraviolet (UV) radiation. There are several of the photosensitive rashes which mainly affect the UV exposed areas of the skin. It can be classified into four groups: immunology mediated photodermatoses, chemical and drug induced photosensitivity, photoaggravated dermatoses, and genetic disorders. A systematic approach including history, physical examination, phototesting, photopatch testing, and laboratory tests are important in diagnosis of a photodermatosis patient. In order to optimally treat a disease of photodermatoses, we need to consider which treatment offers the most appropriate result in each disease, such as sunscreens, systemic medication, topical medication, phototherapy, and others. For all groups of photodermatoses, photoprotection is one of the essential parts of management. Photoprotection, which includes sunscreening and wearing photoprotective clothing, a wide brimmed hat, and sunglasses, is important. There are also promising emerging photoprotective agents.

Original

Patient satisfaction with current psoriasis treatment: a real-world study in the USA

Psoriasis patients often report dissatisfaction with treatment. However, it is less clear how the severity of key psoriasis symptoms (painful skin, itching, and scaling) as well as overall disease severity influence patient dissatisfaction levels. Using the Adelphi 2011/2013 Psoriasis Disease Specific Programmes, two “real world” surveys of US dermatologists and their patients, patient satisfaction was evaluated. Dermatologists provided data on disease characteristics, while patients indicated their satisfaction with existing treatment. Physician-reported severity (none, mild, moderate/severe) of psoriasis-related itching, pain, and scaling, overall disease severity (mild, moderate and severe) and therapy type were compared by patient satisfaction levels (satisfied vs. dissatisfied). Multivariate regressions examined the relationship between patient satisfaction, clinical symptoms, and psoriasis overall disease severity, controlling for differences in patient demographics and comorbidities. The sample comprised 633 psoriasis patients (56% male) with a mean age of 45. Overall, 18% of patients reported dissatisfaction with their psoriasis treatment. Dissatisfied patients were more likely to have moderate (65% vs. 40%) or severe (21% vs 3%) psoriasis compared to patients who were satisfied (both p<0.05). Dissatisfied patients were also more likely to have more severe pain (30% moderate-to-severe pain vs. 9%), more severe itching (61% moderate-to-severe itching vs. 25%), and more severe scaling (68% moderate-to-severe scaling vs. 33%) than satisfied patients (all p< 0.05). Multivariate analyses confirmed these results. Clinicians should be aware that some psoriasis patients, especially those with severe overall disease or symptoms, may be dissatisfied and are in need of better treatment.

New Technology

Use of high frequency ultrasonography in dermatology departments in Spain

Background: High Frequency Ultrasonography (HFUS) is a rapid, reliable, and effective diagnostic technique that has become progressively employed within the past years among dermatologists.

Objective: To describe the use of HFUS among dermatology departments in Spain.

Methods: An observational, cross-sectional descriptive study was conducted in 23 dermatology departments at Spanish public hospitals. Study data was collected during 10 working days.

Results: Fifty dermatologists from 11 Spanish regions participated in the study; 659 HFUS procedures were undertaken in 633 patients. HFUS indications were benign tumors (41%), malignant tumors (28%), inflammatory diseases (18%), skin appendage conditions (10%), and cosmetic conditions (1%). HFUS was performed for assessing the clinical differential diagnosis (58% of cases), surgical planning (17%), follow-up (17%), and other reasons (7%). Among the diagnostic group, the scan confirmed clinical diagnosis in 82% of cases and helped to change the clinical diagnosis in 17% cases (10% of total scans).  HFUS procedure duration was less than 5 minutes (45%), between 5 and 10 minutes (32%), and more than 10 minutes (21%). Mean patient satisfaction score (range 0 to 5) was 4.8.

Conclusions: The use of HFUS in usual practice is increasing, especially among younger dermatologists. This procedure is effective both for confirming and changing diagnosis, as well as for therapy decision making and follow-up. It is also worth remarking that a very high degree of patient satisfaction was recorded. The descriptive results reported in this study support the use of ultrasonography techniques and further reinforce their use in Dermatology.

Dermatology and Social Media

Dermatology in Doximity

Doximity, currently the largest online social networking service for United States (US) health care professionals and medical students, provides a wide variety of content to a large audience. In fact, its database includes 1,078,305 physicians in the US. It is therefore important to evaluate this content from time to time. Our objective is to analyze both the residency rankings and news content presented in Doximity, with respect to dermatology. The study compared the residency rankings created by Doximity to another dermatology residency ranking system that used a different algorithm. In terms of dermatology content, seven dermatology-related search terms were entered into the Doximity search query and data was collected on the first 20 “relevant” articles. Our study evaluated a total of 140 articles. The search term “skin cancer” yielded the most articles totaling 6,001. Informative articles were the most common type of article for each content item searched except for “dermatology”, yielding research articles as the most common content type (70%). The search term “melanoma awareness” had the largest number of shares (19,032). In comparing dermatology residency rankings on Doximity with another ranking system that accounted for scholarly achievement, there was 50% overlap. In conclusion, it is vital to evaluate content on social media websites that are utilized by US medical students and health care professionals. We hope this information presented provides an up-to-date analysis on the quality of one particular social media platform.

Case Report

Amitriptyline-induced cutaneous hyperpigmentation: case report and review of psychotropic drug-associated mucocutaneous hyperpigmentation

Background:  Several drugs can be associated with hyperpigmentation of mucosa or skin. They include antibiotic, antimalarial, antineoplastic, and psychotropic medications.

Purpose:  To describe a 42-year-old woman with amitriptyline-associated photo-distributed hyperpigmentation and to review psychotropic drug-induced hyperpigmentation of the skin.

Materials and Methods:  The features of a woman with amitriptyline-induced hyperpigmentation are presented. Using PubMed, the following terms were searched and relevant citations were assessed and discussed for context: amitriptyline, chlorpromazine, citalopram, desipramine, drug-associated, drug-induced, Fontana Masson, hyperpigmentation, imipramine, melanin, melanophages, mirtazapine, phenytoin, psychotropic, sertraline, thioridazine, tricyclic antidepressant.

Results:  Photo-distributed hyperpigmentation on the upper back of a woman developed six and a half years after initiation of amitriptyline therapy. Biopsy of the affected area showed pigment-laden melanophages and intradermal melanin deposition.

Conclusions:  Psychotropic drugs associated with cutaneous hyperpigmentation include amitriptyline, chlorpromazine, citalopram, desipramine, imipramine, mirtazapine, phenytoin, sertraline, and thioridazine. The hyperpigmentation may initially appear many years after starting the medication. Pathology typically shows melanophages and melanin in the dermis. Fontana Masson stain confirms the presence of melanin; Perl stain for hemosiderin or iron is negative. Discontinuation of the drug may result in spontaneous improvement. Further studies are needed to better understand the role of Q-switched laser in treating drug-induced hyperpigmentation.

Case Presentation

An uncommon presentation of an uncommon disease: relapsing polychondritis overlap with systemic lupus erythematosus

Relapsing polychondritis (RP) is a rare rheumatologic disorder in which recurrent episodes of inflammation result in destruction of cartilage of the ears and nose. The joints, eyes, audio-vestibular system, heart valves, respiratory tract, kidneys, and skin can also be involved. Skin involvement is most frequently linked to concomitant myelodysplastic syndrome and has rarely been associated with systemic lupus erythematosus.

A 47-year-old woman presented with violaceous, indurated, tender plaques on the bilateral cartilaginous ears with sparing of the lobes, consistent with RP. Further investigations revealed positive ANA and anti-Smith antibody, oral ulcers, a photo-distributed skin eruption, and biopsy-proven lupus nephritis, leading to a second concomitant diagnosis of systemic lupus erythematosus (SLE). The diagnosis of SLE associated with RP was made and the patient was started on oral prednisone and hydroxychloroquine.

This is a rare report of SLE associated with RP. It is unclear whether RP occurring in patients with SLE represents another clinical manifestation of SLE or a coexisting disease. However, a significant ANA titer in a patient with RP strongly suggests the presence of an associated autoimmune disorder. If immunologic abnormalities usually found in SLE are detected in patients with RP, it is important to monitor patients for the development of other manifestations of SLE.

Wade histoid leprosy revisited

An 18-year-old man presented with a 4-year history of erythematous patches on the trunk, followed 2-years later by multiple nodules, mostly located on the limbs, and distal paresthesias. Two close contacts were treated for leprosy during his childhood. Histopathological examination revealed a histiocytic infiltrate with acid-fast bacilli on Ziehl-Neelsen stain. The slit-skin and nasal smears showed numerous acid-fast bacilli. The correlation between clinical, epidemiological, histopathological, and microbiological features allowed the diagnosis of lepromatous leprosy, histoid variant. Multidrug therapy as recommended by the WHO was initiated. A rapid and sustained improvement was seen. Histoid leprosy is a rare manifestation of lepromatous leprosy, first described by Wade in 1960. Since then few cases have been reported, the majority of them from countries with a high prevalence of the disease. Early recognition and treatment are of most importance to prevent neurological disabilities and achieve epidemiological control.

Progeria and the early aging in children: a case report

The Hutchinson-Gilford syndrome or progeria is a rare autosomal dominant syndrome characterized by premature aging and involvement of internal systems, such as the circulatory and locomotor. The diagnosis is essentially clinical and the manifestations become more evident from the first year of life. Long term outcome data from Progeria Research Foundation clinical trials have demonstrated an increase in survival in recent years. Even though new trials are ongoing, the recognition of this syndrome is essential to prevent cardiovascular and cerebrovascular complications. A patient, initially asymptomatic, who developed characteristic signs of the syndrome at the age of 6 months is reported. She was referred for evaluation only when she was two years and eleven months old. The diagnosis of Hutchinson-Gilford syndrome was suspected owing to clinical characteristics. The diagnosis was confirmed by genetic testing. A mutation c.1824C> T in exon 11 of the LMNA gene was detected. She was registered in the Progeria Research Foundation and was invited to participate in the weighing and supplementation program. She was included in the lonafarnib protocol study. This medication is a farnesyl transferase inhibitor that prevents the production of progerina and slows cardiovascular and neurological complications of the syndrome. This case highlights the importance of diagnosing progeria patients because they may be referred to the Progeria Research Foundation, which offers genetic screening and inclusion in clinical and therapeutic follow-up protocols without any costs. Progeria trials and research may also contribute to new drug developments related to prevention of aging and atherosclerosis in the near future.

Generalized morphea as the first sign of breast carcinoma: a case report

Generalized morphea is a rare idiopathic form of scleroderma that literally means “hard skin.” Morphea is usually considered an isolated event that is not associated with malignancy. However, case reports of lung, hematologic, and breast cancer occurring simultaneously with large plaque morphea have caused dermatologists to question whether a work-up for malignancy is appropriate. We highlight a case of generalized morphea that preceded invasive ductal carcinoma of the breast and provide a discussion about the possible paraneoplastic origin of generalized morphea and systemic sclerosis (SSc).

Neutrophilic dermatosis of the dorsal hands in an elderly man

recently diagnosed with stage II lung adenocarcinoma, who presented to the clinic with a 14-day history of painful macules that progressed to bullae on the dorsal surface of his hands; decreased range of motion was noted. Examination revealed bilateral small, tender violaceous vesicopustules admixed with larger tense hemorrhagic pus-filled bullae on the dorsal aspect of his hands. Biopsy demonstrated changes consistent with neutrophilic dermatosis of the dorsal hands. The patient had been diagnosed with ulcerative colitis in the 1970s, although the condition was asymptomatic at the time of presentation. Treatment with prednisone 60 mg daily resulted in significant improvement by the next day. This regimen was continued for two weeks and was followed by a 6-week steroid taper. After a review of the approximate 75 cases currently reported, we also discuss the recurrence rate of NDDH of approximately 10%.

Photo Vignette

Nevoid hyperkeratosis of the nipple and the areola

A 25-year-old woman presented to our dermatology unit with progressive diffuse thickening of the both areolas. A skin biopsy was taken from the right areola showing irregular filiform acanthosis with elongation and anastomosis of rete ridges, irregular orthokeratotic hyperkeratosis, pseudocyst formation, and hyperpigmentation of the basal layer. The cause of nevoid hyperkeratosis of the nipple and areola is unknown. Eighty percent of cases occur in women and are often manifested during puberty or pregnancy. The disease may also occur in men receiving hormonal therapy. There is no uniformly effective treatment. Dermatologists, gynecologists, and general practitioners have an important part to play in recognizing this condition that may be underdiagnosed because of lack of awareness.

Verrucous tumor mimicking squamous cell carcinoma in immunocompetent patient

Mycobacteria cause a range of diseases in both immunocompetent and immunosuppressed individuals. An increase in non-tuberculous mycobacterial (NTM) infections targeting skin has been described. Many hypotheses have been developed in order to explain it: the increasing burden of immunocompromised individuals, immigration from endemic countries, improved laboratory identification techniques, and changes inhuman behavior that expose individuals to this NTM. Mycobacterium mucogenicum group comprises M. mucogenicum, Mycobacterium aubagnense, and Mycobacterium phocaicum. This group of organisms was first named Mycobacterium chelonae-like organism in 1982. Most clinically significant cases of those organisms involved catheter-related infections. Nevertheless, we report an interesting patient with a cutaneous infection produced by M. mucogenicum mimicking a squamous cell carcinoma; an excellent response to combined therapy with rifampicin and clarythromicin was observed.

Letter

Dapsone for treatment of erythema nodosum

Erythema Nodosum (EN) is the most common form of septal panniculitis, with over 100 reported infectious, malignant, inflammatory, idiopathic, and autoimmune causes.

Multiple cutaneous lipomatous neurofibromas

Cutaneous lipomatous neurofibroma is a rare variant of neurofibroma with a little known pathogenesis; its presence has never been described in Brazil. A 61- year-old woman complaining scalp papules for one year, presented with skin colored to yellowish papules on the scalp. She noted that they were sometimes itchy and occasionally bled.

Dermoscopy showed amorphous material, milky white, with a small poorly delimited yellowish area. Histopathological examination revealed spindle cell proliferation associated with mature adipocytes. Cutaneous lipomatous neurofibroma appears to be an underdiagnosed condition owing to the low index of suspicion and lack of knowledge of its existence. Few cases have been reported in the literature.

Atypical rosacea in a male patient: case study

Rosacea fulminans is a rare disorder of unknown cause, almost exclusively affecting women. There are only a few reported cases in men. The condition is characterized by the abrupt onset of pustules and nodules predominantly affecting the cheeks or chin without any systemic upset. We report the case of a 37-year-old man who presented with papulopustules, predominantly localized to his nose. Histopathological features were consistent with rosacea fulminans. The patient was managed with treatments including oral prednisolone, isotretinoin, and trimethioprim.