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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 19, Issue 6, 2013

Review

Psychological Stress and skin aging: A review of possible mechanisms and potential therapies

The link between psychological stress and aging is intuitive although the underlying mechanisms are not well defined. Evidence suggests that chronic psychological stress stimulates the autonomic nervous system, renin-angiotensin system, and the hypothalamic-pituitary-adrenal axis when the body attempts to resolve perceived threats to homeostasis. Prolonged activation of these pathways can result in chronic immune dysfunction, increased production of reactive oxygen species, and DNA damage, which are known to contribute to the again of skin and other tissues.

Despite the lack of conclusive evidence directly linking psychological stress to skin aging, mechanisms by which stress leads to immune dysfunction, oxidative radicals, and ultimately DNA damage via neuronal, endocrine, and immune modulation may present a possible intervention for skin aging. In addition to the wide array of anti-oxidant therapies being developed to combat aging, the topical use of beta-blockers such as timolol, angiotensin receptor blockers such as valsartan, glucocorticoid blockers such as mifepristone, and cholinergic modulators including botulinum toxin, might be potential therapeutic strategies to prevent skin aging. Given the current understanding of these pathways, it would be premature to utilize such modalities for prevention of skin aging at this time, but future research into this type of topical pharmacologic anti-aging intervention may be promising.

Update on the treatment of genital warts

This review summarizes new treatments from the last seven years employed for the treatment of genital warts caused by human papillomavirus (HPV). Imquimod 3.75% is a new agent with fewer side effects and perhaps a better dosing schedule than imquimod 5%, but is not more effective. Sinecatechins/Polyphenon E 15%, a novel extract from green tea can be effective against genital warts but requires three times a day dosing and is not more effective than existing treatments; the treatment course is 12-16 weeks. Photodynamic therapy combined with other destructive modalities might increase the cure rate for genital warts. The quadrivalent vaccine against HPV 6, 11, 16, 18 is decreasing the incidence of warts in the western world but the evidence does not support vaccination as a treatment for those already infected by HPV. Hyperthermia and immunomodulators might be positive additions to the armamentarium of clinicians. In sum, there are new tools that physicians can use but none is really a great advance over what was available a decade ago.

Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis

Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation.    The diseases with the strongest association are obesity, depression, and pain.  HS is associated with many diseases including acne conglobata (AC), dissecting cellulitis, pilonidal cysts, and obesity.  Pyoderma fistulans sinifica (fox den disease) appears to be the same entity as Hurley Stage 2 of 3 HS. The rate of acne vulgaris in HS patients mirrors unaffected controls. The most common, albeit still uncommon, association is with seronegative, haplotype unlinked arthritis (most importantly B27), in particular spondolyarthritis.  Crohn disease and HS occur together at a rate that varies from 0.6% to 38% in retrospective cases series.  Ulcerative colitis occurred with HS in 14% of patients in one series.  The next most common association is with pyoderma gangrenosum, but this association is likely under-reported.  Synovitis-Acne-Pustulosis Hyperostosis-Osteitis (SAPHO) syndrome, which is rare, has more than 10 reports linking it to HS.  Nine case reports have linked Dowling-Degos disease (DDD) to HS and two reports related HS to Fox-Fordyce disease (FF), but because both occur in the axilla this might be a mere coincidence.   HS is rarely associated with ophthalmic pathology.  Specifically, more than 5 reports link it to Keratitis-Ichthyosis-Deafness syndrome (KID); greater than10 cases link it to interstitial keratitis and 2 cases are linked to Behçet’s disease. The presence of proteinuria and acute nephritis link HS to the kidney, especially since and reports have documented resolution of HS after renal transplant.  Florid steatocystoma multiplex, Sjogren Syndrome, and HS have been linked and their reports likely underestimate their coincidence because all these entities involve occlusion (albeit by different mechanisms).  Three reports link HS and amyloid, but both share some common genetic underpinnings and thus the coincidence of these diseases is likely underreported.  Pyoderma vegetans has been noted in 2 cases of HS and 4 cases of Inflammatory Bowel Disease (IBD) and is likely a clue to the linkage of the pathology of IBD and HS.  Pityriasis rubra pilaris, in particular Type VI related to HIV, has a relationship more commonly with acne conglobata, but with HS also. Single case reports of diseases associated with HS include systemic lupus erythematosus, acromegaly, Down syndrome, Bazex–Dupre´–Christol, and prurtis ani, but these might be coincidences.  Pyogenic Arthritis, Pyoderma gangrenosum, and Acne  (PAPA Syndrome) and Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis (PASH Syndrome) are pyodermic-arthritic syndromes that are associated with HS.  Erythema nodosum and granulomatous lobular mastitis have been reported with HS but the significance of these reports is uncertain.  Because of scarring, HS can result in lymphedema including scrotal elephantiasis and verrucous lymphedema.  HS is sometimes accompanied by obesity, hypertension, and anemia and can be considered a disease in the spectrum of metabolic syndrome, a skin disease with systemic consequences.  HS, like other types of chronic inflammation when long standing in the perianal and perineal areas, can result in squamous cell cancer.  A variety of drugs can induce HS. These include lithium, sirolimus, cyclosporine,  vemurafenib, and oral contraceptives.  Inverse psoriasis or psoriasis vulgaris as a side effect of infliximab therapy may be associated with HS. These associations aside, most cases of HS occur in isolation without coincident morbidity.

Original

Segmental phenolization for the treatment of Ingrown toenails: technique report, follow up of 146 patients, and review of the literature

Background: Ingrown toenail is a common problem causing significant morbidity. Various procedures used for treating this painful condition were discussed in the literature.

Objetctive: Through a prospective study we aimed to evaluate in a long-term follow-up the efficacy of partial nail plate avulsion combined with phenolic chemical matricectomy for the treatment of Ingrown toenails.

Methods: We recruited outpatient from January 2006 to December 2009; all treated by phenol matricectomy and assured a regular follow up.

Results: Consecutive 146 patients with a total of 171 Ingrown toenails were enrolled. There was no significant morbidity and the procedure was tolerated well by all patients. The short-term results were excellent. No severe complications occurred during the 21-month follow-up period. The overall recurrence was 2.7 %.

Conclusion: Phenol cauterization compared with other surgical ablation techniques may be the treatment of choice of Ingrown toenails. It’s an effective technique for the treatment of Ingrown toenails accompanied by less morbidity and lower recurrence rates.

Commentary

Explicit and implicit copayments for phototherapy: examining the cost of commuting

Background: Whereas phototherapy is a safe and cost-effective treatment modality for psoriasis, economic disincentives discourage its use, including both direct and indirect costs to the patient.

Purpose: To determine when it may be cost-effective for patients to purchase a home light unit versus driving to clinic for outpatient phototherapy sessions.

Methods: Estimates of expenses associated with 3 months of outpatient phototherapy were determined and compared to the price of a home phototherapy unit. Factors examined included the cost of gasoline (based on the national average), fuel efficiency of the vehicle, cost of owning and operating a motor vehicle, lost wages, and copayments.

Results: The cost for a standard 6-bulb narrowband UVB home unit is approximately $2600. Direct and indirect expenses imposed on patients increase with distance travelled to the dermatologist. If a patient lives 20 or more miles away from the dermatologist, the expenses associated with travel can total more than the out of pocket expense of purchasing a home phototherapy unit.

Limitations: This small analysis only accounted for the first 3 months of treatment and likely underestimates the total costs that patients would experience over a lifetime of treatment.

Conclusions: It may be beneficial for physicians to educate patients on the cost-burden of in-office versus home phototherapy because patients can use these parameters to determine which option would be more cost-effective for them.

A 3D Ultrasound Study of Sinus Tract Formation in Hidradenitis Suppurativa

Imaging of hidradenitis suppurativa allows the study of both the lesion morphology and evolution. Hidradenitis lesions of different stages were studied using  3D ultrasound in a cross sectional pilot study. A total of 25 HS patients (18 female/ 7 male, aged 18-46 year-old) and 10 healthy controls (5 female/5 male, aged 21-49 year-old) were studied. All patients were referred by dermatologists. All examinations were performed on the right axilla and compared with both controls and the skin outside the lesional areas.

3D ultrasound images demonstrated enlargement of the deepest portion of the hair follicles in early stages. In more advanced stages dermal and subcutaneous sinus tracts were identified that were commonly connected to the base of the regional hair follicles. At the end stage of the disease, these sinus tracts were further dilated and multiple.

Ultrasound allows early detection and characterization of the morphological changes in hidradenitis, which include the variable degrees of involvement of the hair follicles and the appearance of fluid collections and sinus tracts. Knowledge of this anatomical information may allow the identification of lesions that can benefit from medical or surgical management.

Case Report

Indolent systemic mastocytosis: a case and review of the current available treatment options

Mastocytosis denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells. Cutaneous mastocytosis is characterized by skin-limited disease and is the most common subtype. Systemic mastocytosis has extracutaneous organ involvement with variable symptomatology and prognosis. Clinical manifestations are secondary to mediator release or direct organ infiltration of mast cells. Current available treatment for systemic mastocytosis is non-curative with conventional symptom-directed therapy for all subtypes. Cytoreductive agents are available for those with refractory symptoms or extensive extracutaneous disease. To date, clinical responses remain mixed and systemic mastocytosis is still an incurable condition.

Extensive darier’s disease with pityriasis amiantacea, alopecia and congenital facial nerve palsy

We present a 65-year-old man with Darier disease with pityriasis amiantacea on the scalp, alopecia, and congenital facial nerve palsy.

Progressive nodular histiocytosis with normal karyotypic analysis

Non-Langerhans cell histiocytoses (NLH) comprise a spectrum of diseases that includes sinus histiocytosis with massive lymphadenopathy, hemophagocytic lymphohistiocytosis, xanthogranuloma, and reticulohistiocytoma. Progressive nodular histiocytosis (PNH) is a rare NLH that microscopically mimics juvenile xanthogranuloma but presents with disseminated persistent and progressive papulonodules in adults. Herein, we describe a case of PNH presenting as diffuse, progressively enlarging papules, nodules, and pedunculated tumors in a 38-year-old male. The diagnosis is supported microscopically by the morphologic and immunohistochemical findings. Whereas conventional cytogenetic analysis of Langerhans cell histiocytosis and juvenile xanthogranuloma has previously been described, there are no reports of the karyotype of PNH. In our patient, conventional cytogenetic analysis of the tumor revealed a normal karyotype. Although these results may represent the overgrowth of normal stromal cells rather than lesional cells, we believe this to be an important finding, indicating karyotypic analysis will not allow for distinction between PCH and other NLH or Langerhans cell histiocytoses.

Cutaneous angiosarcoma of the scalp mimicking a keratoacanthoma

Cutaneous angiosarcoma (CA) has a wide range of clinical presentations. In this case report, we discuss a 78-year-old gentleman, who presented with a keratoacanthoma-like scalp lesion that turned out histologically to be a cutaneous angiosarcoma. A brief overview of CA, including its etiology, prognostic factors, clinical manifestations, and treatment options will also be discussed.

Secondary bilateral striopallidodentate calcinosis associated with generalized pustular psoriasis (Von Zumbusch)

Bilateral striopallidodentate calcinosis (BSPDC) mentioned in the literature as Fahr's disease (a misnomer), is characterized by symmetrical and bilateral intracerebral calcifications located in the basal ganglia with or without deposits in the dentate nucleus, thalamus, and white matter. This entity is usually asymptomatic but may be manifested by neurological symptoms. Idiopathic BSPDC can occur either as sporadic or autosomal dominant familial forms. Secondary presentations of BSPDC are associated with infections, neoplastic diseases, toxicological or traumatic factors, and metabolic disorders. We describe a case of generalized pustular psoriasis associated with secondary BSPDC owing to pseudohypoparathyroidism. Laboratory tests revealed hypocalcemia, hyperphosphatemia, and a normal serum level of parathormone. The correction of the phosphorus-calcium metabolism disorder produced clinical improvement.

Additive effect of propranolol and pulsed dye laser for infantile hemangioma

The combination of propranolol and pulsed dye laser for the treatment of infantile hemangiomas may be superior to either alone. This case report illustrates the additive effect of propranolol and pulsed dye laser for an infantile hemangioma in a high-risk location. Although thorough clinical trials are needed, combination therapy for infantile hemangiomas may prove to be optimal for efficacy.

Case Presentation

Cutaneous metastasis of primitive neuroectodermal lung tumor

Primary sarcomas of the chest are rare. Although primitive neuroectodermal tumor (PNET) usually develops in the chest wall, it has been described as a primary pulmonary tumor. We present an unusual case of PNET arising in the lung of an 89-year-old man.

“Blueberry muffin” rash and large right thigh mass: a unique presentation of Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a clonal proliferation of bone marrow derived antigen-presenting cells that can involve a spectrum of cutaneous findings, with or without internal organ involvement. Neonatal LCH almost always presents with skin findings, usually petechial papules and/or erosions in a seborrheic distribution, with or without extracutaneous involvement. Previously described as varying entities, LCH is now considered a single disease process demonstrating a spectrum of clinical findings. We report a unique case of neonatal LCH presenting with a “blueberry muffin” rash in conjunction with a large soft tissue tumor.

Lichen planus pigmentosus-inversus involving the post-auricular sulci

Lichen planus pigmentosus-inversus is rare with only twenty patients (including our patient) formally described in the literature. The reported twenty patients with lichen planus pigmentosus-inversus include eight men and twelve women with ages ranging from 25–84 years (average age of 55.3). Twelve were Caucasian, five were Asian, one was Hispanic and two were of unknown ethnicity. Seventeen out of twenty patients had lesions in the axillae and mild pruritus was present in eight patients. Our patient is the first patient reported to have post-auricular sulcus involvement.

Localized interstitial granuloma annulare induced by subcutaneous injections for desensitization

We describe a patient with interstitial granuloma annulare associated with subcutaneous injection therapy (SIT) for desensitization to a type I allergy. Asymptomatic, erythematous, violaceous annular patches were located at the injection sites on both her arms. Medical history revealed perennial rhinoconjonctivitis treated with SIT (Phostal Stallergen® cat 100% and D. pteronyssinus/D.farinae 50%:50%).

Letter

Köebner phenomenon induced by cupping therapy in a psoriasis patient

Psoriasis is a chronic, immune-mediated inflammatory and refractory disease. The koebner phenomenon, which can be induced by trauma, is common in psoriasis patients. Herein, we report a patient with psoriasis who was treated by cupping therapy and subsequently developed the koebner phenomenon (KP) at the cupped sites. To our knowledge, it is the first report about cupping therapy leading to KP in a psoriasis patient.

Island flaps in the repair of medial canthus: Report of 8 cases

Reconstruction of the medial canthus following skin cancer excision is often challenging because of the complex anatomy and the cosmetic relevance of that region. The island pedicle advancement flap is an extremely versatile flap, which is commonly used to close defects in different body areas, including face, trunk, and extremities. We report our favourable experience with the use of island flaps, mobilized from the nasal saddle or lateral nasal side wall in 8 patients who had skin defects on the medial canthus region after Mohs micrographic surgery for basal cell carcinomas.