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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 24, Issue 3, 2018

Commentary

ProvenCare-Psoriasis: A disease management model to optimize care

There are a variety of evidence-based treatments available for psoriasis. The transition of this evidence into practice is challenging. In this article, we describe the design of our disease management approach for Psoriasis (ProvenCare®) and present preliminary evidence of the effect of its implementation. In designing our approach, we identified three barriers to optimal care: 1) lack of a standardized and discrete disease activity measure within the electronic health record, 2) lack of a system-wide, standardized approach to care, and 3) non-uniform financial access to appropriate non-pharmacologic treatments. We implemented several solutions, which collectively form our approach. We standardized the documentation of clinical data such as body surface area (BSA), created a disease management algorithm for psoriasis, and aligned incentives to facilitate the implementation of the algorithm. This approach provides more coordinated, cost effective care for psoriasis, while being acceptable to key stakeholders. Future work will examine the effect of the implementation of our approach on important clinical and patient outcomes.

Peer reviews: the dreaded rejection

This commentary considers the reasons for rejection of manuscripts during the peer-review process. Poor methodology, inappropriate statistical analysis, irrelevance, and technical errors are cited frequently as motives for manuscript rejection. Guidance, such as selecting an applicable journal, conducting a rigorous study, and writing efficiently, is provided for authors to prevent initial rejection. Researchers are reassured that rejection is a common consequence of peer-review and subsequent submissions to other journals are often successful publications.

Case Report

Perforating osteoma cutis: case report and literature review of patients with a solitary perforating osteoma cutis lesion

Osteoma cutis, the development of bone in the dermis and/or subcutaneous fat, can occur as either a primary or secondary condition. Perforating osteoma cutis is rare. A man with a solitary lesion of perforating osteoma cutis is described and the features of individuals with a single perforating osteoma cutis skin lesion are reviewed. A solitary lesion of either primary or secondary perforating osteoma cutis has only been observed in two men and one woman; the lesions had been present from less than one month to 19 or 20 years prior to establishing the diagnosis. The lesion was either located on the forehead (two men) or the breast (one woman). The erythematous (two lesions) or flesh-colored nodules ranged in size from 8×8 millimeters to 1.5×0.5 centimeters. Each had epidermal perforation by bone through a central area that was either crateriform or crusted or keratotic. The clinical differential diagnosis included keratoacanthoma, phlebolith, pilomatricoma, pilomatrical carcinoma, and squamous cell carcinoma. The perforating osteoma cutis lesion was successfully treated with either excision or shave biopsy without recurrence at either 10 or 12-months follow-up.

Case Presentation

Kindler syndrome in a patient with colitis and primary sclerosing cholangitis: coincidence or association?

Kindler syndrome is a rare, autosomal recessive genodermatosis, caused by mutations in the FERMT1 gene. It is thought to be primarily a skin disease, but other organs may also be involved. We report a case of a novel mutation of FERMT1 gene in a patient with a probable new phenotype of Kindler syndrome, including colitis and primary sclerosing cholangitis. A 42-year-old man, born to first cousin parents, was referred to our outpatient dermatology clinic with an unknown dermatosis since birth. He presented with neonatal blistering and developed photosensitivity and changes in skin pigmentation during childhood. Since the age of 20, he has had regular follow-up in the gastroenterology clinic, owing to esophageal stenosis, ulcerative colitis, and primary sclerosing cholangitis. Clinical examination revealed jaundice, poikiloderma, diffuse cigarette paper-like atrophy on dorsal surfaces of the hands, and palmoplantar hyperkeratosis. Skin biopsy showed epidermal atrophy covered by orthokeratotic hyperkeratosis. DNA molecular analysis revealed FERMT1 homozygous mutation c.1179G>A, p.W393X, which has not been reported before. The intestinal phenotype of Kindler syndrome has already been defined previously. However, to the best of our knowledge, no other case of primary sclerosing cholangitis in a patient with Kindler syndrome has been reported.

Mycoplasma-induced pustulosis with perifollicular involvement

Mycoplasma pneumoniae-induced rash and mucositis (MIRM) is a disease characterized by mucosal involvement with variable cutaneous manifestations induced by M. pneumoniae infection. Previously reported rash morphologies include vesiculobullous, targetoid, papular, macular, and morbilliform lesions. Pustulosis is a rare presentation of MIRM that has been described only once before in the literature to our knowledge. We report a case of a 13-year-old boy presenting with a pustular skin eruption induced by Mycoplasma infection. Ours’ is the second report of MIRM, to our knowledge, presenting with pustulosis and the first, to our knowledge, to first to describe the histopathologic finding of perifollicular neutrophilic infiltration in MIRM.

Thalidomide for the treatment of chronic refractory prurigo nodularis

Prurigo nodularis (PN) is a highly pruritic skin condition that is caused by chronic scratching. It occurs in patients with chronic itch and is characterized by multiple hyperkeratotic papules and nodules. The pathogenesis of PN is unclear, but involves a complex interplay of numerous pathways including neurogenic and inflammatory factors. As such, PN is very difficult to treat and patients are often refractory to multiple medications before finding a treatment that is effective. We present a woman with a 20-year history of exuberant prurigo nodularis who failed multiple therapies, including dapsone, azathioprine, mycophenolic acid, prednisone, topical steroids, and phototherapy. She only obtained significant relief of chronic pruritus and lesion flattening with thalidomide 100mg daily. Thalidomide is an antipruritic and anti-inflammatory agent that has shown to be very effective in treating a variety of dermatologic conditions. However, its use today is limited by concerns for its teratogenic and neuropathic side effects. With strict adherence to medication protocols, these adverse effects can be minimized. As such, thalidomide should be considered for patients with refractory dermatologic conditions.

Genitogluteal porokeratosis in an HIV-positive man: a case report and review of the literature on genital porokeratosis

Genitogluteal porokeratosis is a disorder of keratinization that may present in men in their fourth decade of life. We describe a 52-year-old human immunodeficiency virus (HIV)-positive man with history of anal squamous cell carcinoma who developed verrucous lesions on the buttocks and genitals. The buttock lesions presented shortly after radiotherapy for anal carcinoma a decade prior, whereas the genital lesions presented three months prior in areas treated with injectable medication for erectile dysfunction. Skin biopsy revealed a cornoid lamella, leading to the diagnosis of genitogluteal porokeratosis. The buttock lesions were treated with shave excision and the genital lesions were treated with topical agents. Using the PubMed database, a literature search was performed with combinations of the following key words: acuminata, condyloma, cornoid lamella, genital, genitogluteal, HIV, penile, porokeratosis, verrucous, vulvar. The generated papers and their references were reviewed. To the best of our knowledge, we present the first reported case of genitogluteal porokeratosis in an HIV-positive man. Notably, these lesions developed in sites of prior radiation or injection. This condition should be included in the differential diagnosis of chronic lesions of the genitals and buttocks in patients with HIV and/or history of radiation treatment and/or trauma to the genitogluteal region.

Persistent perpendicular pigmentation: a peculiar but specific type of persistent nevus

Persistent nevi are commonly encountered in clinical and dermatopathology practice. Although they may mimic melanoma on clinical presentation as well as microscopic review, they behave in a benign fashion and are not associated with more aggressive behavior than the original nevus. Persistent nevi may exhibit asymmetry and irregular features that prompt concern for melanoma. However, the relative circumscription and restriction of pigment to the surgical scar provides a valuable clue to diagnosis. Some persistent nevi may have a linear pattern when they are confined to a widening scar. In this situation, the pigment spreads along the lines of skin tension (Langer lines). This unique pattern can be easily identified and should be a reassuring finding. We present a case of persistent nevus with a striking perpendicular pigmentation confined to the scar; the clinical and histologic features of persistent nevi are reviewed.

An amelanotic nail bed melanoma presenting as persistent onychodystrophy

Nail apparatus melanomas are rare and may present with a wide variety of clinical presentations. In particular, the amelanotic subtype can pose a diagnostic challenge, often leading to a poor prognosis related to a delayed diagnosis. We report a 69-year-old man with an unusual subungual amelanotic melanoma presenting as a persistent single nail dystrophy that was repeatedly treated as onychomycosis. Owing to the delayed diagnosis of the melanoma and to minimize recurrence risk, the patient underwent a partial amputation of his left thumb.

Photo Vignette

A case of segmental Darier disease treated with doxycycline monotherapy

Darier disease is a rare autosomal dominant disorder that results from a mutation in the gene coding for the endoplasmic reticulum membrane calcium pump Ca2+-ATPase type 2 (SERCA2), leading to compromised intercellular adhesion. Patients typically present in the first two decades of life with keratotic, greasy papules in a seborrheic distribution. Segmental Darier disease is a variant with localized disease that follows Blaschko lines. Treatment options include topical and systemic agents including corticosteroids, retinoids, and antibiotics. We present a 67-year-old woman who came to our clinic with segmental Darier disease recalcitrant to topical therapy. Owing to cost and side effect profile, the patient declined treatment with oral retinoids. Doxycycline 100mg daily was started with significant improvement. Tetracyclines both chelate and assist calcium to cross membranes. This mechanism may correct the cellular calcium imbalance present in Darier disease. In addition, tetracyclines have been shown to inhibit metalloproteinase 9, an important part of Darier disease pathogenesis. Owing to its favorable side effect profile, further investigation is warranted to establish doxycycline as a more widely utilized treatment option for Darier disease.

Temozolomide-induced inflammation of disseminated superficial actinic porokeratosis

We report a case of temozolomide (TMZ)-induced inflammation of disseminated superficial actinic porokeratosis (DSAP), an uncommon and pre-malignant cutaneous disorder. Dermatologists and oncologists should be aware of this cutaneous eruption of DSAP associated with TMZ to prevent the discontinuation of effective medical therapy in cancer patients.

Cutaneous complication of perinatal hypoxia

Subcutaneous fat necrosis of the newborn is an uncommon, transient, and self-healing panniculitis, mostly affecting term newborns with perinatal complications. The authors present a case of a female full-term neonate, born from an uncomplicated pregnancy, admitted into the neonatology unit 5 hours after delivery because of refractory multifocal seizures in the context of hypoxic-ischemic encephalopathy. Nine days after birth, indurated and erythematous nodules and plaques were noted on the left arm and back. Skin biopsy was compatible with subcutaneous fat necrosis of the newborn. Laboratory evaluation including serum calcium showed normal values. No treatment was initiated. This entity generally follows an uncomplicated course. However, there are important complications for which the patient must be regularly monitored, including thrombocytopenia, hypoglycemia, hypertriglyceridemia, and most importantly, hypercalcemia. Patients should have serial serum calcium determinations for up to 6 months after the appearance of the skin lesions. The early diagnosis and prompt treatment of hypercalcemia are essential to prevent severe complications.

Circumscribed palmar hypokeratosis

Circumscribed palmar hypokeratosis (CPH) is a benign dermatologic condition characterized by local thinning of the stratum corneum. Herein, we present a 52-year-old woman who presented with an asymptomatic pink depressed papule on the right palm. This was treated with clobetasol 0.05% topically twice a day with no improvement.

Infiltrative basal cell carcinoma mimicking tinea corporis

The diagnosis of infiltrative basal cell carcinoma (BCC) can be delayed owing to its often subtle clinical findings. A 90-year-old woman presented with an asymptomatic annular pink plaque on her left shin that was clinically diagnosed as tinea corporis. After years of not responding to topical anti-fungal therapy, biopsies confirmed a diagnosis of infiltrative BCC. We discuss the differential diagnosis of the case, the difficulties in identifying infiltrative BCC, and the pathologic features of infiltrative BCC.

Vulvar syringomas as a part of nonfamilial generalized eruptive form: Unusual lesions leading to pseudohyper-trophy of the labia majora

We present a case of eruptive generalized syringoma with vulvar involvement. This case is unique in terms of being a nonfamilial, linear variant, leading to pseudohypertrophy of the labia majora.

Capillary hemangioma associated with dermal atrophy masquerading as a deep fungal infection

Hemangiomas are benign vascular neoplasms which arise in early adulthood. Herein we present a 79-year-old woman with a hemangioma of the lower flank masquerading as a cutaneous manifestation of a systemic fungal infection upon initial histological analysis. Decreased elastin and collagen within the lesion likely accounted for the clumping and splaying of the capillaries into “hyphae-like” structures. Loss of dermal elastic tissue and collagen apparently concentrated the capillary proliferation into an unusual morphology mimicking the hyphal structures. Through additional staining methods the lesion was confirmed to be an unusual presentation of a capillary hemangioma.

Letter

Secukinumab shows significant efficacy in two patients with difficult-to-treat areas of psoriasis: a Greek experience

Psoriasis is one of the most frequently occurring chronic inflammatory skin diseases. There are some specialized regions of the body that are considered difficult to treat. Secukinumab is a human monoclonal immunoglobulin G antibody that blocks the interleukin 17A ligand and has been shown to be highly efficacious in treating moderate-to-severe psoriasis. We studied two Greek patients, one with scalp psoriasis and the other with palmoplantar psoriasis, both resistant to treatment. Patients were treated with secukinumab and efficacy and safety were recorded. The patient with severe, refractory palmoplantar psoriasis achieved complete clearance at the end of the 4-week treatment period with secukinumab. The patient with moderate to severe, chronic scalp psoriasis was successfully treated with secukinumab, obtaining complete clearance of symptoms and remission of disease after approximately 16 weeks. In both cases clinical response was maintained through week 52. Secukinumab has been shown to be highly efficacious in the treatment of psoriasis of specific anatomical sites with an acceptable safety profile.

Cutaneous Staphylococcus lugdunensis infection: an emerging bacterial pathogen

Staphylococcus lugdunensis is a part of the normal skin flora. However, this organism can be a pathogen in certain situations such as advanced age or immunosuppression. Further study regarding situations in which this bacterium becomes a pathogen is warranted.

Ectopic intestinal mucosa implanted on the perianal skin of a patient with Crohn disease

ntestinal mucosa implanted in skin is an exceedingly rare occurrence. Implantations are thought to occur during the creation of ostomy sites or other surgical procedures in which suture goes through bowel mucosa and then skin. Current ostomy literature reports this as a very uncommon complication. We present a 54-year-old man diagnosed with Crohn disease with severe perianal involvement who was referred to our outpatient clinic because of two persistent perianal cutaneous ulcerations. He previously underwent several interventions to drain complex perianal fistulas and abscesses, the last of them involved placing seton stitches to ensure continuous draining during the healing process. Physical examination revealed two painful ulcerations with bleeding on contact. A skin biopsy was performed, revealing ectopic intestinal mucosa with crypts, villi, and goblet cells. Perianal ulcerations characteristic of Crohn disease might be difficult to differentiate from ectopic implant of bowel mucosa secondary to a surgical procedure in the perianal area. Therefore, we believe a high degree of suspicion and skin biopsy are key to the diagnosis.

Gel manicures and ultraviolet A light: A call for patient education

Gel manicures have become a popular beauty trend in recent years. The specially formulated nail polish must undergo curing under light-based units. The majority of these curing lamps emit high-intensity ultraviolet A, which can cause skin damage and increase the risk for skin cancers. Incorporating relevant information in patient education endeavors can help them practice avoidance, apply broad-spectrum sunscreen prior, or use nitrile exam gloves in order to prevent photoaging and skin cancer resulting from these procedures.