A Case of Circumscribed Palmar or Plantar Hypokeratosis and a Mini-review of the Literature.

ABSTRACT: Circumscribed palmar or plantar hypokeratosis (CPPH) is a new dermatologic disorder that firstly defined Pérez A et al in 2002. Since that time, further cases of CPPH have been reported by different authors in different countries. We report a 69-year-old Turkish woman who presented with asymptomatic, erythematous patches on the thenar region of the left hand and on the second left finger. Skin biopsy showed histological features of CPPH. In this article, it was emphasized that CPPH may be seen more frequently than expected and the clinical and pathological features of this disease with suspected malignant transformation should be known.

A case of filgrastim-induced neutrophilic dermatosis of the dorsal hands in a patient with Felty Syndrome.

Neutrophilic dermatosis of the dorsal hands (NDDH) is a variant of Sweet syndrome that presents with erythematous bullae, papules/plaques, or pustules on the dorsal hands. It is most commonly associated with hematologic and solid organ malignancies, though cases of NDDH associated with inflammatory bowel disease, rheumatologic disorders, and medication exposure have also been described in the literature. Felty syndrome is a rare complication of long-standing rheumatoid arthritis characterized by neuropathy, splenomegaly, and neutropenia. Granulocyte colony stimulating factors (e.g., filgrastim) can be utilized to rescue the neutropenia observed in Felty syndrome, but this treatment may subsequently cause Sweet syndrome. Herein, we present a 64-year-old man with Felty syndrome and a complex medical history who presented with sudden onset, painful blisters located on the dorsal and palmar aspects of his bilateral hands. Given the patient's past medical history, a broad differential diagnosis, including disseminated fungal and viral infection was initially considered. A punch biopsy of the skin lesion disclosed neutrophilic dermatosis, which together with laboratory data satisfied the von den Driesch criteria for Sweet syndrome. As the lesions were localized exclusively on the patient's hands, the qualification of NDDH was also endorsed.

Aquagenic wrinkling of the palms: review of the literature.

Aquagenic wrinkling of the palms (AWP) is a rare dermatological disease characterized by development of rapid and excessive wrinkling and oedema of the palms and transient whitish or yellowish papules without erythema on the palmar surfaces after immersion in water. This phenomenon can be accompanied by pain and/or pruritus. The most common treatment of AWP involves aluminium-based topicals. This article discusses the associations, pathological mechanisms and treatment options of AWP.

Gout-like hands.

A 65-year-old man presented to our hospital with a 1-year history of multiple nodules on his arms and hands without itching or pain.

Bowen Disease Within a Circumscribed Palmar Hypokeratosis.

ABSTRACT: Circumscribed palmar or plantar hypokeratosis is a focal disorder of keratinization that consists of a reduction in the thickness of the corneal layer of the epidermis of palms or soles. Although it is considered a benign entity, the thinning of the stratum corneum facilitates ultraviolet damage in the affected skin, which may result in an increased risk of developing focal epidermal dysplasia. Other factors, such as immunosuppression in transplanted patients, may play a role as well. We present a case of circumscribed palmar or plantar hypokeratosis with features of Bowen disease limited to the hypokeratotic epidermis.

The chameleon rash: a review of the polyphenotypic dermatoses of dermatomyositis.

Dermatomyositis (DM) is an autoimmune connective tissue disease that is included in the idiopathic inflammatory myopathies. Cutaneous manifestations are a prominent part of the condition: some skin signs in DM are common to most patients, while other signs are encountered infrequently. A number of features are pathognomic for DM. The demonstration of myositis-specific antibodies (MSAs) in DM has extended the ability to define phenotypic subgroups. It appears that the presence of certain MSAs confers susceptibility to specific clinical features, an association which reveals a serotype-phenotype relationship. In this review article we have provided a detailed summary of common and under-recognized cutaneous manifestations of DM.

Occupational Localized Cutaneous Argyria With Pseudo-Ochronosis in a Jeweler.

ABSTRACT: A case of localized argyria in a 36-year-old female jeweler is described who presented with 2 discrete and asymptomatic bluish-black pigmented macules on the pulp of her left middle finger. A skin biopsy from both lesions demonstrated deposition of brown/black pigmented granules along the basement membrane zone of eccrine glands, blood vessels, nerves, and the dermo-epidermal junction fully in keeping with silver deposition. In addition, there was yellow-brown deposition seen within the interstitial dermis mimicking an early form of ochronosis, so called "pseudo-ochronosis." This latter feature is rarely described in cases of argyria. Transmission electron microscopy and energy dispersive x-ray spectroscopy confirmed the presence of electron dense particles up to 150 nm in diameter and the presence of silver, respectively. On further questioning, the patient had a history of localized and chronic exposure to silver, which specifically involved holding and manipulating silver wires and rings over the left middle finger. This case highlights an unusual and rare presentation of localized argyria in a jeweler. In addition, our case showed preferential silver deposition on dermal elastic fibers which has not been previously described in the literature.

Knuckle pads: an ancient disease frequently misdiagnosed because of minimal modern attention.

Knuckle pads are benign painless papules and nodules that most commonly appear on the extensor surfaces of the proximal interphalangeal joints. Knuckle pads are frequently misdiagnosed due to their location overlying joints, which can lead to costly interventions and patient discomfort for a relatively harmless condition. We describe a 44-year-old woman who presented with mildly painful nodules on multiple bilateral proximal interphalangeal joints. The patient did not have a family history of fibromatosis and the rheumatoid factor was negative. Histology showed mild epidermal hyperkeratosis, papillomatosis, and acanthosis with a deep dermal, poorly circumscribed, proliferative nodule made of spindled myofibroblasts without cytological atypia. The diagnosis of knuckle pads was established based on the clinical and morphological presentation of the nodules. Treatment with intralesional triamcinolone acetonide injection produced significant clinical improvement. Our findings highlight the challenging diagnosis of knuckle pads and the importance of increasing the familiarity of knuckle pads in modern medical practice.

Adult colloid milium is clinically distinguishable from its histopathologic mimic cutaneous amyloidosis.

Colloid milium, also known as colloid degeneration of the skin or dermal hyalinosis, is a cutaneous deposition disease that presents as three subtypes: juvenile, nodular, and adult. Adult colloid milium is characterized by amyloid-like depositions in the dermis, mimicking cutaneous amyloidosis histologically. A 70-year-old man presented with lesions on the sun-exposed skin of the face, dorsal hands, and dorsal forearms resembling adult colloid milium. A punch biopsy was performed on the left zygoma and histopathological features were consistent with this diagnosis, though cutaneous amyloidosis was considered. A case of adult colloid milium is presented to emphasize the clinical and histopathologic differentiation from cutaneous amyloidosis.

Milia-like calcinosis cutis in Down syndrome: a new case with a review of the literature.

We report an 11-year-old girl who presented with white papules on the dorsal and palmar region of the hands bilaterally. The parents reported that the lesions had appeared four months before and some had resolved spontaneously. The girl was suffering from celiac disease, Down syndrome, and alopecia areata treated with topical corticosteroids. At the first visit, the girl presented with alopecia areata, corticosteroid acne, and a dozen white papules located on the hands. On dermoscopy, a whitish structureless area was seen. Histological examination showed the presence of calcium deposits without tissue damage, thus confirming the diagnosis of milia-like idiopathic calcinosis cutis. At 6-month follow up, the lesions had completely disappeared. Milia-like idiopathic calcinosis cutis is a benign cutaneous disorder consisting of calcium deposits in an apparently undamaged dermis and is typically associated with Down syndrome. Up to a quarter of patients have coexisting syringomas. The milia-like papules tend to self-resolve as patients reach adulthood, so a wait-and-see approach is recommended.

Atypical paronychia: don't forget herpesvirus.

Paronychia is usually caused by bacterial infections. Herpetic whitlow is an acute infection of the fingers or toes caused by herpes simplex viruses and it typically presents with vesicles. We report the case of a 78-year-old woman with gingivostomatitis and atypical paronychia in several fingers without blisters.

Onycholemmal variant of keratoacanthoma centrifugum marginatum as an expression of mutated committed stem cells in a conceptual pathway.

We describe a 43-year-old woman with a 10-year history of grossly hyperkeratotic nodules which progressively extended over the right ring finger. These involuted leaving pale, atrophic skin in their wake. At presentation, the advancing border had an arciform series of nodules in the pattern of keratoacanthoma centrifugum marginatum. The presence of filiform keratinisation that encased the nail plate, gross onychogryphotic masses of keratin on the ventral finger surface and a flat nail-like plate of keratin on the dorsal finger surface were distinctive features. Skin biopsy showed epidermal acanthosis, gross papillomatous cutaneous horn formation that had onycholemmal features. The pathology differed from keratoacanthoma and was not crateriform or infundibulocystic. Although HPV was not detected on immunohistochemistry, pathogenesis may still represent an HPV-related transfection of onycholemmal keratin committed stem cells producing an onycholemmal variant of keratoacanthoma centrifugum marginatum. A conceptual model linked to advances in follicular stem cell biology is formulated to explore this case.

Hand-foot skin reaction with primarily dorsal involvement in a patient with metastatic renal cell carcinoma on cabozantinib.

A 61-year-old man with metastatic renal cell carcinoma on cabozantinib developed hand-foot skin reaction with predominantly dorsal involvement including painful violaceous plaques over the joints and keratotic yellow plaques on the palmar fingers. The medication was discontinued with resolution of the plaques and later reinitiated at a lower dose uneventfully.

Infrared spectroscopy as a novel tool to diagnose onychomycosis.

BACKGROUND: The determination of causative organisms of onychomycosis is still not optimal. There remains a need for a cheap, fast and easy-to-perform diagnostic tool with a high capacity to distinguish between organisms. OBJECTIVES: To determine whether attenuated total-reflectance Fourier transform infrared (ATR-FTIR) spectroscopy can detect and differentiate causative agents in culture-based, ex vivo nail and in vivo nail models. METHODS: A methodological study was conducted. Both the ex vivo nail model and in vivo pilot study were carried out in an academic university hospital. RESULTS: Analysis of cultured fungi revealed spectral differences for dermatophytes (1692-1606 and 1044-1004 cm-1 ) and nondermatophytes and yeasts (973-937 cm-1 ), confirmed by dendrograms showing an excellent separation between samples from different genera or species. Exploration of dermatophytes, nondermatophytes and yeasts growing on ex vivo nails exposed prominent differences from 1200 to 900 cm-1 . Prediction models resulted in a 96·9% accurate classification of uninfected nails and nails infected with dermatophytes, nondermatophytes and yeasts. Overall correct classification rates of 91·0%, 97·7% and 98·6% were obtained for discrimination between dermatophyte, nondermatophyte and yeast genera or species, respectively. Spectra of in vivo infected and uninfected nails also revealed distinct spectral differences (3000-2811 cm-1 , 1043-950 cm-1 and 1676-1553 cm-1 ), illustrated by two main clusters (uninfected vs. infected) on a dendrogram. CONCLUSIONS: Our data suggest that ATR-FTIR spectroscopy may be a promising, fast and accurate method to determine onychomycosis, including identification of the causative organism, bypassing the need for lengthy fungal cultures.

Onychomycosis: Clinical overview and diagnosis.

Onychomycosis is a fungal nail infection caused by dermatophytes, nondermatophytes, and yeast, and is the most common nail disorder seen in clinical practice. It is an important problem because it may cause local pain, paresthesias, difficulties performing activities of daily life, and impair social interactions. In this continuing medical education series we review the epidemiology, risk factors, and clinical presentation of onychomycosis and demonstrate current and emerging diagnostic strategies.

Current and emerging therapies for hand eczema.

Although hand eczema (HE) and chronic hand eczema (CHE) are common conditions with significant disease burden, they traditionally have had limited treatment options beyond topical and short-term systemic corticosteroids. We reviewed published and preliminary evidence on the current and emerging topical and systemic therapeutic agents for HE and CHE. The etiologies of various HE subtypes are discussed, and remaining knowledge and practice gaps are highlighted to encourage further investigations. A comprehensive search of ClinicalTrials.gov and PubMed was completed for clinical trials that utilized known and emerging treatment options for HE and CHE. Several agents that target IL-4 and IL-13 signaling, keratinocyte proliferation, inflammatory cytokine production, bacterial protein synthesis, and inflammatory mediator (TNF-α, JAK1, JAK2, and JAK3) proliferation are shown to be involved in the pathogenesis of CHE. Systemic agents include dupilumab, alitretinoin, acitretin, cyclosporine, azathioprine, and probiotics. Topical agents include delgocitinib, retapamulin, halometasone/triclosan, calcipotriol/betamethasone, tacrolimus, and pimecrolimus. These modalities have demonstrated varying degrees of clinical efficacy, evaluated by subjective assessments and scoring indexes. Targeted therapies are emerging for HE, but options are still limited, partially due to our narrow understanding of this heterogeneous condition. Additional and targeted therapeutic options are needed to match the rising prevalence and burden of HE. KEYPOINTS: Hand eczema (HE) is a heterogenous dermatosis with limited therapeutic options due to a lack of international guidelines regarding classification of HE subtypes and treatment. This review discusses current and emerging topical and systemic agents and their efficacies in the treatment of different types of hand eczema.

Chronic hand eczema and hepatogenic pruritus with good response to apremilast.

Hand eczema (HE) presents a high prevalence and severe impact on the quality of life of the patients. HE is a worldwide problem recognized for his high socio-occupational involvement. Treatment is mainly based on adequate recommendations for hand care together with topical anti-inflammatory treatments. Frequently, patients require systemic treatments such alitretinoin, among others. The comorbidities of the patients and the side effects of the treatments sometimes require prescribing other treatments off label on the technical data. This role in immunomodulation has led to the development of new studies that investigate what role does apremilast have in eczematous diseases, such as atopic dermatitis. We present this case for the quick and complete response of HE with apremilast, and the effective control of hepatogenic pruritus. These finding open a new possibility and alternative treatment of this complex and difficult of control with the treatments already described in the literature.