Keratotic Plaques Research Articles

Application of dapsone 5% gel in a patient with dermatitis herpetiformis

Application of dapsone 5% gel in a patient with dermatitis herpetiformis

Skin lesions in histoplasmosis

Histoplasmosis is a granulomatous infection caused by Histoplasma capsulatum, a dimorphic fungus. It is distributed worldwide and prevalent in certain regions of North and Central America. Pulmonary involvement is the most common clinical presentation. Cutaneous manifestations are reported to occur in 10% to 25% of AIDS patients with disseminated histoplasmosis. The skin lesions are polymorphic papules, plaques with or without crusts, pustules, nodules, mucosal ulcers, erosions, punched out ulcers, lesions resembling molluscum contagiosum, acneiform eruptions, erythematosus papules and keratotic plaques, purpuric lesions, and localized and generalized vegetant forms of dermatitis, sometimes an eruption similar to rosacea, keratotic papules with transepidermal elimination, polymorphous erythema, erythroderma syndromes, pyoderma gangrenosum, panniculitis, diffuse hyperpigmentation, abscesses, and cellulitis.

Intracellular Proton-mediated Activation of TRPV3 Channels Accounts for the Exfoliation Effect of α-Hydroxyl Acids on Keratinocytes

α-Hydroxyl acids (AHAs) from natural sources act as proton donors and topical compounds that penetrate skin and are well known in the cosmetic industry for their use in chemical peels and improvement of the skin. However, little is known about how AHAs cause exfoliation to expose fresh skin cells. Here we report that the transient receptor potential vanilloid 3 (TRPV3) channel in keratinocytes is potently activated by intracellular acidification induced by glycolic acid. Patch clamp recordings and cell death assay of both human keratinocyte HaCaT cells and TRPV3-expressing HEK-293 cells confirmed that intracellular acidification led to direct activation of TRPV3 and promoted cell death. Site-directed mutagenesis revealed that an N-terminal histidine residue, His-426, known to be involved in 2-aminoethyl diphenylborinate-mediated TRPV3 activation, is critical for sensing intracellular proton levels. Taken together, our findings suggest that intracellular protons can strongly activate TRPV3, and TRPV3-mediated proton sensing and cell death in keratinocytes may serve as a molecular basis for the cosmetic use of AHAs and their therapeutic potential in acidic pH-related skin disorders.

Papillon- Lefevre Syndrome: Report of a case and its management

Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis. The etiopathogenesis of the syndrome is relatively obscure and immunologic, genetic or possible bacterial etiologies have been proposed. Due to the vast degree of periodontal breakdown involved at such an early age, the dental surgeon is often the first to diagnose the syndrome. This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lefèvre Syndrome. Key words:Papillon-Lefèvre Syndrome, palmoplantar keratoderma, rapidly progressing periodontitis.

Nephrogenic systemic fibrosis: A case report and review on Japanese patients

Nephrogenic systemic fibrosis is a fibrosing disorder that affects patients with advanced renal dysfunction and is associated with gadolinium-based contrast media. As the number of reports increase, it is becoming clear that its clinical course and symptoms are various. We describe a 14-year-old boy and 71-year-old man with nephrogenic systemic fibrosis and review the Japanese cases documented thus far. In Japan, there are only 10 cases definitely associated with gadolinium, whereas over 500 cases have been recorded worldwide. We found a remarkable difference in clinical signs among Japanese cases. Some cases showed keratotic papules or plaques on the extremities. This group tended to develop symptoms after a shorter interval following gadolinium exposure. The remainder of the cases presented glossy and smooth surfaces, with symptoms tending to develop after a longer interval following their last gadolinium exposure. The discrepancies between the actual and the estimated number of patients, and the various clinical manifestations can be explained by the comparatively smaller dosage of gadolinium-based contrast agents commonly administrated in Japan, in contrast to the higher dosages administrated in the USA and Europe.

Olmsted syndrome in a baby girl and her mother

A 2-month-old baby girl developed universal keratotic plaques soon after birth. Physical examination revealed well-defined, dark erythematous, keratotic plaques with thick scales and mild infiltration at the periorbital, perioral, perianal and vulvar regions, as well as deep fissures of both hands and feet covered with thick yellowish crusts. Another case was a 24-year-old female, the mother of the baby, who presented with hyperkeratotic plaques at perioral and perianal regions, congenital alopecia universalis, mutilation of fingers and toes with massive thick keratotic yellow crusts and scales. Histopathology of skin lesions from the gluteal region of the baby showed psoriasiform hyperplasia of the epidermis, slight inflammatory infiltration of dermal papillae and superficial dermal perivascular regions. Immunohistochemistry demonstrated the positive staining for acidic keratin (AE1) in the prickle cell layer and granular layer and for CK10 in the upper prickle cell layer and granular layer. Electron microscopy showed increased cell space and decreased tonofilament. Both the baby girl and her mother were diagnosed with Olmsted syndrome. Key words: Keratoderma, palmoplantar; Olmsted syndrome; Case reports [Publication type]

Acrokeratosis paraneoplastica (Bazex syndrome): Report of a case associated with small cell lung carcinoma and review of the literature

Acrokeratosis paraneoplastic (Bazex syndrome) is a rare, but distinctive paraneoplastic dermatosis characterized by erythematosquamous lesions located at the acral sites and is most commonly associated with carcinomas of the upper aerodigestive tract. We report a 58-year-old female with a history of a pigmented rash on her extremities, thick keratotic plaques on her hands, and brittle nails. Chest imaging revealed a right upper lobe mass that was proven to be small cell lung carcinoma. While Bazex syndrome has been described in the dermatology literature, it is also important for the radiologist to be aware of this entity and its common presentations.

Squamous cell carcinoma presenting as multiple discharging sinuses on the chin

Squamous cell carcinoma (SCC) usually presents as a raised, firm, pink to skin-coloured keratotic papule, plaque or ulcer, arising on sun-exposed skin. We report an unusual case of a 65-year-old male patient who presented with a 1-year history of multiple discharging sinuses on his chin, which on investigation was found to be an SCC. A malignancy should be considered in the differential diagnoses of patients presenting with multiple discharging sinuses on the chin. We discuss other possible diagnoses for sinuses presenting on the face.

Frictional Keratoses on the Facial Attached Gingiva Are Rare Clinical Findings and Do Not Belong to the Category of Leukoplakia

To investigate the clinical and histologic features of frictional keratoses located exclusively on the facial attached gingiva and establish whether these belong to the category of leukoplakia. Over a period of 15 years, 159 patients presenting with oral keratotic plaques, located exclusively on the facial attached gingival mucosa, excluding the edentulous alveolar ridge and retromolar pad area, were retrospectively selected. Clinical and histologic features and the symptoms and progression of these lesions were carefully assessed. The presence of oral frictional keratosis located exclusively on the facial attached gingival mucosa was clinically and immunohistologically diagnosed in 14 of 159 patients (8.8%). Eleven patients (78.5%) showed unilateral involvement, whereas 3 patients (21.5%) had bilateral involvement. The disappearance of the lesions was accomplished in only 9 of 14 patients, resulting from discontinuation of bad habits. Clinically, these lesions appeared as distinct, sharply demarcated, isolated, asymptomatic, homogeneous whitish-plaques that were neither removable nor painful. The plaques did not create discomfort, change shape, or develop into malignancy. Histologically, these plaques showed features superimposable to those present in benign alveolar ridge keratoses. The results highlighted that frictional keratoses on the facial attached gingival mucosa 1) are rare findings, 2) clinically appear as "true leukoplakia" but histologically have the same features as benign alveolar ridge keratoses, 3) have no propensity for malignant transformation, 4) have a good prognosis, and 5) have a specific cause, and resolution is accomplished if the frictional element is eliminated. Thus, these must be removed from the category of leukoplakia.

Actinic Cheilitis: A Case Report and a Review of the Literature

In actinic cheilitis, the current view is that the keratinocytes have undergone transformation forming a field of epithelium with the potential for neoplastic transformation. Clinical features include diffuse and poorly demarcated atrophic, erosive or keratotic plaques that may affect some parts of, or the entire vermilion border. Fair-complexioned people, those with albinism and people with eversion of the lip are all subject to actinic cheilitis. Prophylactic measures against all forms of sunlight-induced lesions must include limitation of exposure to the sun during peak sunlight hours, the use of appropriate protective clothing, and the use of a sunscreen cream. In this article, a case of albinism is used to illustrate the nature of actinic cheilitis, its clinical features and its treatment.

A Case of Familial Comedonal Darier's Disease

Darier's disease is a genetic disorder of keratinization with autosomal dominant inheritance. Its appearance is usually in the form of greasy, crusted, keratotic yellow-brown papules and plaques found particularly on seborrheic areas of the body. However, there are some clinical variants showing atypical skin lesions. Here we report an unusual case of Darier's disease, which mainly showed prominent comedonal papules over the face.

Porokeratotic Eccrine Ostial and Dermal Duct Nevus Showing Partial Remission by Topical Photodynamic Therapy

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon, benign dermatosis and is characterized by asymptomatic grouped keratotic papules and plaques with a linear pattern on the extremities. Various treatments, including topical steroids, topical calcipotriol, topical 5-fluorouracil, retinoid, cryotherapy, and carbon dioxide laser ablation have been tried for PEODDN, but the results were unsatisfactory. Recently, topical photodynamic therapy (PDT) has been shown to be effective for various cutaneous disorders. We report a case of PEODDN showing partial remission with PDT using topical 5-aminolevulanic acid in a 4-year-old girl.

Porokeratotic eccrine ostial and dermal duct nevus

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare variant of porokeratosis with characteristic histological feature of cornoid lamella involving the acrosyringium. We report a classic case of a 20-year-old male, who clinically presented to us with keratotic papules and plaque with pits, few having comedo like plugs, on right palm and sole since 1 year of age. A punch biopsy from palm was diagnostic as well as confirmatory showing cornoid lamella involving an eccrine duct which is the characteristic histopathological feature of PEODDN.

Keratotic papules and plaques in an adolescent with Down syndrome

Conflict of interest: none declared. A 16‐year‐old adolescent boy presented with multiple asymptomatic skin lesions involving mainly the arms. The lesions started appearing around 1 month previously, and had been persistent since then. Treatment with topical steroids did not improve the condition. The patient’s family history was negative for a similar condition. The patient has Down syndrome (DS), which was diagnosed during early childhood. On physical examination, multiple erythematous keratotic papules and plaques were seen, with some assuming an annular configuration on the arms (Fig. 1a). A punch biopsy specimen from a representative lesion was sent for histopathological examination (Fig. 1b–d). ... On histopathological examination, focal parakeratosis was seen, overlying an area of epidermal acanthosis with epidermal transepithelial elimination of basophilic material composed of neutrophils, nuclear debris and eosinophilic fibres. The latter were seen to enter the epidermis through an epithelial channel from the underlying papillary dermis. A Verhoeff–van Gieson stain confirmed theses to be elastic fibres.

Fixed keratotic plaques and migrating erythema

From D Fund Conf Repr G 17 A 43-year-old woman presented with a progressive rash that had been present since early infancy. She noted fixed, dry, scaling plaques and fluctuating red patches that changed daily; the latter caused discomfort or burning sensations, especially after exposure to damp or cold weather. Her father and two brothers are similarly affected. The physical examination revealed hyperpigmented and psoriasiform plaques with peripheral erythema and hypopigmentation on the dorsal surfaces of her hands, legs, arms, and flanks (Fig 1 to 3).

Four novel ATP2A2 mutations in Slovenian patients with Darier disease

Darier disease (DD) is an autosomal dominant genodermatosis caused by mutations in the ATP2A2 gene. It has been reported that depletion of Ca(2+) stores within the endoplasmic reticulum of keratinocytes is associated with impaired cell cycle regulation and terminal differentiation. Mechanical stress, heat, or UV irradiation might delay cell cycle exit and permit progression into the quiescent stage without repair. When there is associated DNA damage, this can lead to an accumulation of secondary somatic mutations and possible clonal proliferation of damaged keratinocyes within keratotic papules and plaques. We sought to present clinical, demographic, and genetic analysis of the cohort of Slovenian patients with DD, which represents 52% of DD patients in the country. We examined 28 Slovenians with DD and screened genomic DNA for ATP2A2 mutations and RNA for splice site mutations. The estimated prevalence of the disease in Slovenia is 2.7/100.000. We identified 7 different ATP2A2 mutations, 4 of which are novel: A516P, R559G, 463-6del6, and 1762-6del18. We also found two previously described polymorphisms in intron XVIII (2741 + 54 G>A) and in exon 15 (2172 G>A; A724A), with allele frequencies of 64.15% and 11.32%, respectively. There was a history of perceptive deafness in two DD patients from two families. Analysis of SERCA2 expression, measurements of Ca(2+) uptake and their influence on desmosomal assembly in vitro would add additional value to the study. Although single-stranded conformational analysis (SSCP) is a common and accepted method for screening for the presence of mutations, it does miss 10% to 20% of mutations. We identified 4 novel ATP2A2 mutations in Slovenian patients with DD. Deafness seems to be a new phenotypic characteristic of DD patients.

Identification a novel missense mutation p.R761L in Chinese patients with Darier’s disease

Darier's disease (DD, MIM 124200) is an autosomal dominant inherited disease. It is usually present in teenagers or adults with multiple keratotic papules or plaques in seborrheic areas. Pathogenic mutations in the ATP2A2 gene have been identified. It encodes the sarcoplasmic or endoplasmic reticulum Ca(2+) ATPase isoform 2 (SERCA2). Polymerase chain reaction and direct sequencing of the full coding sequence of ATP2A2 gene were performed to identify the mutation in this family. In this report, we identified a novel mutation of ATP2A2 gene in a Chinese family with DD. It is a novel heterozygous nucleotide G --> T transition at position 2,282 in exon 15 of the ATP2A2 gene. Our study expands the database on the ATP2A2 gene mutations in DD.

Dermoscopic Features of Eccrine Porocarcinoma Arising from Hidroacanthoma Simplex

Eccrine porocarcinoma is a rare cutaneous neoplasm that mainly affects elderly people and grows slowly over a long period of time but often experiences an accelerated growth phase. Eccrine porocarcinoma may arise de novo or evolve from a pre-existing benign eccrine poroma. We reported a 86-year-old Japanese woman presenting with two reddish-colored pendulated lesions on a keratotic light brown plaque on the right thigh. Dermoscopic examination of the light-brown plaque demonstrated many whitish globular structures in a light-brown background. At the two reddish-colored pendulated lesions, polymorphous and prominent vessel proliferation was observed together with irregularly shaped whitish negative network. Immunohistochemical study demonstrated a positive CEA staining at ductal structures and atypical clear cells of reddish nodules. A diagnosis of eccrine porocarcinoma arising in a pigmented hidroacanthoma simplex was eventually established, and the dermoscopic features of eccrine porocarcinoma from hidroacanthoma simplex was described for the first time.

Punctate keratotic papules and plaques over palm

Punctate keratotic papules and plaques over palm

Elastosis Perforans Serpignosa: Treatment with Liquid Nitrogen Cryotherapy and Review of the Literature

Elastosis perforans serpiginosa (EPS) is a rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis via transepithelial elimination. It is characterized by an eruption of small grouped hyperkeratotic papules in a serpiginous arrangement. This condition presents a therapeutic challenge as many treatments have been reported with inconsistent efficacy. This article reports a case of a 13-year-old male who presented to our outpatient clinic with a 1-year history of facial lesions showing multiple annular keratotic plaques with slight central atrophy. We report a case of EPS treated successfully with liquid nitrogen cryotherapy. Liquid nitrogen cryotherapy is well tolerated, with few side effects, and can be considered in the management of EPS.