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Indian Journal of Pathology &... Mar 2024Darier disease (DD) is a rare genodermatosis. Literature on this topic is overwhelmingly dominated by case reports with rare clinical presentations, which have mentioned...
Darier disease (DD) is a rare genodermatosis. Literature on this topic is overwhelmingly dominated by case reports with rare clinical presentations, which have mentioned the histopathologic features briefly. The aim of this study was to document the histopathology of DD. Skin biopsies diagnosed as Darier disease based on clinicopathologic correlation over 12 years were reviewed for various epidermal and dermal features. There were 16 patients included, who most commonly presented in the third decade, with slight female predilection. The most common clinical presentation was hyperpigmented, hyperkeratotic, papules and plaques (91%), with 69% affecting the trunk. In addition to the classic suprabasal acantholytic clefts, we noted some unusual features: absence of parakeratosis (19%), a cornoid lamella-like pattern (62%), follicular acantholysis (13%) and multiple foci of involvement within a single biopsy (63%). Features such as the presence of dyskeratotic cells and minimal dermal lymphocytic infiltrates were concordant with previous literature. The limitation of this study was the small sample size. To conclude, pathologists must be aware of the variations in histopathology of Darier's disease, especially when challenged with atypical clinical presentations. The Darier-like pattern is met within several acantholytic diseases, and clinicopathologic correlation has the last word in arriving at a diagnosis.
PubMed: 38563701
DOI: 10.4103/ijpm.ijpm_610_23 -
Journal of Clinical Medicine Jan 2023Psoriasis is a common chronic, inflammatory skin disease characterised by keratinocyte hyperproliferation, parakeratosis, and T-cell infiltration. Adipose tissue has an...
Psoriasis is a common chronic, inflammatory skin disease characterised by keratinocyte hyperproliferation, parakeratosis, and T-cell infiltration. Adipose tissue has an endocrine function, producing an abundance of cytokines and adipokines. It has also been described that the major adipokines, leptin, resistin, and adiponectin, may be involved in the pathogenesis of psoriasis. The aim of the study was to examine the plasma levels of adiponectin, leptin, and resistin in patients with psoriasis and their correlations with disease activity parameters: Psoriasis Activity Severity Index (PASI), Dermatology Life Quality Index (DLQI), and Body Surface Area (BSA) index, as well as selected clinical parameters. The study included 53 patients with the plaque type and 31 healthy controls. The plasma concentrations of adiponectin were significantly lower in patients with psoriasis (p < 0.001) than in the control group. The plasma concentrations of leptin were higher in patients with psoriasis, however, due to high intra-patient variability of leptin plasma concentrations these differences did not reach statistical significance (p = 0.2). The plasma concentrations of resistin were significantly increased in patients with psoriasis compared to healthy controls (p = 0.02). There were no statistically significant correlations between adiponectin and leptin plasma concentrations and values of PASI, DLQI, and BSA. The resistin plasma concentrations correlated significantly with DLQI values. Additionally, we examined the correlations between adiponectin, leptin, and resistin plasma concentrations, and selected clinical parameters. Plasma concentrations of adiponectin correlated significantly with CRP values and ALT values. Leptin plasma concentrations correlated significantly with creatinine values. The results of our study confirm the role of adiponectin, leptin, and resistin in the pathogenesis of psoriasis.
PubMed: 36675592
DOI: 10.3390/jcm12020663 -
Clinical features, histology, and treatment outcomes of granular parakeratosis: a systematic review.International Journal of Dermatology Aug 2022Granular parakeratosis is a rare disorder characterized by erythematous-brown hyperkeratotic papules and erythematous patches with scaling, occurring predominantly in...
BACKGROUND
Granular parakeratosis is a rare disorder characterized by erythematous-brown hyperkeratotic papules and erythematous patches with scaling, occurring predominantly in the flexures and sites of occlusion. While the exact underlying pathogenesis remains unknown, there has been a wide variety of precipitating factors and treatment options reported in the literature.
OBJECTIVE
We systematically reviewed and identified precipitants of granular parakeratosis, as well as its clinical and histologic features and treatment outcomes.
METHOD
A comprehensive literature search was conducted using MEDLINE and Embase in March 2021.
RESULTS
A total of 60 studies with 129 patients were included for analysis. An inciting factor was identified in 53.4%, the most common being topical agents including zinc oxide (17.1%), deodorant/antiperspirant (15.5%), and those containing benzalkonium chloride (7.0%). The majority presented with bilateral (68.2%) eruption of hyperkeratotic papules or erythematous patches and plaques, most frequently involving the axilla (56.5%). The prevailing histologic feature was retained keratohyalin granules within the stratum corneum in punch biopsy (97.2%) and curette (100%) specimens. Treatment options with reported success ranged from topical corticosteroids and systemic antibiotics to surgical interventions.
CONCLUSION
We provide a systematic review of reported precipitants, clinical features, and treatment options that clinicians should consider when granular parakeratosis is considered.
Topics: Dermatologic Agents; Humans; Keratosis; Parakeratosis; Skin; Treatment Outcome
PubMed: 35094385
DOI: 10.1111/ijd.16107 -
Dermatology Online Journal Feb 2021Epidermolytic ichthyosis (EI, OMIM 113800) is a rare autosomal dominant keratinization disorder that is caused by keratin 1 or 10 gene mutation. It can be classified...
Epidermolytic ichthyosis (EI, OMIM 113800) is a rare autosomal dominant keratinization disorder that is caused by keratin 1 or 10 gene mutation. It can be classified clinically based on the presence of palmoplantar hyperkeratosis involvement and extent of skin involvement. The diagnosis is made by clinical and histopathological examinations that can be confirmed by genetic testing. We present a 2-year-old girl who presented with erythematous and thick scaling skin. Her condition began at birth as multiple flaccid blisters that would easily break into erosions. There was no history of similar condition nor consanguinity within her family. Skin examination revealed diffuse erythematous skin covered with thick scales and erosion, predominantly on her face, extremities, palms, and soles. The skin histopathology examination showed diffuse parakeratosis with vacuolar and granular degeneration within granular and spinous layers along the epidermis. She was diagnosed with generalized EI with palmoplantar hyperkeratosis based on the clinical and histopathological examinations. Clinical improvement was observed after a one-month treatment with mupirocin cream, sodium bicarbonate bath, and moisturizer after bathing.
Topics: Child, Preschool; Female; Humans; Hyperkeratosis, Epidermolytic; Keratoderma, Palmoplantar
PubMed: 33818988
DOI: No ID Found -
Journal of Cutaneous Pathology May 2020Pathogenic mutations in caspase recruitment domain-containing protein 14 (CARD14) lead to CARD14-associated papulosquamous eruption, which shares clinicopathologic...
BACKGROUND
Pathogenic mutations in caspase recruitment domain-containing protein 14 (CARD14) lead to CARD14-associated papulosquamous eruption, which shares clinicopathologic findings with psoriasis and pityriasis rubra pilaris. We aimed to describe distinguishing histopathologic features of CARD14-associated papulosquamous eruption.
METHODS
This retrospective study examined the histopathologic features of specimens from patients with confirmed CARD14-associated papulosquamous eruption and adult patients with plaque psoriasis and pityriasis rubra pilaris.
RESULTS
Lesional skin biopsies from patients with CARD14-associated papulosquamous eruption consistently showed alternating checkerboard parakeratosis and orthokeratosis, acanthosis without acantholysis, and dilated vessels in the dermal papillae, with some cases also showing follicular plugging.
CONCLUSION
CARD14-associated papulosquamous eruption has a range of findings, with a predominance of features typically associated with pityriasis rubra pilaris.
Topics: Adult; Aged; Biopsy; CARD Signaling Adaptor Proteins; Case-Control Studies; Exanthema; Guanylate Cyclase; Humans; Membrane Proteins; Middle Aged; Mutation; Pityriasis Rubra Pilaris; Proteins; Psoriasis; Retrospective Studies; Skin; Skin Diseases, Papulosquamous
PubMed: 31849081
DOI: 10.1111/cup.13633 -
Head and Neck Pathology Sep 2023Verruciform xanthoma (VX) is an uncommon, benign epithelial lesion of the oral mucosa. While this entity can also present extraorally, including on the skin and in...
BACKGROUND
Verruciform xanthoma (VX) is an uncommon, benign epithelial lesion of the oral mucosa. While this entity can also present extraorally, including on the skin and in anogenital areas, the variation in its histologic features in extraoral sites is not yet well defined. Differences in the demographics and morphologic features of oral versus extraoral VX were assessed to help facilitate the accurate diagnosis and management of this lesion.
METHODS
After obtaining IRB approval, 110 cases of diagnosed VX were retrospectively collected from our institutional archives spanning from 2000 to 2022. Patient age, gender, available medical history, lesion appearance, and duration were obtained for each case.
RESULTS
The median age was 55 years (range 13-86) with a male-to-female ratio of 1.2:1. The most common oral sites, in descending order, were the palate (n = 24, 22%), buccal mucosa (n = 18, 16%), gingiva (n = 16, 15%), and tongue (n = 13, 12%). Extraoral sites comprised 9% of all lesions, including the scrotum (9), vulva (2), cheek (1), wrist (1), gluteal region (1), and abdominal wall (1). The median size for all lesions was 6.0 mm, and extraoral lesions were associated with a 6.7 mm larger size compared to oral lesions (B ± SE: 6.7 ± 2.5 cm, p = 0.01). The lesions were most frequently pink or white in color and often described as papillary, pedunculated, verrucous, and/or exophytic. Microscopically, the presence of wedge-shaped parakeratosis, keratin projections above the epithelium/epidermis, and associated inflammation significantly differed between oral and extraoral lesions. Prominent wedge-shaped parakeratosis (p = 0.04) and keratin projections above the epithelium/epidermis (p < 0.001) were more prevalent in extraoral lesions. There was no significant link between keratin projections and epithelial atypia (p = 0.44).
CONCLUSIONS
Familiarity with the broad morphological spectrum of VX, including the presence and degree of wedge-shaped parakeratosis, keratin projections above the epithelium/epidermis, and associated underlying inflammation, will be helpful in diagnosing it in unusual locations.
Topics: Humans; Male; Female; Adolescent; Young Adult; Adult; Middle Aged; Aged; Aged, 80 and over; Retrospective Studies; Parakeratosis; Mouth Diseases; Xanthomatosis; Keratins; Genitalia
PubMed: 37420145
DOI: 10.1007/s12105-023-01568-4 -
Annales de Pathologie Jan 2022We report a case of a vulvar verruciform xanthoma. Verruciform xanthoma is a rare benign lesion that occurs most commonly on the oral and genital mucosa. Under the...
We report a case of a vulvar verruciform xanthoma. Verruciform xanthoma is a rare benign lesion that occurs most commonly on the oral and genital mucosa. Under the microscope, this lesion displays acanthotic papillary epidermis with parakeratosis that extends deep into the epithelium, elongated rete ridges and xanthomatous cells in the papillary dermis. Vulvar lesions almost always occur in a local pathological context (lichen planus or sclerosus). It is important to be aware of this entity as it can mimic squamous carcinoma.
Topics: Dermis; Humans; Keratosis; Xanthomatosis
PubMed: 33478785
DOI: 10.1016/j.annpat.2020.12.006 -
Journal of Cutaneous Pathology Jul 2021
Topics: Aged; Carcinoma in Situ; Carcinoma, Squamous Cell; Female; Humans; Male; Middle Aged; Parakeratosis; Polyomavirus Infections; Skin Neoplasms
PubMed: 33813761
DOI: 10.1111/cup.14022 -
Journal of Cosmetic Dermatology Aug 2021Baboon syndrome is a rare, type IV hypersensitivity reaction causing a maculopapular rash. Tamoxifen is an antineoplastic agent, working as an estrogen receptor...
BACKGROUND
Baboon syndrome is a rare, type IV hypersensitivity reaction causing a maculopapular rash. Tamoxifen is an antineoplastic agent, working as an estrogen receptor antagonist, also called a selective estrogen receptor modulator. A variety of rashes were reported with Tamoxifen use to-date except baboon syndrome. The Tamoxifen-induced baboon syndrome seems to be reversible, as discontinuation of the drug improves clinical outcomes.
AIM
Herein, we present the first case of Tamoxifen-induced baboon syndrome which occurred 8 years after initiation of Tamoxifen use.
PATIENTS
A 44-year-old woman presented with papulovesicular eruption on her body and erythema on her face for a duration of 6 months. There was no evidence of ocular or mucosal involvement. She was diagnosed with breast cancer and treated with tamoxifen 10 mg twice daily over the past 8 years. She was not taking other medications or over-the-counter supplements at the time of presentation. The patient underwent urgent skin biopsies of two lesions on her buttock and thigh. No organisms were seen on Gram stain. The patient's skin biopsy revealed extensive hyperorthokeratosis, minimal parakeratosis, hypergranulosis, and lichenoid interface dermatitis in the irregularly acanthotic epidermis supporting diagnosis of fixed drug eruption. Following a multidisciplinary discussion, the patient was diagnosed with baboon syndrome or symmetrical drug-related intertriginous and flexural exanthema (SDRIFE) associated with Tamoxifen.
RESULTS
Hence, Tamoxifen was immediately discontinued and treated with oral steroid along with topical agents. She showed improvement of clinical abnormalities within days after discontinuation of Tamoxifen.
CONCLUSIONS
Given the widespread use of Tamoxifen in the management of patients with breast cancer, it is important that healthcare professionals monitor for rare, however clinically significant, and potentially life-threatening dermatological manifestations of Tamoxifen use, such as baboon syndrome.
Topics: Adult; Animals; Drug Eruptions; Exanthema; Female; Humans; Papio; Syndrome; Tamoxifen
PubMed: 33253493
DOI: 10.1111/jocd.13863 -
Journal of Cutaneous Pathology May 2024
Topics: Humans; Parakeratosis; Axilla
PubMed: 38126159
DOI: 10.1111/cup.14580