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Cureus Jul 2023We present a case of seronegative amyopathic dermatomyositis (SADM). This clinical entity should be considered in the differential diagnosis of patients with recurring,...
We present a case of seronegative amyopathic dermatomyositis (SADM). This clinical entity should be considered in the differential diagnosis of patients with recurring, painful erythematous skin manifestations, and requires close monitoring for the development of neurological manifestations and malignancy. SADM is a rare autoimmune disease that affects the skin and muscles. It is considered a subtype of dermatomyositis (DM), which is a systemic autoimmune disease. The exact cause of SADM is not fully understood but is believed to involve a complex interplay between genetic, environmental, and immunological factors. The diagnosis of SADM is typically made based on clinical evaluation, blood tests, muscle biopsy, and skin biopsy. Treatment options for SADM may include corticosteroids, immunosuppressive drugs, and other supportive measures to manage symptoms and prevent disease progression. A 30-year-old female presented with symptoms of intermittent burning, painful rash primarily on the hands and face. Her medical history was remarkable for a six-year history of multifocal joint pain, chronic low back pain, and intermittent, painful recurring rash in the upper body (face, neck, and chest). Neurological examination revealed scalp tenderness and arthralgia in the upper extremities, with normal motor strength examination. Skin findings included described an erythematous rash on the arms and hands bilaterally. Skin punch biopsy showed compact orthokeratosis, atrophy of the epidermis, interface changes, and increased dermal mucin on the colloidal iron stain, which are suggestive of DM. Electromyography and nerve conduction study were normal. The MRI of the left thigh was normal. C3 and C4 levels were reduced. The extended muscle-specific myositis panel including MDA5 was negative. The patient was placed on a multidrug regimen, including methotrexate, hydroxychloroquine, and prednisone. Within one year of follow-up, she was found to have reductions in skin manifestation and flare-ups. Clinicians should consider amyopathic DM (ADM) in the differential diagnosis of patients with recurring, painful skin manifestations. This condition can be easily overlooked as the development of neurological sequelae may be present much later in the course. We highlight the need for a multi-disciplinary management approach for patients with this unique diagnosis. Close monitoring for the development of neurological manifestations and associated sequelae including malignancy is recommended.
PubMed: 37654955
DOI: 10.7759/cureus.42727 -
Cureus Jun 2023Neuroectodermal disease involves abnormalities that arise from the ectodermal origin, such as the nervous system, eyeball, retina, and skin. Due to the rarity of the...
Neuroectodermal disease involves abnormalities that arise from the ectodermal origin, such as the nervous system, eyeball, retina, and skin. Due to the rarity of the disease, it is often underdiagnosed or misdiagnosed. In this study, the researcher presents two cases of pediatric patients with no fetomaternal complications who presented with focal seizures as their initial complaint. During the examination, varying skin color pigmentation and an abnormal neurophysical examination were observed. Cranial imaging showed hemimegalencephaly and voltage asymmetry on EEG. Skin biopsy was performed on both cases, which revealed basketweave orthokeratosis. The combination of a triad of intractable epilepsy, developmental delay, and cutaneous lesion prompted the consideration of a neuroectodermal disease. The study shows two cases of hypomelanosis of Ito and nevus syndrome, both of which may be due to mTOR and RAS pathways, respectively.
PubMed: 37456443
DOI: 10.7759/cureus.40349 -
Indian Journal of Dermatology 2023Psoriasis is a chronic, immune-mediated skin disorder characterised by well-demarcated erythematous plaque with micaceous scale. Naevoid Blaschkoid psoriasis is an...
Psoriasis is a chronic, immune-mediated skin disorder characterised by well-demarcated erythematous plaque with micaceous scale. Naevoid Blaschkoid psoriasis is an unusual subtype that occurs along Blaschko lines. A two-year-old boy presented with erythematous scaly lesions distributed along the lines of Blaschko over the bilateral upper limb, the front of the chest, back, and the right lower limb. The lesions appeared four months ago and slowly progressed. There was no history of trauma, no skin lesions in other parts of the body, and no significant family history. Nail and mucosa were normal. Histopathology showed psoriasiform dermatitis with characteristics of psoriasis. The child was treated with topical clobetasol propionate 0.05% cream. Naevoid Blaschkoid psoriasis, in the absence of psoriatic lesion elsewhere on the body, is a rare manifestation. It has striking similarity with inflammatory linear verrucous epidermal nevus (ILVEN), both clinically and histologically. Naevoid psoriasis usually presents late, is asymptomatic or mildly pruritic, progresses rapidly, and responds favourably to antipsoriatic treatment. In contrast, ILVEN presents early, is intensely pruritic, slowly progressive, and is usually refractory to antipsoriatic treatment. Histologically, ILVEN demonstrates abruptly alternating areas of hypergranulosis with orthokeratosis, and parakeratosis with agranulosis. An inflammatory infiltrate is present in the upper dermis. Psoriasis presents with papillomatosis, acanthosis, and parakeratosis with absent or minimal granular layer. Immunohistochemical staining can be done in such doubtful cases. Involucrin would be detectable in psoriasis, but it is absent in ILVEN. Pathogenesis of linear psoriasis is unknown but might be explained by the concept of genetic mosaicism. Although rare, there have been a few reported cases of linear psoriasis occurring in early childhood.
PubMed: 37151253
DOI: 10.4103/ijd.ijd_894_22 -
Maedica Dec 2022Our previous research showed that, in a mice tail model for psoriasis, topical celecoxib might have an antipsoriatic effect by increasing granular layer development....
Our previous research showed that, in a mice tail model for psoriasis, topical celecoxib might have an antipsoriatic effect by increasing granular layer development. The aim of the present study is to assess the effect of three celecoxib concentrations using a mice model for psoriasis. Topical application of celecoxib (2%, 4% and 8%), with two negative controls (untreated and white soft paraffin as ointment base) and one positive control (tretinoin 0.05%), was performed for two weeks. The effect on granular layer development and epidermal thickness was measured on hematoxylin-eosin staining. The morphometric assessment was made by using mean orthokeratosis degree and mean epidermal thickness as main parameters. All celecoxib concentrations have significantly increased the mean orthokeratosis degree as a marker of an anti-psoriatic effect. Celecoxib 8% and 4% lead to a statistically significant increase in orthokeratosis degree when compared with celecoxib 2% and tretinoin 0.05%. Along with cyclo-oxygenase inhibition, other mechanisms of action of celecoxib are discussed. This study offers valuable information for future clinical trials with celecoxib as a topical anti-psoriasis agent.
PubMed: 36818260
DOI: 10.26574/maedica.2022.17.4.805 -
Cureus Oct 2022Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous skin disease that has six distinct types. Type 5 PRP is called atypical juvenile PRP. Here we report...
Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous skin disease that has six distinct types. Type 5 PRP is called atypical juvenile PRP. Here we report the case of a 17-year-old boy with insignificant past medical history presenting with a history of persistent slowly progressing very itchy skin lesions since the age of seven years. The lesions were photoaggravated. No similar cases in the family were observed and the parents were not consanguint. Skin examination revealed scaly erythematous patches, papules and plaques all over his body. There were also ichthyosiform-like scales covering the whole body. Hair, nails, and mucus membranes examinations were normal. A 4-mm punch skin biopsy was taken. The dermis revealed hyperkeratosis with checkerboard pattern of orthokeratosis and parakeratosis, the granular layer was preserved and acanthosis with thick and short rete ridges. The dermis showed mild perivascular lymphocytic infiltrates. On the basis of the above clinicopathological findings, the diagnosis of pityriasis rubra pilaris (atypical juvenile type) (type 5) was made. The patient was started on isotretinoin capsule 20 mg twice a day and placed under periodic follow-up.
PubMed: 36381776
DOI: 10.7759/cureus.30234 -
Journal of Veterinary Diagnostic... Sep 2022A 5-mm diameter mass developed on the nasal planum of a 4.5-y-old castrated male domestic shorthaired cat; the mass was raised ~2 mm above the surrounding skin....
A 5-mm diameter mass developed on the nasal planum of a 4.5-y-old castrated male domestic shorthaired cat; the mass was raised ~2 mm above the surrounding skin. Histology revealed focal thickening of the epidermis with marked orthokeratosis. Many of the epidermal cells within the mass had prominent papillomavirus-induced changes. A diagnosis of a viral papilloma was made, and a DNA sequence from a novel papillomavirus type was amplified from the lesion. Although the sequence was most similar to other feline papillomavirus types, the low level of similarity was suggestive of a novel papillomavirus genus. There has been no recurrence of the mass or development of additional lesions in the 6 mo since the mass was removed. This is the third cutaneous papilloma reported in a cat; a putative feline papillomavirus type has not been identified previously within these lesions, to our knowledge. Our findings expand the range of lesions associated with papillomaviruses in cats and increase the number of papillomavirus types that infect cats.
Topics: Animals; Cat Diseases; Cats; DNA, Viral; Male; Papilloma; Papillomaviridae; Papillomavirus Infections; Skin Neoplasms
PubMed: 35762121
DOI: 10.1177/10406387221107152 -
Pharmaceutics Apr 2022Non-steroidal anti-inflammatory drugs (NSAIDs) showed effects in some hyperproliferative dermatologic pathologies. The aim of the study is the assessment of...
Non-steroidal anti-inflammatory drugs (NSAIDs) showed effects in some hyperproliferative dermatologic pathologies. The aim of the study is the assessment of anti-psoriasis effect of diclofenac and celecoxib using a mice tail model. The topical application of substances on the proximal mice tails was performed for two weeks. The effects on the epidermal granular layer and mean epidermal thickness (excluding the stratum corneum) were evaluated using hematoxylin-eosin staining. Orthokeratosis degree and percentual drug activity were calculated. A positive control group treated with tretinoin and two negative controls (white soft paraffin and untreated mice) were used. Orthokeratosis degree significantly increased in all the NSAIDs groups (celecoxib 1%, 2% and diclofenac 1%, 2%) and in the tretinoin 0.05% group, versus negative controls. Celecoxib 1% and 2%, tretinoin 0.05% and white soft paraffin significantly increased mean epidermal thickness, versus untreated mice. The values obtained in the case of celecoxib 2% ointment regarding the orthokeratosis degree and percentual drug activity are providing premises for further investigations regarding this effect and the mechanisms of action involved. Celecoxib 2% had the greatest percentual drug activity and is a promising substance for the anti-psoriasis topical treatment. Along with the COX-2 inhibition, celecoxib might have an anti-psoriasis effect by other independent mechanisms.
PubMed: 35456720
DOI: 10.3390/pharmaceutics14040885 -
The American Journal of Dermatopathology Jul 2022Bulboma is an appellation proposed to designate a distinctive solitary keratosis exhibiting differentiation toward the bulb of the lower segment of the hair follicle....
Bulboma is an appellation proposed to designate a distinctive solitary keratosis exhibiting differentiation toward the bulb of the lower segment of the hair follicle. Bulboma is composed of a papillated proliferation of epithelial cells beneath which is a broad front of interconnected follicular papillae. Overlying these coalescing follicular papillae are follicular germinative cells ascending into matrical cells and supramatrical cells. Cells demonstrating differentiation toward Huxley's and Henle's layer of the inner root sheath at the level of the bulb are present including strikingly bright eosinophilic trichohyalin granules and the blue-grey corneocytes of the stem. There is overlying hypergranulosis and orthokeratosis. The clinical and histopathological findings in 4 cases of bulboma, a rare, benign solitary keratosis are presented.
Topics: Cell Differentiation; Hair Follicle; Humans; Keratosis, Actinic; Skin
PubMed: 35315371
DOI: 10.1097/DAD.0000000000002173 -
Journal of Cutaneous Pathology Jul 2022Papular-purpuric "gloves and socks" syndrome (PPGSS) is a unique, self-limited dermatosis characterized by edema, erythema, and pruritic petechiae and papules in a...
Papular-purpuric "gloves and socks" syndrome (PPGSS) is a unique, self-limited dermatosis characterized by edema, erythema, and pruritic petechiae and papules in a distinct "gloves and socks" distribution. This is often accompanied by systemic symptoms, including fever, lymphadenopathy, asthenia, myalgia, and arthralgias. PPGSS has also been described as a manifestation of an underlying immunological mechanism that can be triggered by viral or drug-related antigens. A 32-year-old male developed a painful eruption on the bilateral hands and feet after being diagnosed with influenza B. On examination, scattered papular purpura with occasional overlying scale was noted on the bilateral hands, fingers, feet, toes, volar wrists, and ankles. Histopathologic sections showed a mixed pattern of inflammation with interface and spongiotic changes. A parakeratotic scale with overlying basket-weave orthokeratosis was also seen. Within the epidermis, there was intraepidermal vesicles and Langerhans cell microabscess formation with scattered apoptotic keratinocytes. The underlying dermis showed a superficial perivascular lymphocytic infiltrate with mild edematous changes, and extravasation of red blood cells. Clinicopathologic correlation strongly supported a diagnosis of popular-purpuric gloves and socks syndrome. The influenza virus has never been reported in association with PPGSS; thus, this case outlines an important new variant that clinicians should be familiar with.
Topics: Adult; Edema; Foot Dermatoses; Hand Dermatoses; Humans; Influenza, Human; Male; Parvovirus B19, Human; Purpura
PubMed: 35148432
DOI: 10.1111/cup.14213 -
Indian Journal of Dermatology 2021Clinical diagnosis of vesiculobullous disorders (VBD) is not always straightforward. It is a challenge for a dermatologist to make the right diagnosis noninvasively in a...
BACKGROUND
Clinical diagnosis of vesiculobullous disorders (VBD) is not always straightforward. It is a challenge for a dermatologist to make the right diagnosis noninvasively in a short time.
OBJECTIVE
To evaluate dermoscopic patterns associated with vesiculobullous disorders.
METHODS
A total of 230 patients, irrespective of age and gender, with a history and clinical presentation suggestive of VBD (including primarily infectious, inflammatory, genetic, antibody-mediated, mechanical, environmental, metabolic, and drug-related) were recruited into the study. Patients with secondarily infected lesions were excluded. Dermoscopic examination along with Tzanck smear/skin biopsy smear test was performed on the most representative lesions. Data were compiled and statistically analyzed using SPSS version 21.0.
RESULTS
Lesions with erythematous (vascular) and yellowish (serum) translucent background with regular margins were seen in most of the VBD studied. Chickenpox (CP) and herpes zoster (HZ) lesions evolved with the progress of their clinical stages. Follicular and eccrine openings were commonly seen, but the pigmentation around them was specific to pemphigus vulgaris. A distorted pigment network was noted in bullous pemphigoid. White rosettes (keratin blockage) were characteristic of epidermolysis bullosa, Wickham striae (orthokeratosis) of lichen planus, and crumpled fabric appearance (flaccidity) of Hailey-Hailey disease. Globules/dots (microvesicles) of different colors were also seen in various VBD. Blue/black color usually corresponded to retained melanin.
CONCLUSION
Some dermoscopic patterns are observed consistently with certain diseases, and these can be used for their diagnosis, complementary to histopathological examination.
PubMed: 34759418
DOI: 10.4103/ijd.IJD_294_20