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Dermatology Online Journal Oct 2023Pemphigus foliaceus is a superficial blistering disorder characterized by erosions and scaling in a seborrheic distribution. The condition typically occurs in healthy...
Pemphigus foliaceus is a superficial blistering disorder characterized by erosions and scaling in a seborrheic distribution. The condition typically occurs in healthy individuals but issues arise from delayed diagnosis. Many cases remain undiagnosed or misdiagnosed due to the lack of awareness of the condition. With use of common diagnostic tools, pemphigus foliaceus can be easily identified and monitored. Histological analysis exhibits "chicken wire" patterning along keratinocytes in the upper epidermis, whereas immunofluorescence study displays subcorneal acantholysis. Pemphigus foliaceus is confirmed via ELISA studies revealing the presence of autoantibodies against desmoglein 1. Once correctly diagnosed, typically the condition is responsive to corticosteroid therapy. However in recalcitrant cases such as in ours, adjunctive immunosuppressive therapy with dapsone or rituximab may be indicated.
Topics: Male; Humans; Pemphigus; Autoantibodies; Epidermis; Rituximab; Keratinocytes; Desmoglein 1
PubMed: 38478647
DOI: 10.5070/D329562411 -
Dermatology Online Journal Oct 2023Erythema elevatum diutinum (EED) is a rare cutaneous neutrophilic vasculitis with many associated diseases reported in the literature. We report a 65-year-old woman with...
Erythema elevatum diutinum (EED) is a rare cutaneous neutrophilic vasculitis with many associated diseases reported in the literature. We report a 65-year-old woman with painful and itchy lesions on her elbows, hands, knees, and foot for a year. Histopathologic examination confirmed the diagnosis of erythema elevatum diutinum and treatment with dapsone produced significant clinical improvement within few weeks. Erythema elevatum diutinum is a rare disease that should be considered in patients with violaceous nodular plaques located over the extensor regions of the limbs. Knowledge of this unusual pathology and its association helps to avoid misdiagnosis and late treatment.
Topics: Humans; Female; Aged; Vasculitis, Leukocytoclastic, Cutaneous; Skin; Dapsone; Arthritis, Rheumatoid; Erythema
PubMed: 38478644
DOI: 10.5070/D329562408 -
Oral and Maxillofacial Surgery Mar 2024Pyodermatitis-pyostomatitis vegetans (PPV) is a rare mucocutaneous disease characterized by multiple pustules and it is considered a marker for inflammatory bowel...
BACKGROUND
Pyodermatitis-pyostomatitis vegetans (PPV) is a rare mucocutaneous disease characterized by multiple pustules and it is considered a marker for inflammatory bowel disease (IBD). The oral manifestations of this condition are referred to as pyostomatitis vegetans (PSV).
PURPOSE
To investigate which features could help in establishing the diagnosis of PSV, with or without cutaneous lesions, based on information retrieved from all cases of PSV described in the literature. A case of PV from the authors was also included in the analysis.
METHODS
An electronic search was undertaken, last updated in August 2022. Inclusion criteria included publications reporting cases of PSV, with the diagnosis confirmed by the pathological examination of oral or skin lesions, and presence of IBD.
RESULTS/CONCLUSIONS
Sixty-two publications with 77 cases of PSV and an associated IBD were included. Features that are helpful in establishing the diagnosis of PSV are snail track appearance of oral lesions, an associated IBD (which is not always symptomatic), evidence of intraepithelial clefting on microscopic examination of oral lesions, and peripheral blood eosinophilia. A gold standard for the management of PSV does not exist and high-level evidence is limited. There is no established therapeutic protocol for PSV and management primarily consists of topical and/or systemic corticosteroids, antirheumatic drugs (sulfasalazine, mesalazine), monoclonal antibody (infliximab, adalimumab) immunosuppressives (azathioprine, methotrexate), antibiotics (dapsone), or a combination of these. The risk of recurrence of oral lesions is considerable when the medication dose is decreased or fully interrupted.
PubMed: 38467949
DOI: 10.1007/s10006-024-01234-1 -
BMJ Open Ophthalmology Mar 2024Subretinal drusenoid deposits (SDDs) in age-related macular degeneration (AMD) are associated with systemic vascular diseases that compromise ocular perfusion. We...
PURPOSE
Subretinal drusenoid deposits (SDDs) in age-related macular degeneration (AMD) are associated with systemic vascular diseases that compromise ocular perfusion. We demonstrate that SDDs are associated with decreased ellipsoid zone (EZ) thickness, further evidence of hypoxic damage.
METHODS
Post hoc analysis of a cross-sectional study. 165 AMD subjects (aged 51-100; 61% women). Spectral-domain optical coherence tomography was obtained in both eyes. Masked readers assigned subjects to three groups: drusen only, SDD+drusen (SDD+D) and SDD only. EZ thickness was measured subfoveally and 2000 µm nasally, temporally, superiorly and inferiorly from the fovea. Univariate testing was performed using two-tailed t-tests with Bonferroni correction.
RESULTS
The mean EZ thickness differences between the SDD+D and drusen-only groups were (in μm) 1.10, 0.67, 1.21, 1.10 and 0.50 at the foveal, nasal, temporal, superior and inferior locations, respectively (p=0.08 inferiorly, otherwise p≤0.01); between the SDD-only and drusen-only groups, the differences were 3.48, 2.48, 2.42, 2.08 and 1.42 (p≤0.0002). Differences in EZ thicknesses across all subjects and between groups were not significantly different based on gender, race or age.
CONCLUSION
Subjects with SDDs (±drusen) had thinner EZs than those with drusen only, and the inferior EZ was least affected. EZs were thinnest in SDD-only subjects. This thinning gradation is consistent with progressive destruction of highly oxygen-sensitive mitochondria in the EZ from hypoxia. These findings support the reduced ophthalmic perfusion hypothesis for the formation of SDDs secondary to high-risk systemic vasculopathy.
Topics: Humans; Female; Male; Retinal Drusen; Cross-Sectional Studies; Macular Degeneration; Retina; Tomography, Optical Coherence; Dapsone
PubMed: 38460964
DOI: 10.1136/bmjophth-2023-001622 -
American Journal of Clinical Dermatology May 2024Acne is one of the most common dermatological conditions to affect women of childbearing age, so it is important to consider the safety of long-term acne treatments on... (Review)
Review
Acne is one of the most common dermatological conditions to affect women of childbearing age, so it is important to consider the safety of long-term acne treatments on women who could become pregnant. In this review article, we clarify what management options are available to treat acne during pregnancy. Topical treatments, typically first-line for acne, such as azelaic acid, clindamycin, erythromycin, metronidazole, benzoyl peroxide, salicylic acid, dapsone, and retinoids, were reviewed. Systemic treatments, such as zinc supplements, cephalexin, cefadroxil, amoxicillin, azithromycin, erythromycin, and corticosteroids, typically second-line for acne, were also reviewed. Alternative treatments such as light therapy and cosmetic procedures were also evaluated. Due to recommendation of sunscreen utilization during acne treatments, sunscreen usage during pregnancy was also assessed. Management of acne during unplanned pregnancy was discussed in further detail regarding safety and adverse effects. Through summarized tables and examples of studies demonstrating safety and efficacy of treatments, the following is a resource for providers and patients to utilize for management of acne during pregnancy.
Topics: Humans; Acne Vulgaris; Pregnancy; Female; Dermatologic Agents; Pregnancy Complications; Anti-Bacterial Agents; Sunscreening Agents; Pregnancy, Unplanned; Phototherapy; Administration, Cutaneous
PubMed: 38453786
DOI: 10.1007/s40257-024-00851-6 -
Narra J Aug 2023Necrotic erythema nodosum leprosum (ENL) is an uncommon manifestation of type 2 lepra reaction, encountered in lepromatous and borderline lepromatous cases of leprosy....
Necrotic erythema nodosum leprosum (ENL) is an uncommon manifestation of type 2 lepra reaction, encountered in lepromatous and borderline lepromatous cases of leprosy. Necrotic ENL is associated with the involvement of multiple organs, therefore delayed diagnosis and treatment will lead to complications and poor prognosis. The aim of this case report was to report a challenging case of necrotic ENL misdiagnosed with multiple cellulitis since there were no signs of prior leprosy nor had any antimycobacterial treatment. A 45-year-old man was presented to the surgery department of Dr. Zainoel Abidin Hospital, Banda Aceh, Indonesia, with complaints of fever, joint pain, and painful tender skin lesions with ulceration over the trunk, extremities, and ears for one month. The patient was diagnosed clinically with multiple cellulitis and underwent a debridement procedure. Clinical improvement was absent, the patient was then consulted to the dermatology department. Physical examination showed normal vital signs, madarosis, inguinal lymphadenopathy, thickening of nerves, and sensation of numbness in both hands and feet. Laboratory examinations on admission showed leucocytosis, anemia, thrombocytopenia, hypoalbuminemia, hypocalcemia, and elevated creatinine and ureum level. A slit skin smears examination yielded positive acid-fast bacilli (AFB) with a bacteriological index (BI) value of 3+ and morphological index (MI) of 72%. The patient was diagnosed with lepromatous leprosy with necrotic ENL reaction. Intravenous methylprednisolone and cefoperazone-sulbactam were given. Multidrug therapy mulitbacillary (MDT-MB) without dapsone, and ofloxacin 400 mg was initiated. On day 17, the patient had septic shock. The patient became unconscious and experienced death. This case highlights that medical professionals should be aware of the various manifestations of necrotic ENL to correctly diagnose and provide treatment as soon as possible to prevent mortality, especially in leprosy-endemic country, Indonesia.
PubMed: 38450265
DOI: 10.52225/narra.v3i2.216 -
Retinal Cases & Brief Reports Mar 2024Hansen's disease is endemic in over 140 countries worldwide and a potentially blinding condition. We describe a case of retinal vasculitis in a patient with Hansen's...
PURPOSE
Hansen's disease is endemic in over 140 countries worldwide and a potentially blinding condition. We describe a case of retinal vasculitis in a patient with Hansen's disease with concomitant positive antiphospholipid antibody serology, a potentially under-reported complication in this setting.
METHODS
A 37-year-old Brazilian man systemically stable on triple therapy (clofazimine, rifampin, dapsone) for Hansen's disease presented for a comprehensive ophthalmic evaluation.
RESULTS
Dilated exam revealed diffuse peripheral intraretinal hemorrhages in his right eye. Fluorescein angiography showed peripheral non-perfusion, abnormal shunt vessels and leakage from the retinal veins in the right eye and peripheral non-perfusion and vascular leakage in the fellow eye, consistent with vasculitis. Laboratory workup was notable for positive antiphospholipid antibodies (lupus anticoagulant, anticardiolipin IgM, anti-beta-2 glycoprotein 1 IgM) and normocytic anemia.
CONCLUSION
As antiphospholipid antibodies are present in a large proportion of patients with Hansen's disease, it is possible that retinal vasculitis may be more common than previously thought. The extent to which retinal vasculitis occurs in Hansen's disease remains uncertain and possibly underestimated due to the frequency of anterior segment scarring, which impedes retinal evaluation. Heightened surveillance for potential retinal vascular complications is warranted.
PubMed: 38447130
DOI: 10.1097/ICB.0000000000001560 -
Indian Journal of Otolaryngology and... Feb 2024Rhinosporidiosis is a granulomatous disease commonly affecting the mucous membrane. It is caused by Rhinosporidium seeberi, an aquatic parasite & seen affecting the...
Rhinosporidiosis is a granulomatous disease commonly affecting the mucous membrane. It is caused by Rhinosporidium seeberi, an aquatic parasite & seen affecting the nose, paranasal sinuses most commonly. A retrospective study was conducted at a tertiary care hospital situated in Indian peninsula and five patients who were diagnosed and treated for rhinosporidiosis were analysed. Surgical excision by coblator along with medical management using Dapsone 100 mg once daily for 6 months given promising results in view of reducing recurrence. Combined approach of management including surgical excision using coblator and medical therapy with dapsone is effective in managing the rhinosporidiosis with no recurrence.
PubMed: 38440563
DOI: 10.1007/s12070-023-04155-9 -
Acta Dermatovenerologica Croatica : ADC Dec 2023Neutrophilic dermatosis of the hands (NDDH) is a localized variant of Sweet's syndrome which has been recently introduced. Strutton et al.in 1996 and then in Galaria et...
Neutrophilic dermatosis of the hands (NDDH) is a localized variant of Sweet's syndrome which has been recently introduced. Strutton et al.in 1996 and then in Galaria et al. in 2000 reported cases with violaceous papulonodules on the dorsal surfaces of the hands with histopathological findings of a neutrophilic dermatosis in association with leukocytoclasia, but clinically and histologically without true vasculitis findings. Eventually, they proposed the term NDDH for these lesions (1,2). A 46-year-old man was referred to our outpatient dermatology clinic with a painful ulcerative lesion on the dorsal side of the left hand that had been present for one year. Initially, the lesion had appeared as a small purulent papule, which gradually extended to a large ulcer. The patient underwent frequent referrals to several physicians and had taken a variety of topical and systemic antibiotics, meglumine antimoniate (Glucantime), and amphotericin with the clinical diagnosis of cutaneous bacterial or fungal infections, or leishmaniasis. All of these therapeutic regimes were ineffective in eradicating the lesion. Given the history, he denied any trauma to the site of lesion; he also did not report any similar lesions in his family. The physical examination revealed an extensive tender ulcer of 4×7 cm2 in size, with a shallow violaceous border superimposed on an edematous region on the dorsal side of the left hand. Atrophic scars resulting from old similar lesions were visible on the dorsal aspects of the 3rd and 4th proximal and middle metacarpal joints (Figure 1). The examination of the other parts of the body was unremarkable. Laboratory tests showed an impaired white blood cell count and their differentiation, including leukocytosis (white blood cell count of 16.12/mm3) with neutrophilia (neutrophil percent at 65.9%). Additionally, altered liver function tests were remarkable for high serum levels of AST (SGOT) (105 IU/L) and ALT(SGPT) (355 IU/L), while the total bilirubin and alkaline phosphatase were within normal limits. Hemoglobin levels (13.90 g/dL) and platelet count (272/mm3) were within normal range. The other laboratory tests, including serological tests for fasting blood sugar, hemoglobin A1c, creatinine, BUN, and an immunoassay for ruling out vasculitis lesions (anti-MPO (P-ANCA) and anti-PR3 (C-ANCA)) revealed no remarkable results. An erythrocyte sedimentation rate of 16 mm/h was reported. A biopsy was performed. Histologic features demonstrated a dense, diffuse dermal infiltrate comprised almost entirely of neutrophils. The epidermis was slightly acanthotic and showed small foci of spongiosis, but the inflammatory infiltrate remained largely in the dermis. Sheets of neutrophils were present, admixed with karyorrhectic debris. The infiltrate did not appear to be peri-vascular, and most vessels that could be observed clearly appeared to be undamaged. However, some vessels appeared to show some neutrophils infiltrating vessel walls (Figures 2a, b). Based upon histopathologic examination, the diagnosis of neutrophilic dermatosis of the hands (NDDH) was suggested. The work-up findings for ruling out neoplastic diseases were unremarkable. Clinically, patients with NDDH show various morphologic patterns of the lesions on the dorsal aspect of the hands, including violaceous edematous plaques or ulcers with undermined borders, hemorrhagic bullae, necrotic pyoderma-like lesions with pseudovesiculation, and atypical pyoderma gangrenosum-like lesions (1). This disease is more common in women (70%) than in men (3). NDDH has been reported in association with malignancies (such as leukemia and lymphoma), myelodysplasia, inflammatory bowel diseases, seropositive arthritis, sarcoidosis, HCV infection, and medications (such as lenalidomide, thalidomide, vaccinations, fertilizer, etc.) (1). Among them, neoplastic diseases are the most common association, which has been reported in 27% of the cases. It may thus represent a paraneoplastic phenomenon (3). Histopathological study is mandatory for achieving a definite diagnosis of NDDH. Its pathological findings include subepidermal edema, a dense and diffuse dermal infiltration of neutrophils along with leukocytoclastic debris, and extravasated erythrocytes, which are not associated with true vasculitis (1,3). However, the presence or absence of some vasculitic features as a histopathological finding depends on the time of biopsy with regard to the evolutionary phases of the lesion (3). In our case, the diffuse nature of the infiltrate was somewhat indicative against the diagnosis of leukocytoclastic vasculitis. Additionally, the possibility of infection was excluded empirically (due to the ineffectiveness of previous therapies without doing cultures or PCR), and indirectly through biopsy. Cohen (4) and Cohen and Kurzrok (5) explained the presence of vasculitis in Sweet's syndrome and NDDH as an epiphenomenon in which the damaged vessel is as an "innocent bystander" in the background of an inflammatory dermatosis. Eventually, they concluded that the presence or absence of vasculitis has a secondary importance in the diagnosis of NDDH. The following entities should be considered in the differential diagnoses of NDDH: cutaneous infections, vesiculobullous pyoderma gangrenosum (atypical), bullous erythema multiforme, pustular drug reactions, rheumatoid neutrophilic dermatosis, bowel-associated dermatosis-arthritis syndrome, and erythema elevatum diutinum (1-3). In our case, based on the pathological examination, the differential diagnosis included neutrophilic dermatosis such as Sweet's syndrome or neutrophilic dermatosis of the dorsal hands. It is essential to exclude an infectious etiology that might include a bacterial infection, or less likely a fungal or atypical mycobacterial infection, given the lack of any granulomatous component. However, some atypical mycobacterial infections can demonstrate a brisk neutrophilic infiltrate and relatively sparse granulomatous responses (6). For the same reason (lack of significant histiocytes), we thought that palisaded neutrophilic and granulomatous dermatosis associated with connective tissue disease was less likely. The relationship between this disease entity and a superficial variant of pyoderma gangrenosum remains unclear. The treatment of NDDH includes systemic corticosteroids, dapsone, methotrexate, potassium iodide, colchicine, and minocycline (2). NDDH is often misdiagnosed as an infectious condition, which can result in inappropriate antibiotic therapy, surgical debridement, and even amputation (7). Therefore, early diagnosis and initiation of appropriate treatment should be mainstay of its treatment.
Topics: Male; Humans; Female; Middle Aged; Pyoderma Gangrenosum; Sweet Syndrome; Ulcer; Dermatitis; Vasculitis; Anti-Bacterial Agents; Arthritis; Hemoglobins; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 38439725
DOI: No ID Found -
Dermatologic Clinics Apr 2024Pyoderma gangrenosum is a rare neutrophilic dermatosis that results in painful cutaneous ulcers and is frequently associated with underlying hematologic disorders,... (Review)
Review
Pyoderma gangrenosum is a rare neutrophilic dermatosis that results in painful cutaneous ulcers and is frequently associated with underlying hematologic disorders, inflammatory bowel disease, or other autoimmune disorders. Pathogenesis involves an imbalance between proinflammatory and anti-inflammatory mediators, leading to tissue damage from neutrophils. First-line treatment options with the greatest evidence include systemic corticosteroids, cyclosporine, and tumor necrosis factor alpha inhibitors. Other steroid-sparing therapies such as dapsone, mycophenolate mofetil, intravenous immunoglobulin, and targeted biologic or small molecule inhibitors also have evidence supporting their use. Wound care and management of underlying associated disorders are critical parts of the treatment regimen.
Topics: Humans; Pyoderma Gangrenosum; Immunosuppressive Agents; Cyclosporine; Adrenal Cortex Hormones; Skin Ulcer
PubMed: 38423680
DOI: 10.1016/j.det.2023.12.002