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JAAD Case Reports Feb 2024
PubMed: 38362123
DOI: 10.1016/j.jdcr.2023.11.033 -
Journal of Medical Case Reports Feb 2024Scleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made...
BACKGROUND
Scleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made on the basis of clinical findings and skin biopsy.
CASE PRESENTATION
Here, we describe a 14-year-old Iranian girl presenting with non-pitting edema and woody thickening of the skin that progressed within a month. She was evaluated for possible underlying malignancy or connective tissue disorders, which were excluded by multiple laboratory workups. She underwent a skin biopsy which confirmed the diagnosis of scleredema, and she was successfully treated with intravenous immunoglobulin and mycophenolate mofetil.
CONCLUSION
While scleredema adultorum of Buschke is a rare disease with no definite treatment, our effort through this report was to highlight the possible benefits of treatment by intravenous immunoglobulin and mycophenolate mofetil.
Topics: Female; Humans; Adolescent; Scleredema Adultorum; Immunoglobulins, Intravenous; Mycophenolic Acid; Iran; Rare Diseases; Edema
PubMed: 38350929
DOI: 10.1186/s13256-024-04427-0 -
Skin Health and Disease Feb 2024Scleredema diabeticorum (SD) is a common cause of scleredema with limited effective treatment options available. Patients with SD may experience significant discomfort...
Scleredema diabeticorum (SD) is a common cause of scleredema with limited effective treatment options available. Patients with SD may experience significant discomfort due to symptoms of itching, burning, or pain. SD typically develops due to poor glycaemic control as demonstrated in this case.
PubMed: 38312250
DOI: 10.1002/ski2.311 -
Frontiers in Medicine 2023Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after...
The realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study.
BACKGROUND
Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after 30 years, its use has remained limited to few dermatological centers.
OBJECTIVE
To analyze the changes over the years and the current position of UVA1 phototherapy through a Real-World Evidence (RWE) study at a single tertiary referral center.
METHODS
We reviewed the medical files of 740 patients treated between 1998 and 2022. Treatment results were collected, efficacy was assessed by a grading scale and acute adverse effects were registered.
RESULTS
We treated patients with 26 different diseases. We registered marked improvement (MI) or complete remission (CR) in 42.8% of patients with morphea, 50% with Urticaria Pigmentosa, 40.7% with Granuloma annulare and 85.7% with skin sarcoidosis. Good results were obtained also in the treatment of chronic Graft Versus Host Disease (GVHD), Eosinophilic Fasciitis, Sclero-atrophic Lichen, skin manifestations of systemic lupus erythematosus and psoriasis of HIV+ patients. Systemic Sclerosis, Romberg's Syndrome, Bushke's Scleredema, Nephrogenic Fibrosing Dermopathy, REM Syndrome, Follicular Mucinosis, Pretibial Myxedema, Scleromyxedema, pemphigus foliaceus, chronic cutaneous lupus erythematosus, erythroderma of Netherton Syndrome and Necrobiosis Lipoidica were no or poorly responsive. In clinical indications where UVA1 was used as a second line phototherapy after narrow-band (NB)-UVB, we saw good MI or CR rates in Mycosis Fungoides (57% of patients), Atopic Dermatitis (33.9%), Pitiryasis Lichenoides chronica (50%), Pityriasis Lichenoides et varioliformis acute (75%) and Lymphomatod Papulosis (62.5%). Short-term adverse events were uncommon and mild.
CONCLUSION
Over the past decade, the annual number of treated patients has progressively declined for several reasons. Firstly, UVA1 phototherapy has taken a backseat to the cheaper and more practical NB-UVB phototherapy, which has proven effective for common indications. Secondly, the emergence of new, safe, and effective drugs for conditions such as atopic dermatitis, GVHD, and connective tissue disorders. Finally, our research has shown that UVA1 therapy is often ineffective or minimally effective for some rare diseases, contrary to previous case reports and small case series. Nonetheless, UVA1 continues to be a valuable treatment option for patients with specific skin disorders.
PubMed: 38076241
DOI: 10.3389/fmed.2023.1295145 -
RMD Open Nov 2023
Topics: Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Scleredema Adultorum; Scleromyxedema; Aged; Male
PubMed: 38016711
DOI: 10.1136/rmdopen-2023-003638 -
Cureus Sep 2023Type 2 scleredema on the background of monoclonal gammopathy of undetermined significance (MGUS) is a rare and progressive connective tissue disorder with very few cases...
Type 2 scleredema on the background of monoclonal gammopathy of undetermined significance (MGUS) is a rare and progressive connective tissue disorder with very few cases reported to date. It is characterized by chronic and diffuse induration of the skin that begins in the upper back and neck and progresses proximally to distally, involving the shoulders, trunk, and arms; the hands are usually spared. Here, we present an unusual case of long-standing scleredema that progressed to involve the hands and fingers. This case was further complicated by new-onset Raynaud's phenomenon, splenomegaly, lymphadenopathy, the development of a plasmacytoma, and eventual progression to multiple myeloma. We highlight the differential diagnoses for his complex presentation, the workup that was completed, and current treatment options.
PubMed: 37701163
DOI: 10.7759/cureus.44968 -
Frontiers in Immunology 2023Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected... (Review)
Review
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Topics: Scleroderma, Systemic; Humans; Diagnosis, Differential
PubMed: 37600771
DOI: 10.3389/fimmu.2023.1180221 -
Cureus Apr 2023Scleredema adultorum of Buschke is a rare condition that presents as a scleroderma mimic and portends a diagnostic challenge to the clinician. It may be associated with...
Scleredema adultorum of Buschke is a rare condition that presents as a scleroderma mimic and portends a diagnostic challenge to the clinician. It may be associated with monoclonal gammopathy, upper respiratory tract infection, or type II diabetes mellitus. In addition, it is associated with dermal collagen and aminoglycan deposits that cause the skin to thicken and stiffen. Typically, thickening and tightening begin in the neck and progress to the upper body, including the face, scalp, shoulders, and trunk, but sparing the palms and soles. Patients with minor skin involvement may not suffer any symptoms, whereas those with significant skin disease may develop stiffness and functional impairment. There are rare reports linking scleredema adultorum of Buschke with several infections such as human immunodeficiency virus infection, acquired immunodeficiency syndrome-related lipodystrophy syndrome, and streptococcal infection of the upper respiratory tract. Here, we present a case of scleredema adultorum of Buschke associated with hepatitis B infection.
PubMed: 37255897
DOI: 10.7759/cureus.38300 -
Reumatismo May 2023cleredema of Buschke is a rare pathological disorder of connective tissue, which is characterized by a woody, diffuse induration of the skin, most often in the upper...
cleredema of Buschke is a rare pathological disorder of connective tissue, which is characterized by a woody, diffuse induration of the skin, most often in the upper extremities. We report an extremely rare complication of post-streptococcal infection in a six-year-old male complaining of gradually progressing, painless skin thickening and tightness which was preceded by a one-month history of fever, cough, and tonsillitis. By reporting this case, we hope to contribute to the creation of a database for future research aimed at better understanding the incidence, pathophysiology, and management of this extremely rare complication.
Topics: Male; Humans; Child; Scleredema Adultorum; Skin
PubMed: 37154255
DOI: 10.4081/reumatismo.2023.1551