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Cureus Dec 2021The term "Mongolian Spot" rather than the preferred descriptive name congenital dermal melanocytosis (CDM) continues to be used despite compelling objections to the...
The term "Mongolian Spot" rather than the preferred descriptive name congenital dermal melanocytosis (CDM) continues to be used despite compelling objections to the contrary. Terms that stigmatize a culture, region, people, country, communities, and ethnic group should be replaced by their more descriptive counterparts. Herein, we clarify terminology, discuss the historical significance, and provide a recommendation about naming this disease.
PubMed: 35036226
DOI: 10.7759/cureus.20396 -
Revue Medicale de Liege Jul 2023Zoon's balanitis is a rare genital dermatosis of unknown etiology, usually presenting as a red-orange macule or plaque with a «cayenne pepper» appearance on the glans...
Zoon's balanitis is a rare genital dermatosis of unknown etiology, usually presenting as a red-orange macule or plaque with a «cayenne pepper» appearance on the glans and/or foreskin. Unfortunately, atypical presentations are not uncommon, including vegetating or ulcerative lesions. Usually, it affects middle-age to older uncircumcised men. Although it is a benign pathology, Zoon's balanitis may be superimposed on another inflammatory or neoplastic dermatosis. As topical treatments are generally non satisfactory and relapses are usual on treatment with-drawal, circumcision remains an interesting option with usually a rapid and complete remission of the symptoms.
Topics: Male; Middle Aged; Humans; Balanitis; Circumcision, Male; Skin Diseases
PubMed: 37560959
DOI: No ID Found -
Endocrinology, Diabetes & Metabolism... May 2023We report the case of a 69-year-old female with systemic mastocytosis, diagnosed based on widespread pigmented papules and macules, elevated serum tryptase levels and...
SUMMARY
We report the case of a 69-year-old female with systemic mastocytosis, diagnosed based on widespread pigmented papules and macules, elevated serum tryptase levels and confirmatory skin and bone marrow biopsy, on a background of osteoporosis. A CT demonstrated multiple sclerotic lesions within lumbar vertebral bodies, sacrum and ileum, with surrounding osteolysis but no obvious compression fractures. She was treated with the RANK-L inhibitor denosumab, resulting in significant bone mineral density gain over the following 5 years. However, her serum tryptase levels gradually increased during this period despite treatment with the multikinase inhibitor, midostaurin. It is thus conceivable that her rapid increase in bone mineral density may be partly contributed by a predominance of pro-osteoblastic mediators released by abnormal mast cells, suggestive of more advanced disease. This case highlights the complexities of systemic mastocytosis-related bone disease and the interplay of numerous mediators contributing to a phenotype of both increased bone resorption and formation.
LEARNING POINTS
Systemic mastocytosis is a neoplastic disease of mast cells characterized by abnormal proliferation and accumulation in the skin and other organs. It is most frequently associated with the somatic gain-of-function KIT D816V mutation. Systemic mastocytosis should be suspected in patients presenting with not only cutaneous symptoms suggestive of mast cell degranulation such as anaphylaxis, flushing or urticaria but also unexplained osteoporosis and gastrointestinal and constitutional symptoms. The prevalence of osteoporosis in systemic mastocytosis is high. Mast cell activation leads to the secretion of numerous chemical mediators which either promote or inhibit osteoclastic and/or osteoblastic activity, with the balance usually in favour of increased bone resorption. However, in advanced diseases with high mast cell burden, mast-cell-derived cytokines and mediators may promote osteoblastic activity, leading to osteosclerosis and apparent increases in bone mineral density. Treatment of osteoporosis in systemic mastocytosis involves antiresorptive therapy with bisphosphonates and more recently, denosumab. There are limited data on the role of osteoanabolic agents.
PubMed: 37166908
DOI: 10.1530/EDM-22-0408 -
JAAD International Mar 2021Because of the increasing emergence of skin manifestations of COVID-19 worldwide, we investigated the published reports of these lesions. (Review)
Review
OBJECTIVE
Because of the increasing emergence of skin manifestations of COVID-19 worldwide, we investigated the published reports of these lesions.
METHODS
We conducted a literature search for original and review articles published from November 11, 2019 to September 30, 2020.
RESULTS
We identified 5 skin lesions common in patients with COVID-19: pseudo-chilblains, rashes containing macules and papules, and urticarial, vesicular, and vaso-occlusive lesions. These lesions manifested at various times in relation to the COVID-19 symptoms, which may indicate whether the lesions are virus-induced or are delayed immunological responses to the infection. Skin lesions were more prevalent among Europeans and United States residents than among Asians, as was pseudo-chilblain, and the morphology of the skin lesions varied among continents. Pseudo-chilblains were the most common COVID-19 skin manifestation in Europe and the United States, but there was only 1 reported case from Asian populations. Additionally, patients with vaso-occlusive lesions were more likely than those with pseudo-chilblains to be admitted to the intensive care unit and to die.
CONCLUSION
Different cutaneous manifestations in patients with COVID-19 could reflect a wide spectrum of viral interactions with the skin, though reporting bias may play a role as well.
PubMed: 33479703
DOI: 10.1016/j.jdin.2020.12.003 -
Actas Dermo-sifiliograficas Jan 2021
Topics: Humans; Melanosis; Nevus, Pigmented; Scalp; Skin Abnormalities; Skin Neoplasms
PubMed: 33049275
DOI: 10.1016/j.ad.2019.04.021 -
Dermatopathology (Basel, Switzerland) Jan 2022About 20 years after its first description, Annular Lichenoid Dermatitis of Youth (ALDY) is recognized as a distinctive lichenoid dermatosis with specific clinical and...
About 20 years after its first description, Annular Lichenoid Dermatitis of Youth (ALDY) is recognized as a distinctive lichenoid dermatosis with specific clinical and histological features. The disease occurs mostly in young persons all over the world, runs a chronic course, and has an obscure etiopathogenesis. Clinically, lesions consist of persistent, asymptomatic erythematous macules and round-oval annular patches with a red-violaceous non-scaling border and central hypopigmentation, mostly localized on the groin and flanks. Histology shows a peculiar lichenoid dermatitis characterized by irregular epidermal hyperplasia with an alternation of thinned and quadrangular rete ridges and a dense band-like lichenoid infiltrate of lymphocytes in the papillary dermis. Typically, there is infiltration of lymphocytes into the lower epidermal layers with massive necrosis/apoptosis of keratinocytes, which is limited to the tips of rete ridges. Dermal lymphocytes are usually CD3, CD4, while most of the intraepidermal T cells are CD8. Analysis of TCR-γ-chain gene rearrangement displayed polyclonality in all cases examined. Differential diagnosis mainly includes morphea, mycosis fungoides, annular erythemas and inflammatory lesions of vitiligo. Topical corticosteroids and topical tacrolimus represent the most effective drugs for ALDY treatment.
PubMed: 35076479
DOI: 10.3390/dermatopathology9010004 -
Indian Journal of Dermatology 2020Chemical leucoderma, an under-diagnosed common condition often mimicking idiopathic vitiligo, represents an acquired depigmentation induced by repeated exposure to... (Review)
Review
Chemical leucoderma, an under-diagnosed common condition often mimicking idiopathic vitiligo, represents an acquired depigmentation induced by repeated exposure to specific chemical compounds in subjects with genetic susceptibility to vitiligo. This has been increasing rapidly in incidence in recent decades in developing countries like India. The term 'chemical vitiligo' was first coined by us to indicate the possible relationship between chemical leucoderma and vitiligo, which has been supported recently by other authors to designate the term 'chemical-induced vitiligo'. The largest case series showed that household chemical exposure was the major etiological factor. Causative chemicals are mostly phenolic and catecholic derivatives. Vitiligo pathogenesis is induced by genetic and environmental factors like many other autoimmune diseases. Innate immunity acts as a bridge between cellular stress and adaptive immunity. Multiple patches are commonly seen; children below 12 years are also affected in good numbers. The most common presence of confetti macules indicates these as characteristic, although not pathognomonic, of chemical leucoderma. Chemical leucoderma has been broadened into 'chemical leucoderma syndrome' with proper staging. The clinical criteria for diagnosis of chemical leucoderma have been specifically outlined. Same pathomechanism of chemical leucoderma might elucidate trigger factors and reasons for progression and chronicity in idiopathic vitiligo. Depigmentation in chemical vitiligo spreads to distant sites, in the same way as generalized idiopathic vitiligo. The study showed that chemical triggering factors played a very significant role in the induction and progression of vitiligo. Thus it should be rational to consider chemical vitiligo not as a separate entity but as a major subset of vitiligo spectrum.
PubMed: 33487697
DOI: 10.4103/ijd.IJD_291_20 -
Dermatopathology (Basel, Switzerland) Feb 2024Galli-Galli disease (GGD) is a rare genodermatosis that exhibits autosomal dominant inheritance with variable penetrance. GGD typically manifests with erythematous... (Review)
Review
Galli-Galli disease (GGD) is a rare genodermatosis that exhibits autosomal dominant inheritance with variable penetrance. GGD typically manifests with erythematous macules, papules, and reticulate hyperpigmentation in flexural areas. A distinct atypical variant exists, which features brown macules predominantly on the trunk, lower limbs, and extremities, with a notable absence of the hallmark reticulated hyperpigmentation in flexural areas. This review includes a detailed literature search and examines cases since GGD's first description in 1982. It aims to synthesize the current knowledge on GGD, covering its etiology, clinical presentation, histopathology, diagnosis, and treatment. A significant aspect of this review is the exploration of the genetic, histopathological, and clinical parallels between GGD and Dowling-Degos disease (DDD), which is another rare autosomal dominant genodermatosis, particularly focusing on their shared mutations in the and genes. This supports the hypothesis that GGD and DDD may be different phenotypic expressions of the same pathological condition, although they have traditionally been recognized as separate entities, with suprabasal acantholysis being a distinctive feature of GGD. Lastly, this review discusses the existing treatment approaches, underscoring the absence of established guidelines and the limited effectiveness of various treatments.
PubMed: 38390850
DOI: 10.3390/dermatopathology11010008 -
International Journal of Women's... Oct 2022Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in... (Review)
Review
Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III-V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants.
PubMed: 35923586
DOI: 10.1097/JW9.0000000000000034