Eruptive Syringomas with Calcium Deposits in a Young Woman with Down’s Syndrome

Schepis, Carmelo; Torre, Valerio; Siragusa, Maddalena; Albiero, Francesca; Cicciarello, Rocco; Gagliardi, Maria; Cavallari, Vittorio

doi:10.1159/000051788

Cited by 32 publications

(25 citation statements)

References 10 publications

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“…24 Butterworth et al described syringomas in 18.5% of 200 institutionalized DS patients, with a prevalence of 26% among female and 13% among male patients. 24 The same authors have also noted MICC in association with syringomas in DS, thus supporting the hypothesis that syringeal structures may play an important role in the pathogenesis of MICC in DS. 24 The same authors have also noted MICC in association with syringomas in DS, thus supporting the hypothesis that syringeal structures may play an important role in the pathogenesis of MICC in DS.…”

Section: Syringomasmentioning

confidence: 99%

Dermatological manifestations of Down's syndrome

2006

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Down's syndrome (DS) is associated with rare dermatological disorders and increased frequency of some common dermatoses. Owing to advances in medical care and changes in attitude, the median age of death in this population has increased to 49 years, and the life expectancy of a 1-year-old person with DS today is more than 60 years and is likely to improve. With the increase in the number of individuals with DS in the population and an increased life span, dermatologists are more likely to encounter the wide spectrum of dermatological disorders that occurs in these patients. Furthermore, new reports of possible associations are frequent in the literature. The purpose of this article is to discuss the various dermatological conditions that affect DS individuals. A brief overview is given of the new information on genetics and the immunology of DS. We also discuss the molecular mechanisms of premature ageing, to which DS individuals are prone. We review the literature and discuss the known dermatological manifestations, concentrating on recent reports.

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Section: Syringomasmentioning

confidence: 99%

Dermatological manifestations of Down's syndrome

2006

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“…Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, because calcinosis cutis limits are not always well defined and surgical trauma itself may induce calcification, a recurrence of the lesions may occur 1–3,5–13 …”

Section: Discussionmentioning

confidence: 99%

Idiopathic Circumscripta Calcinosis Cutis of the Knee

Valdatta¹,

Buoro²,

Thioné³

et al. 2003

Dermatol Surg

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“…SCN are usually classified as an idiopathic entity, but in our patient, calcified lesions were associated with multiple skin‐coloured lesions of upper and lower eyelids which have preceded them by several years and histopathologically correspond to syringomas. Syringomas have been previously described in association with idiopathic calcinosis cutis especially in patients with Down's syndrome 9 but also in healthy patients 10 . But to our knowledge, syringomas have never been described in association with SCN.…”

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confidence: 85%

“…Schepis et al . stated that syringeal ductal structures play a role in calcium deposit since ultrastructural study revealed calcium deposits in many lumina and in the mitochondria in a trisomic patient with eruptive syringomas 9 . In SCN without associated syringomas, the cause of calcium deposit is not well established.…”

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confidence: 99%

Multiple subepidermal calcified nodules on the eyelids with eruptive syringomas: a possible ethiopathogenic relationship

Zeglaoui¹,

Khaled²,

Fazaa³

et al. 2009

Acad Dermatol Venereol

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generalized pustular eruption occurred 3 days after the delivery. A partial resolution was achieved with oral prednisolone (40 mg od) slowly tapered, but a satisfactory clinical response was only obtained with methotrexate (20 mg/week), leading to a complete resolution of the skin lesions at the 8th week of therapy. The newborn was a healthy male with no significant skin lesions.A new episode of generalized pustular eruption arose 1 year later, during the 20th week of an additional pregnancy. The patient was readmitted, and a very satisfactory clinical response was obtained with oral prednisolone (30 mg od), but a complete resolution was only observed 2 months after the delivery. The second son was also a male with no skin lesions until the age of 3 months, when he was observed with several well-demarcated erythematous plaques with superficial silver-white coloured crusts on the scalp and anterior aspect of lower limbs, clinically suggestive of plaquetype psoriasis.IH is a rare pustular eruption of pregnancy with unknown aetiology. It is frequently classified as a form of generalized pustular psoriasis, 1,2 and it normally occurs during the third trimester of pregnancy, significantly improving in the puerperium with possible recurrences in subsequent pregnancies. 3 It is associated with increased mother/foetal morbidity and mortality. 4 Systemic steroids are the mainstay of treatment. However, other drugs such as PUVA, retinoids and cyclosporin have been used either as single-agent or in combination therapies, particularly in poorly controlled disease. 5 The use of methotrexate in IH has been reported in three cases, two of them successfully. 3,5,6 Principally during the puerperium, a good outcome with methotrexate can be expected, as occurred in our patient.Several remarkable features have to be signalled in this case (i.e. the manifestation of the disease in a primigravida, the uncommon puerperal outbreak obligating to perform an aggressive systemic treatment, the recurrence during the second pregnancy at an early stage and the presence of psoriatic lesions in the second 3-monthold son). According to the clinical and histopathologic signs, the excellent response to methotrexate, which has proven efficacy in pustular psoriasis, and the coexistence of early onset psoriasis on the second son, we believe that IH can be really considered a variety of generalized pustular psoriasis. References1 Chang SE, Kim HH, Choi JH, Sung KJ, Moon KC, Koh JK. Impetigo herpetiformis followed by generalized pustular psoriasis: more evidence of the same disease entity. Int J Dermatol 2003; 42: 754-755. 2 Breier-Maly J, Ortel B, Breier F, Schmidt JB, Hönigsmann H. Generalized pustular psoriasis of pregnancy (impetigo herpetiformis). Dermatology 1999; 198: 61-64. 3 Katsambas A, Stavropoulos PG, Katsiboulas V et al. Impetigo herpetiformis during the puerperium. Dermatology 1999; 198: 400-402. 4 Brightman L, Stefanato CM, Bhawan J, Phillips TJ. Third-trimester impetigo herpetiformis treated with cyclosporine. J Am Acad Dermatol 2007; 56...

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Eruptive Syringomas with Calcium Deposits in a Young Woman with Down’s Syndrome

Cited by 32 publications

References 10 publications

Dermatological manifestations of Down's syndrome

Dermatological manifestations of Down's syndrome

Idiopathic Circumscripta Calcinosis Cutis of the Knee

Multiple subepidermal calcified nodules on the eyelids with eruptive syringomas: a possible ethiopathogenic relationship

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