Cutis laxa-like pseudoxanthoma elasticum with osteoma cutis

Leblebıcı, Cem; Falay, Tuğba; Zırtıloğlu, Sibel; Demirkesen, Cüyan

doi:10.4103/ijdvl.ijdvl_690_16

Cited by 4 publications

(9 citation statements)

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“…Pseudoxanthoma elasticum and its association with reticulate papules and plaques as seen in our case along with multiple open comedones and neurofibroma is an extremely rare association. A study of Pseudoxanthoma elasticum cases in the Indian subcontinent showed that skin lesions and eye involvement are common but systemic involvement is relatively rare [5]. Eye involvement and systemic symptoms were not seen in our case.…”

Section: Discussioncontrasting

confidence: 47%

Pseudoxanthoma Elasticum and its Rare Co-Existence with Comedones and Neurofibroma

Sg¹

2018

CDOAJ

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A 68 year old male patient, presented with multiple asymptomatic elevated skin lesions over the face, V area of neck since last 40 years. Physical examination revealed numerous open comedones all over the face, forehead and V area of neck. Numerous firm, skin colored to yellowish, non-follicular papules coalescing into plaques were seen around the neck, suprascapular region, both axillae, cubital and popliteal region with redundant lax skin folds. A single pedunculated nontender firm swelling of 1X1 cm size is seen over the left side of the chest. Histopathological examination of punch biopsy from lesional skin showed irregularly clumped faintly basophilic elastic fibres admixed with mucoid material in the mid and lower dermis. Excisional biopsy from the lesion over the chest showed skin with underlying dermal circumscribed, non encapsulated tumour composed of spindle cells arranged in fascicles. Patient improved with topical and systemic retinoids. We are reporting this case as it's a rare association with pseudoxanthoma elasticum.

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Section: Discussioncontrasting

confidence: 47%

Pseudoxanthoma Elasticum and its Rare Co-Existence with Comedones and Neurofibroma

Sg¹

2018

CDOAJ

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“…7,8 Histopathologic features include circumscribed nodular foci of calcification with irregular clumped elastic fibers restricted in the mid dermis. 7 Ultrastructurally, mineralization occurs only in the core of elastic fibers, without peripheral deposits. 8 Inherited CL syndromes comprise a rare group of multisystem disorders characterized by loose redundant skinfolds.…”

Section: Discussionmentioning

confidence: 99%

“…Patients are also characterized by detectable coagulation deficits, especially factor X deficiency . Common ophthalmological symptoms include angioid streaks and peau d’orange, which seldom decreases visual acuity . Histopathologic results reveal polymorphous, fragmented, and mineralized elastic fibers in the upper and mid dermis .…”

Section: Discussionmentioning

confidence: 99%

“…1,5 Common ophthalmological symptoms include angioid streaks and peau d'orange, which seldom decreases visual acuity. 1,7 Histopathologic results reveal polymorphous, fragmented, and mineralized elastic fibers in the upper and mid dermis. 6 Electron microscopy further indicates that calcification can also occur in the marginal areas of elastic fibers.…”

Section: Discussionmentioning

confidence: 99%

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Progressive Generalized Skin Laxity in a Young Woman

2023

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“…This condition may be acquired or inherited (AD, AR, or X-linked), each with different genetic mutations (ELN, FBLN4 and 5, ATP6V0A2, PYCR1, ATP7A, SLC2A10, LTBP4, and RIN2), and significant clinical overlap. [45][46][47][48] In addition to multisystem defects, all subtypes present with loose skin that slowly returns to normal after stretching, unlike in EDS, and gives rise to a characteristic "bloodhound" appearance. 3,5,19,45 Cardiac involvement might be variable across the subtypes, but patients can develop aortic aneurysms, arterial tortuosity, aortic stenosis, and MVP.…”

Section: Cutis Laxamentioning

confidence: 99%

Dermatologic manifestations of pediatric cardiovascular diseases: Skin as a reflection of the heart

Jones

Patel

Valikodath

et al. 2021

Pediatric Dermatology

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Often dermatologists are the first providers to examine patients presenting with cutaneous manifestations of an occult cardiovascular disease. Significant morbidity and mortality exist between conditions with comorbid cardiovascular and cutaneous findings, and clinicians should be cognizant of such associations. From a cardiology standpoint, an initial workup will vary based on the reason for referral but frequently consists of upper and lower extremity blood pressure, a focused history of present illness (ie, associated symptoms of exertional chest pain, exertional syncope or syncope of unknown etiology, positional chest pain), family history (rule out cardiomyopathy, sudden cardiac death, etc), and a focused physical examination with an emphasis on cardiopulmonary systems. If any concerning findings are present, a complete echocardiogram will be performed to assess for pathology, which will guide the next steps. Given the care needed to treat these patients, this review aims to provide the tools needed to aid dermatologists in detecting underlying cardiovascular diseases by their associated cutaneous findings. | CONG ENITAL C ARDIOVA SCUL AR DISE A SE | RAS/MAPK pathway disordersRas is a small but important GTPase that induces the mitogen-activated protein kinase (MAPK) cascade, a critical mechanism for all cells during embryological development. Dysregulation of the Ras-MAPK pathway leads to the characteristic craniofacial phenotypes with underlying cardiac, cutaneous, musculoskeletal, and ocular abnormalities.Mutations in the Ras/MAPK pathway result in numerous syndromes, the most common of which include Noonan syndrome, Costello syndrome, cardio-facio-cutaneous syndrome, neurofibromatosis type 1, and Noonan with multiple lentigines. These are discussed below. | Noonan syndromeNoonan syndrome (NS) is characterized by congenital heart defects and cutaneous manifestations, among other clinical findings. 1,2 The

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Cutis laxa-like pseudoxanthoma elasticum with osteoma cutis

Cited by 4 publications

References 1 publication

Pseudoxanthoma Elasticum and its Rare Co-Existence with Comedones and Neurofibroma

Pseudoxanthoma Elasticum and its Rare Co-Existence with Comedones and Neurofibroma

Progressive Generalized Skin Laxity in a Young Woman

Dermatologic manifestations of pediatric cardiovascular diseases: Skin as a reflection of the heart

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