Cutaneous lentiginosis with atrial myxomas

Reed, O. M.; Mellette, J. Ramsey; Fitzpatrick, James E.

doi:10.1016/s0190-9622(86)70188-6

Cited by 34 publications

(11 citation statements)

References 17 publications

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“…Nevertheless most cases of widespread lentiginosis are associated with a variety of physical non-cutaneous abnormalities affecting the cardiac, musculoskeletal, neurological, reproductive, gastrointestinal and auditory system [21,22,23,24,25,26,27,28,29,30,31,32,33,34,35]. LEOPARD syndrome and Carney complex (LAMB and NAME syndrome) are well-characterized disorders, both showing autosomal dominant hereditary patterns [21,36,37].…”

Section: Discussionmentioning

confidence: 99%

Generalized Solar Lentigines in a Patient with a History of Radon Exposure

Mauerer

Betz²,

Pasternack³

et al. 2010

Dermatology

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A woman with generalized lentigines without associated non-cutaneous abnormalities is described. The patient showed brownish-pigmented flat or slightly elevated spots with a diameter of 1–5 mm. The histopathology of the lesions was compatible with a diagnosis of solar lentigines (SLs) or flat seborrhoeic keratosis. Unlike SLs, which develop typically on sun-damaged skin of the face, the dorsum of the hands and forearms, this patient showed the lentigines most prominently on the thighs and lower legs. Besides increased recreational UV exposure, the patient had a history of occupational radon exposure in a spa with radon-containing water. Genetic analysis identified a p.S249C FGFR3 hotspot mutation in 1 lesion, supporting the diagnosis of SLs. It remains elusive whether the occupational exposure to radon-containing water in addition to the recreational UV light exposure caused the unusual distribution of the SLs in this patient.

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Section: Discussionmentioning

confidence: 99%

Generalized Solar Lentigines in a Patient with a History of Radon Exposure

Mauerer

Betz²,

Pasternack³

et al. 2010

Dermatology

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“…Pigmented macules on the vulva have been described as part of the LAMB syndrome (lentigines, atrial myxoma, mucocutaneous myxomas, blue naevi) (Reed et al 1986, Rhodes et al 1986). In the Carney complex (cutaneous and atrial myxomas, mucocutaneous pigmentation, endocrine abnormalities and schwannomas), facial pigmentation is usual, but multiple blue/black macules on the vulva were the presenting feature in one case (Pandolfini et al 2001).…”

Section: Lentiginesmentioning

confidence: 99%

Non‐Infective Cutaneous Conditions of the Vulva

Neill

Lewis

2009

Ridley's the Vulva

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“…These macules, most often facial, were found in 96% of patients in one series 2 . These facial lesions are immediately obvious when reviewing photographs in prior case reports of the Carney complex 4,6 …”

Section: Discussionmentioning

confidence: 87%

“…Initial presentation of the Carney complex with vulvar macules is unusual. Although prior reports of the Carney complex have described patients with darkly pigmented macules of the genitals, all of these cases also exhibited significant darkly pigmented macules of the face 4,6 , 7 . The overall prevalence of vulvar macules in the Carney complex is not known.…”

Section: Discussionmentioning

confidence: 88%

Pigmented vulvar macules as a presenting feature of the Carney complex

2001

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A 27‐year‐old woman was referred by her gynecologist for asymptomatic pigmented vulvar lesions first noted 2 years previously. Medical history was significant for a personal history of atrial myxoma, diagnosed due to a systolic murmur, along with a family (maternal) history of atrial myxoma. The patient also reported irregular menses, increasing and darkening of body and facial hair, and an increase in shoe size. Physical examination revealed multiple 2‐5 mm dark brown to black macules on the labia majora and minora. (Fig. 1) Faint scattered light brown macules were noted on the chest and face. A 2‐mm brown scleral macule was present adjacent to the iris, and scattered small pigmented macules were present on the vermilion border of the lips. A 4‐mm blue‐black papule was found on the upper back, and several firm, 2 mm flesh‐colored papules were found on the abdomen and thigh. Hypertrichosis of both forearms was present, and enlargement of the jaw and hands was noted. 1 Multiple dark brown to black macules on the labia majora and minora Histopathologic evaluation of the blue‐black papule was consistent with a blue nevus, with elongated pigmented spindle cells. Although polygonal cells were noted in the main part of the lesion, these did not meet the criteria for the epithelioid variant of blue nevus, as they were smaller. Histopathologic evaluation of the abdominal lesion (Fig. 2) revealed spindle‐shaped fibroblasts and a mucinous stroma within the upper dermis consistent with a cutaneous myxoma. 2 Hematoxylin‐eosin stain at 112× magnification reveals spindle‐shaped fibroblasts and a mucinous stroma Endocrinologic evaluation revealed normal serum levels of prolactin, free and total testosterone, DHEA‐S, and AM cortisol as well as normal thyroid function tests. A 24‐ h urine collection for 17‐ketosteroids and cortrosyn stimulation tests were also within normal limits. Both somatomedin C and growth hormone levels, however, were elevated, at 970 ng/mL (114‐942 ng/mL) and 41.3 ng/mL (0‐10 ng/mL), respectively, consistent with acromegaly. MRI revealed a focal pituitary mass, compatible with a primary pituitary neoplasm. The patient underwent surgical resection of the tumor, which was found to be a growth hormone‐producing pituitary adenoma. The diagnosis of Carney complex was made, due to cutaneous and mucosal lentigines, cutaneous myxomas, blue nevus, atrial myxoma, pituitary neoplasm with acromegaly, and a positive family history.

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Cutaneous lentiginosis with atrial myxomas

Cited by 34 publications

References 17 publications

Generalized Solar Lentigines in a Patient with a History of Radon Exposure

Generalized Solar Lentigines in a Patient with a History of Radon Exposure

Non‐Infective Cutaneous Conditions of the Vulva

Pigmented vulvar macules as a presenting feature of the Carney complex

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