Verrucous and Malodorous Vegetations on the Legs

Rongioletti, Franco; Cestari, Rossella; Rebora, Alberto

doi:10.1001/archderm.1992.01680130117019

Cited by 4 publications

(2 citation statements)

References 6 publications

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“…Clinical variants include hypertrophic, 83 vesiculobullous, 84–86 and linear 87–93 types. Keratotic lesions disposed in a unilateral, linear, zosteriform, or localized pattern, possessing features of dyskeratotic acantholysis, often create a debate about whether Darier's disease or a localized epidermal nevus is present.…”

Section: Clinical Featuresmentioning

confidence: 99%

Darier's (Darier–White) disease/keratosis follicularis

Sehgal¹,

Srivastava²

2004

Int J Dermatology

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Section: Clinical Featuresmentioning

confidence: 99%

Darier's (Darier–White) disease/keratosis follicularis

Sehgal¹,

Srivastava²

2004

Int J Dermatology

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“…Only four other reports have described this variant. Three cases had typical and extensive involvement of the trunk 5–7 . All cases demonstrated typical histopathologic features.…”

Section: Discussionmentioning

confidence: 88%

Cornifying Darier's disease

2000

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A 48-year-old Caucasian man recounted the onset of keratotic papules on the trunk at the age of 8 years, with subsequent spread to the forearms, scalp, and forehead. His most severe disease was present on the legs. He complained of pain, itching, and noted exacerbations in the summer and with sweating. The family history was negative. On physical examination, the most striking finding was that of extensive, markedly hyperkeratotic plaques on the lower legs >(Fig. 1). His scalp, forehead, chest, and back exhibited mild involvement, with scattered brown keratotic papules, while his forearms showed mildly hyperkeratotic plaques. Flat-topped brown papules were present on the dorsum of the hands, with a few keratotic papules on the palms, and a few nails with distal notching and red longitudinal streaks. There were no palmar pits or oral mucosal lesions. A shave biopsy was performed of a plaque on the leg, and showed a papillomatous and markedly hyperkeratotic lesion >(Fig. 2). Suprabasal acantholysis in the elongated rete produced characteristic lacunae. The acantholysis was associated with dyskeratosis including corps ronds and grains >(Fig. 3). Together, these features were characteristic of Darier's disease. Treatment years earlier with topical retinoids, topical steroids, topical keratolytics, and multiple oral antimicrobials had been unsuccessful, and isotretinoin had been discontinued due to elevated triglycerides. Treatment was initiated with acitretin and, after 3 months, mild improvement was noted

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Inherited Acantholytic Disorders

Zamiri

Munro

2016

Rook's Textbook of Dermatology, Ninth Edition

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Darier disease (DD) and Hailey–Hailey disease (HHD) are autosomal dominant disorders characterized by epidermal acantholysis. They have a similar molecular pathogenesis, with mutations in the intracellular calcium pumps of the endoplasmic reticulum (SERCA2) and Golgi (SPCA1), respectively. DD presents with persistent, hyperkeratotic papules in a seborrhoeic distribution, associated with nail changes and palmar pitting. HHD presents with painful, mainly flexural, erosions. In both, secondary infection is common and hypertrophic flexural disease can be disabling. Histologically, both are characterized by loss of epidermal intercellular adhesion (acantholysis). In DD, acantholysis is predominantly suprabasal and there is overlying dyskeratosis. In HHD, acantholysis is more diffuse. First line topical treatment is with emollients, antimicrobials and corticosteroids. Oral antibiotics are often necessary for exacerbations; other systemic options include retinoids and ciclosporin. Physical measures such as laser resurfacing, photodynamic therapy, botulinum toxin and surgical interventions can be considered in refractory cases.

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Verrucous and Malodorous Vegetations on the Legs

Cited by 4 publications

References 6 publications

Darier's (Darier–White) disease/keratosis follicularis

Darier's (Darier–White) disease/keratosis follicularis

Cornifying Darier's disease

Inherited Acantholytic Disorders

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