91/719. Uncommon vascular naevi associated with focal acantholytic dyskeratosis. Cutis marmorata telangiectatica congenita and vascular twin naevi are rare vascular anomalies in which focal acantholytic dyskeratosis is usually not observed. We describe a 44-year-old-man who presented for evaluation of skin lesions that had been present since birth. physical examination revealed anaemic macules adjacent to a naevus telangiectaticus on the chest. Naevus anaemicus was also seen on the shoulders, arms, and left leg. There was bluish-reddish reticulate marking of the skin and cutaneous atrophy. Shortening and hypoplasia of the left leg was observed. Histologic examination of two biopsy specimens revealed focal acantholytic dyskeratosis. In vivo confocal laser scanning microscopy showed dilated capillaries and vessels of the upper dermal plexus in the telangiectatic and decreased capillary blood flow in the anaemic skin sites. The findings were consistent with a diagnosis of cutis marmorata telangiectatica congenita, vascular twin naevi, and incidental focal acantholytic dyskeratosis. The particularities of the present case are the following: firstly, the association of two rare vascular anomalies to which the genetic concept of mosaicism can be applied; secondly, the occurrence of incidental focal acantholytic dyskeratosis in sites of vascular naevi. ( info) |
92/719. Chronic acquired dyskeratotic papulosis of the face. A vietnam veteran who had been exposed to Agent Orange presented with a 28-year history of a papulonodular disease mainly affecting the face. Over those years, pin-sized papules had developed into dark nodules. Multiple biopsies of both fresh and mature papulonodules had been performed for routine histopathology and electron microscopy. Results had been positive for spongiosis with exocytosis, acanthosis, dyskeratotic keratinocytes, and marked incontinence of pigment, which together are suggestive of a previously unreported clinical entity that we term chronic acquired dyskeratotic papulosis. ( info) |
93/719. aminolevulinic acid hydrochloride with photodynamic therapy: efficacy outcomes and recurrence 4 years after treatment. The safety and efficacy of treating individuals who presented with multiple actinic keratosis (AK) lesions with 5-aminolevulinic acid (ALA) in combination with photodynamic therapy (PDT) were documented in a phase III trial. This report highlights results of this phase III trial and reviews 4 specific cases of sustained AK lesion clearance 4 years after treatment with ALA/PDT Long-term recurrence data were collected from patients who participated in clinical trials of ALA/PDT Long-term evaluation extended to 36 to 48 months (4 years) supports primary efficacy findings of the phase III pivotal trial, with a low incidence of AK recurrence in patients treated with ALA/PDT ( info) |
94/719. The evolving role of aminolevulinic acid hydrochloride with photodynamic therapy in photoaging. The safety and efficacy of treating individuals who presented with multiple actinic keratosis (AK) lesions with 5-aminolevulinic acid (ALA) in combination with photodynamic therapy (PDT) were documented in a phase III trial. This report highlights results of this phase III trial and reviews 2 specific case studies treated with ALA/PDT who presented with both multiple nonhyperkeratotic AK lesions and moderate-to-severe photodamage. Treatment consisted of a 2-step process performed by the investigator. In addition to the pretreatment evaluation, each subject was evaluated at 1- and 2-month intervals after each treatment of ALA/PDT. Clinical findings reveal complete healing at the site of ALA/PDT without scarring or changes in pigmentation, as well as significant changes in signs of photodamage, such as improvement in skin elasticity and reduction in skin thickening. ( info) |
95/719. sports-related pads. Knuckle pads, first described by Garrod in 1893, 1 are benign, asymptomatic, well- circumscribed, smooth, firm, skin colored papules, nodules, or plaques. They most commonly occur on the dorsal aspect of the proximal interphalangeal joint of the finger, 2 but also may occur on the dorsal aspects of the foot over joints. 3-5 Knuckle pads may be inherited or acquired. 1 While some authors suggest that trauma is not a significant factor, 6 acquired knuckle pads have been associated with repetitive friction and trauma, 2,7 and may resemble athlete's nodules (also referred to as collagenomas). 1 Histologically, knuckle pads are characterized by hyperkeratosis, hypergranulosis, proliferation of fibroblasts and capillaries, and thickened and irregular collagen bundles. 1 Few cases of knuckle pads involving the lower extremities have been reported. 7 Knuckle pads of the feet have been described in association with inherited syndromes, such as acrokeratoelastoidosis Costa, 3 a syndrome of knuckle pads, leukonychia and deafness, 4 and a syndrome of knuckle pads, leukonychia, deafness and keratosis palmoplantaris. 5 We report a case that illustrates an unusual presentation of acquired knuckle pads of the feet secondary to repetitive friction from athletic gear. ( info) |
96/719. Vascular variant of keratosis lichenoides chronica associated with hypothyroidism and response to tacalcitol and acitretin. keratosis lichenoides chronica (KLC) is a rare chronic progressive cutaneous disease that is part of the heterogeneous group of lichenoid dermatoses. The typical clinical presentation is characterized by lichenoid hyperkeratotic papules and nodules arranged in a linear and reticular pattern on the trunk and extremities. Our case confirms the existence of a vascular variant of KLC. There is no consensus about its treatment, since it is refractory to many different treatment modalities. We report the effectiveness of acitretin in KLC in combination with tacalcitol. KLC is of unknown aetiology, but is perhaps associated with systemic diseases, most importantly glomerulonephritis and lymphoma. This is the second case associated with hypothyroidism. ( info) |
97/719. arsenic keratosis and pigmentation accompanied by multiple bowen's disease and genitourinary cancer in a psoriasis patient. We report a case of arsenic keratosis and pigmentation accompained by multiple bowen's disease and genitourinary cancer in a 64-year-old man. He was a psoriasis patient with a history of herbal medication for about thirty years. He showed multiple hyperkeratotic plaques on the bilateral palms, soles, and multiple, brownish, scaly, elevated papules on the back in addition to diffuse hyperpigmentation. biopsy confirmed arsenic keratosis and bowen's disease. Transitional cell carcinoma was also detected on his ureter and bladder during follow-up. The skin lesions were treated with topical 5-fluorouracil, etretinate, and excision with improvement. ( info) |
98/719. Acrokeratoelastoidosis. Acrokeratoelastoidosis is a genodermatosis characterized by firm papules or plaques on the sides of the hands and feet. Although poorly understood, the lesions may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. In this report, we will present a patient with this rare condition and review the clinical and histopathologic features, cause, and differential diagnosis. ( info) |
99/719. Multiple hyperkeratotic spicules and myeloma. We report the case of a 58-year-old patient with multiple myeloma with a monoclonal gammopathy of the IgG type, who developed a striking eruption characterized by multiple hyperkeratotic spicules of the face, nose and scalp. light-microscopic studies revealed a follicular orthohyperkeratosis, while direct immunofluorescence microscopy remained negative. Hence, our patient presented features characteristic of a rare distinctive disorder associated with an underlying paraprotein or multiple myeloma, paraneoplastic hyperkeratotic spicules. ( info) |
100/719. A rare association of epidermal nevus syndrome and ainhum-like digital constrictions. Epidermal nevus syndrome is an unusual occurrence of epidermal nevi with central nervous system (CNS), eye, and skeletal developmental abnormalities. We report an as yet undocumented association of generalized epidermal nevus with striate keratoderma, ainhum-like digital constriction, and autoamputation of the right fifth toe in a 10-year-old girl. The excellent response to oral acitretin with softening of the constrictions is also highlighted. ( info) |