1/14. The hyperkeratotic variant of disseminated superficial actinic porokeratosis (DSAP).A 78-year-old South Korean man was referred to us from the Medical intensive care Unit (MICU) for an opinion. He was comatose and was on ventilatory care due to aspiration pneumonia. Multiple tiny papules had developed 10 years previously and since then the number and size had been increasing gradually. He had been diabetic for the past 4 years, and had Parkinson's disease diagnosed 1 year previously. Laboratory examinations revealed an elevated level of white blood cells (WBCs) (25,000/microL) and decreased hemoglobin (8.8 g/dL). Other laboratory results were negative or within normal limits. skin examination showed multiple, discrete, crust-like, brownish papules over the erythematous base on the face, upper extremities, and lower extremities. With the clinical impressions of irritated verruca vulgaris, seborrheic keratosis, or cutaneous fungal infection, a skin biopsy was taken from a papule on the left shin, and histopathologic examination revealed several pronounced hyperkeratotic and parakeratotic columns, and characteristic cornoid lamellae in the stratum corneum. Beneath the cornoid lamellae, the granular layer was decreased. A number of round or oval, dyskeratotic, homogenized eosinophilic cells with pyknotic nuclei were scattered in the prickle cell layer below the cornoid lamellae. A mild lymphohistiocytic infiltrate was observed in the papillary dermis and around the blood vessels in the upper dermis. Also, actinic degeneration was present in the upper dermis.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/14. Squamous cell carcinoma arising from lesions of porokeratosis palmaris et plantaris disseminata.We report a 63-year-old Japanese man with numerous hyperkeratotic papules of porokeratosis palmaris et plantaris disseminata (PPPD) who developed multiple squamous cell carcinomas on the lesional sites of the palms and soles. The hyperkeratotic papules, which showed tightly packed columns of parakeratotic cells in the cornified layer (cornoid lamella), lost granular layer, and dyskeratotic keratinocytes in the epidermis below the cornoid lamella histologically, had been noticed on the palms and soles from the age of 28 and 43, respectively. He has no family history of such hyperkeratotic papules. Treatment with etretinate (10-50 mg/day) was given discontinuously, and the total dose of etretinate amounted to approximately 21 g over 14 years (average: 0.07 mg/kg/day). He noticed erosions on the hyperkeratotic papules on the left sole and palm more than 9 months after cessation of treatment with etretinate. Histological findings showed numerous atypical keratinocytes in the epidermis and upper dermis with mononuclear cell infiltration seen in the upper dermis. The diagnosis of squamous cell carcinoma arising from the lesions of porokeratosis palmaris et plantaris was made. Five erosions with histologically malignant changes were removed 1 cm from the margin of the erosions. These findings suggest that etretinate may have an inhibitory action on malignant changes in PPPD.- - - - - - - - - - ranking = 1.3333333333333keywords = dermis (Clic here for more details about this article) |
3/14. Secondary cutaneous amyloidosis in disseminated superficial porokeratosis: a case report.Disseminated superficial porokeratosis (DSP) is a rare cause of secondary cutaneous amyloidosis. An 83-year-old male patient showed an increase in both size and number of DSP lesions after contracting pulmonary tuberculosis. The DSP lesions of the patient consisted of numerous annular eruptions on both sun-exposed and sun-protected areas, which occurred over a period of 20 years. Multiple skin biopsies were taken from normal or lesional/sun-exposed or sun-protected skin samples. Histopathologic examination included routine H&E stains, congo red stains, thioflavin-T stains and anticytokeratin antibodies (AE1, AE3). And the results were as follows; 1) Positive staining with congo red and thioflavin-T indicated an amyloid nature for the deposits, 2) confinement of the amyloid deposition just below the lesional epidermis (while sparing the neighboring uninvolved or distant normal skin) indicated some role of the lesional epidermis, and 3) positive staining with AE3 further indicated an epidermal origin-type II epithelial keratin-of the amyloid. We present a case of DSP with a local amyloid deposit, characterized by association of positive familial background, severe pruritus and pulmonary tuberculosis.- - - - - - - - - - ranking = 0.66666666666667keywords = dermis (Clic here for more details about this article) |
4/14. Treatment of lichen amyloidosis (LA) and disseminated superficial porokeratosis (DSP) with frequency-doubled Q-switched Nd:YAG laser.BACKGROUND: Under normal practice, the full thickness of the epidermis is peeled off when treating pigmented lesions with a frequency-doubled Nd:YAG laser. Based on this observation, it is postulated that this laser may be effective for treating lichen amyloidosis (LA) and disseminated superficial porokeratosis (DSP) for which the pathologic changes are limited to the epidermis and papillary dermis. OBJECTIVE: To investigate the clinical effect of frequency-doubled Nd:YAG laser treatment for one patient with LA and for one patient with DSP. methods: Frequency-doubled 532 nm Nd:YAG laser pulses were delivered to the lesions on the limbs of a patient with LA and the face and forearms of a patient with DSP. Lesions of LA were treated two or three times, and those of DSP were treated four times, treatment sessions being 1 month apart. For this investigation, biopsies were taken from untreated lesions prior to treatment, lesions immediately after laser treatment, and lesions present at a 9-month follow-up investigation. RESULTS: Both the patient with LA and the patient with DSP responded well to treatment, the results of which remained unchanged at a follow-up conducted 9 months after the final treatment session. CONCLUSION: Frequency-doubled Nd:YAG laser treatment provided excellent results for the patient with LA as for the patient with DSP. The effectiveness of this method deserves further study in a larger group of test patients.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/14. Hereditary non-polyposis colorectal cancer associated with disseminated superficial porokeratosis. microsatellite instability in skin tumours.A 73-year-old man presented with typical lesions of disseminated superficial porokeratosis (DSP) and multiple seborrhoeic keratoses on his face, trunk and extremities, and later developed a keratoacanthoma on his lip. He belonged to a cancer-prone pedigree susceptible to colonic, uterine and other internal cancers, and had a personal history of early gastric cancer and advanced adenocarcinoma of the descending colon without adenomatous polyps at age 59 years. polymerase chain reaction amplification of skin samples for seven separate microsatellite polymorphisms revealed microsatellite instability (MSI) at multiple loci in five of six seborrhoeic keratoses and the keratoacanthoma, strongly suggesting underlying defects in dna mismatch repair. Although no germline mutations in two mismatch repair genes hMSH2 and hMLH1 were found, our patient was recognized as having hereditary non-polyposis colorectal cancer (HNPCC) based on the family history and the findings of the microsatellite analysis of skin tumours. This confirmed the usefulness of detection of MSI in prevalent and readily accessible skin lesions, including non-sebaceous non-dysplastic tumours such as seborrhoeic keratosis in the screening of HNPCC families. Although DSP may also be inherited as an autosomal dominant condition, this particular skin disease appeared to be sporadic in our patient and, to our knowledge, no association of DSP or other forms of porokeratosis with HNPCC has previously been reported. In contrast to the seborrhoeic keratoses and keratoacanthoma, no MSI was observed in two samples from DSP lesional epidermis examined.- - - - - - - - - - ranking = 0.33333333333333keywords = dermis (Clic here for more details about this article) |
6/14. Postirradiation multiple minute digitate porokeratosis.BACKGROUND: Development of multiple minute digitate hyperkeratoses (MMDH) after irradiation has been reported previously. The keratotic lesions in these cases were confined within the irradiation field, and histopathological examination disclosed a focal column of parakeratosis (cornoid lamella) arising from an epidermis devoid of granular layer. OBJECTIVE: We describe a 78-year-old woman who developed multiple, discrete, tiny, filiform, keratotic papules on the anterior aspect of the right chest wall, 13 months after postmastectomy cobalt irradiation therapy for mammary infiltrating ductal carcinoma. CONCLUSION: Postirradiation MMDH represents a peculiar radiation-induced disorder that we believe should be distinguished from other cases of MMDH and included within the spectrum of porokeratosis.- - - - - - - - - - ranking = 0.33333333333333keywords = dermis (Clic here for more details about this article) |
7/14. Disseminated porokeratosis with fatal metastatic squamous cell carcinoma: an additional case of "malignant disseminated porokeratosis".We report on a patient with a disseminated form of porokeratosis in whom bowenoid lesions and squamous cell carcinoma developed in an apparent sequential progression. Local and disseminated metastases ensued, resulting in the death of the patient. Furthermore, we found an overexpression of the p53 protein in the keratinocytes beneath and adjacent to the cornoid lamella in the porokeratotic lesion and throughout the epidermis in a bowenoid lesion. Although malignancy has been reported previously in various types of porokeratosis, the development of fatal metastatic squamous cell carcinoma in the setting of this disease is a rare event. The histopathologic findings of this case document the association of porokeratotic lesions with bowenoid dysplasia and aggressive squamous cell carcinoma and confirm that a p53 functional aberration can be important in a malignant outcome such as this.- - - - - - - - - - ranking = 0.33333333333333keywords = dermis (Clic here for more details about this article) |
8/14. An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease).An 82-year-old female presented with an 11-year history of multiple, symmetrically disseminated hyperkeratotic red-brown papules 3-5 mm in diameter on the dorsal aspects of the upper and lower extremities. The eruption first appeared on the dorsa of the feet and on the lower parts of the legs. During the last few years, the lesions extended gradually to the upper part of the legs and thighs, also disseminating over the trunk. There was no family case history of Flegel's disease. Histopathological examination revealed a hyperkeratosis with a focal parakeratosis overlying the thinned epidermis. In the papillary dermis there was a dense band-like infiltrate composed of lymphocytes and some histiocytes. Electron microscopy showed a normal number of membrane-coated granules (Odland bodies). The clinical and histopathological findings in the patient corresponded with the diagnosis of hyperkeratosis lenticularis perstans. The patient was treated with PUVA (psoralen plus ultraviolet A)-bath therapy, retinoid plus PUVA and calcipotriol. However, all of these treatments were unsuccessful.- - - - - - - - - - ranking = 0.66666666666667keywords = dermis (Clic here for more details about this article) |
9/14. Inflammatory stage of disseminated superficial porokeratosis.Disseminated superficial porokeratosis (DSP) is a keratinization disorder characterized by multiple small lesions with a slightly elevated, sharply defined ridge over the whole body. Unusual DSP cases with acute exacerbation of their lesions accompanied by severe pruritus have been reported and designated as "eruptive pruritic papular porokeratosis" or "inflammatory DSP". Histologically, the pruritic lesions in the majority of these unusual DSP cases had a dense infiltration of eosinophils and lymphocytes in the vicinity of blood vessels in the upper dermis. In this report, we describe an additional case of DSP with a similar clinical course and histopathological findings. A review of the literature showed that the pruritic condition in these unusual DSP cases can be transient and is not necessarily related to spontaneous regression. We propose the term "Inflammatory stage of DSP" for describing this unusual variant of DSP.- - - - - - - - - - ranking = 0.33333333333333keywords = dermis (Clic here for more details about this article) |
10/14. Generalized linear porokeratosis.A 23-year-old woman was seen for widespread skin lesions present since the age of 2.5 years. Twenty years ago, she developed a brown macular lesion on her right buttock. The lesion became hyperkeratotic and subsequently spread through the posterior aspect of her right leg. It later spread to the right side of the trunk and to the right arm. When she was 9 years old, she developed similar lesions on her left arm and leg. After she was 13 years old, no new skin lesions appeared. There was no family history of similar lesions. On examination, there were numerous linear and whorled, reddish-brown, hyperkeratotic plaques, with central atrophy and raised borders, following Blaschko's lines on all of the extremities. These lesions on the extremities extended to the dorsum of the hands and feet (Fig. 1). She had hyperkeratotic lesions on the pressure points of both of the soles, but no palm involvement. The number of lesions on the right side was greater than that on the left. Reddish-brown annular plaques with central atrophy and raised borders, appearing in zosteriform configuration, and numerous individual 2-3-mm erythematous lichenoid papules were observed on the right side of the thorax and the right inguinal region (Fig. 2). No face, scalp, or mucous membrane involvement was seen. The nails of the second and fifth fingers of the right hand and the nail of the third finger of the left hand showed nail dystrophy with longitudinal ridges and pterygium. All the nails of the right foot and the nails of the first and fifth toes of the left foot showed dystrophic changes with subungual keratosis. The patient was otherwise in good health. Two biopsy specimens taken from a hyperkeratotic plaque and a lichenoid papule showed an epidermal invagination with angulated parakeratotic tier, denoting cornoid lamella. The epidermis just underneath the cornoid lamella displayed vacuolization and the granular layer was absent. The adjacent epidermis was atrophic, and hydropic degeneration within the basal cell layer was seen. In the dermis, a nonspecific, mild, chronic, inflammatory cell infiltrate, telangiectatic vessels, and pigment-laden macrophages were present. These findings were consistent with linear porokeratosis (Fig. 3). Microscopic examinations and mycologic cultures of the nails were negative. We decided to treat our case systemically with retinoids, but the patient refused this therapy. So, topical tretinoin 0.05% was started once a day. A marked improvement was observed in hyperkeratosis through the first 4 weeks of treatment and plateaued at 8 weeks. After 10 weeks, the lesions had almost disappeared. We planned to continue the applications every other day. One year later, she remains stable with application of topical tretinoin 0.05% twice a week and is satisfied with the final appearance. She is under regular follow-up.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
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