1/6. 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 = 1keywords = ridge (Clic here for more details about this article) |
2/6. A case of giant porokeratosis with vestiges of a cornoid lamella.We report a case of giant porokeratosis combined with ulcerative squamous cell carcinoma. In our patient, we biopsied the skin, including the edge of the skin lesion, four times until we obtained histologic proof of a cornoid lamella. After we had established the diagnosis, we totally excised the affected skin including a 10-mm safety margin, because such lesions tend to develop into skin cancer. We reconstructed the excised area using a skin graft. After the operation, we took 13 samples of skin (seven from the lower leg and six from the sole of the foot) from the edge of the main lesion, including a putative cornoid lamella. Five of the six samples from the sole and one of the seven from the lower leg were demonstrated histologically to include a cornoid lamella. Our results suggest that skin biopsies should be taken from various sites at the edge of a giant porokeratotic lesion and in particular from the prominent ridge to prove the presence of a cornoid lamella. Shallow keratin-filled invaginations and underlying squamous cells with eosinophilic cytoplasm were observed at the edge of the lesion on the lower thigh, which might suggest a diagnosis of porokeratosis with an incomplete cornoid lamella if porokeratosis was strongly suspected from the patient's clinical features. Better recognition of giant porokeratosis is required, so that an earlier diagnosis can be made and appropriate therapy initiated in a timely manner.- - - - - - - - - - ranking = 1keywords = ridge (Clic here for more details about this article) |
3/6. A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-flourouracil as treatment modalities.A 19-year-old male presented with linear raised lesions over extensive areas of his body that followed Blaschko's lines. The lesions were asymptomatic with ridged borders. Histopathology confirmed a diagnosis of porokeratosis. He was given topical tretinoin and topical flourouracil for local application on specified areas. The cosmetic acceptability and the safety profile of tretinoin were found to be better than those of flourouracil. However, both the agents were equally efficacious. A rare case of extensive porokeratosis with comparative evaluation of two treatment modalities is hereby presented.- - - - - - - - - - ranking = 1keywords = ridge (Clic here for more details about this article) |
4/6. 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 = ridge (Clic here for more details about this article) |
5/6. Genital porokeratosis of Mibelli.porokeratosis of Mibelli is a disorder of epidermal proliferation in which many different clinical forms can be distinguished. Two male patients with a localized type of porokeratosis limited to the genitalia are reported. Later in life they developed an annular skin lesion with peripheral keratotic ridge. The histological examination of a biopsy specimen showed the characteristic features of porokeratosis. There was no family history of similar skin disorders and the patients were not on any drugs. Genital porokeratosis is probably underdiagnosed and we believe that these patients should be followed up on account of the precancerous potential of this disease.- - - - - - - - - - ranking = 1keywords = ridge (Clic here for more details about this article) |
6/6. Porokeratotic palmoplantar keratoderma discreta--a new entity or a variant of porokeratosis plantaris discreta?We report a family with hyperkeratotic lesions on palms and soles. The lesions became evident in the second to third decade, and there is an autosomal dominant mode of transmission. skin biopsy specimens show a central epidermal depression filled by a compact hyperkeratotic plug of columnar parakeratosis, like a broad cornoid lamella. The lesions resemble porokeratosis plantaris discreta clinically and histologically. The cornoid lamella is a broad, solid keratin plug rather than a centrifugally enlarging annular or serpentine ridge as can been seen in other types of porokeratosis. Perhaps the lesions of porokeratosis plantaris discreta should not be classified as a true porokeratosis but as porokeratotic plantar keratoderma discreta. We have therefore called the lesions in our patients porokeratotic palmoplantar keratoderma discreta, and suggest that porokeratotic palmoplantar keratoderma discreta is a variant of porokeratosis plantaris discreta.- - - - - - - - - - ranking = 1keywords = ridge (Clic here for more details about this article) |