A clinicopathological study of flegel s disease hyperkeratosis lenticularis perstans. Flegel disease has been reported to rarely affect persrans families that were also prone to skin and gastrointestinal cancers. The tissue undergoes a series of steps before it reaches the examiners desk to be thoroughly examined microscopically to arrive at a particular diagnosis. Most cases are sporadic although familial cases with an autosomal dominant mode of.
Epidermal atrophy and dermal lymphocytic inflammation, characteristic of flegels disease, were not observed. Flegel first described the clinical features of hyperkeratosis lenticularis perstans. Response of penaeid shrimp to exposure to vibrio species. It is very similar to kyrle disease and has been regarded as a variant of kyrle disease, however its. Many conditions affect the human integumentary systemthe organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. On physical examination, multiple 1 to 4mm erythematous, scaly papules were noted on the anterior. Lesions are located primarily on the dorsal feet and lower legs, with. Flegel disease is also known as hyperkeratosis lenticularis perstans. No instigating factor has been identified clearly however some investigators have implicated uv light. Histopathology of crohns disease and ulcerative colitis. Hyperkeratosis lenticularis perstans also known as flegels disease is a keratinisation. Macdonalda clinicopathological study of flegels disease hyperkeratosis lenticularis perstans br j dermatol, 116 1987, pp. We also found lymphocytes in the epidermis, and these cells and macrophages were noted in the dermis. Pdf hyperkeratosis lenticularis perstans also known as flegels disease is a keratinisation disorder.
Dermatopathology reference describes hyperkeratosis lenticularis perstans flegel s disease histopathology including histologic features and provides links to additional medical references. Flegel disease is a rare dermatosis characterized by asymptomatic hyperkeratotic papules predominantly on the dorsa of the feet and lower legs. Jul 31, 20 dermatopathology reference describes hyperkeratosis lenticularis perstans flegel s disease histopathology including histologic features and provides links to additional medical references. The major function of this system is as a barrier against the external environment. It is characterised by redbrown papules with irregular horny scales located mainly on the top surface of the feet and lower legs. Monoclonal antibodies developed for sensitive detection and comparision of white spot syndrome virus isolates in india. At this stage the cause is unknown, and the possibility of an infectious agent or toxin cannot be discounted. The onset of this dermatosis was delayed until adulthood. This is a hereditary disease, the symptoms of which are hyperkeratosis, blisters and erythema. The basal membrane disappeared at some points, and. Multiple minute digitate hyperkeratosisa peculiar entity. Erythematous scaly papules on the shins and calves mdedge. Hyperkeratosis or skin thickening is often the skins normal protection against rubbing, pressure and other forms of irritation, causing calluses and corns on the hands and feet or whitish areas inside the mouth 3. Langerhans cells are highly reduced in the atrophic areas.
Pdf atypical morphology of hyperkeratosis lenticulari. The samples were taken from the oldest achromic lesions, and the biopsy sites were far from normal skin. Hyperkeratosis lenticularis perstans flegel disease. Overlying the subtle epidermal elevation was a column of compact hyperkeratosis with focal parakeratosis. Hyperkeratosis lenticularis perstans flegel s disease. Hyperkeratosis lenticularis perstans flegels disease is a rare genodermatosis characterised by multiple discrete keratoses usually on the dorsum of the feet and anterior aspect of the lower leg.
Pricklelike, flatly elevated papules with red to dark brown keratotic scales of 1 mm to 5 mm in diameter are attached. Hyperkeratosis lenticularis perstans flegels disease is a rare genodermatosis that was first described in 1958. For example, we can start our morning with corn flake sign reminding us of kyrle s and flegel s disease while enjoying mango we can remember mango dermatitis caused by toxicodendron. Local intraarticular injection of rapamycin delays. There may be a few neutrophils in the stratum corneum. The term hyperkeratosis is often used in connection with lesions of the mucous membranes, such as leukoplakia. Levers histopathology of the skin, 2nd edition cutaneous. Most cases are sporadic although familial cases with an autosomal dominant mode of inheritance have been reported. A case report of unusual multiple minute digitate hyperkeratosis. Hyperkeratosis lenticularis perstans flegel disease pathology. Hyperkeratosis lenticularis perstans also known as flegels disease is a cutaneous condition characterized by rough, yellowbrown keratotic, flattopped papules 639.
The samples were taken from the oldest achromic lesions. Histopathological differences between early and old lesions of hyperkeratosis lenticularis perstans flegel s disease. Summary the clinical and histopathological features of 12 female patients with flegels disease were studied. Flegels disease it occurs in middleaged men and women, most frequently on the extremities, especially on the dorsal surfaces of feet and hands. Add list of cutaneous conditions to your topic list for future reference or share this resource on social media. Hyperkeratosis lenticularis perstans flegels disease. Scant inflammation there is hyperkeratosis and lymphocytes are minimally increased around the superficial plexus. Flegels disease large numbers, small, discrete, greybrownredbrown, circular papules with a welldeveloped scale dorsum of foot, lower legs, upper arms. The system of correlating things will eventually help us to permanently ingrain in. Chapter 1 introducton histopathology definition it is a branch of pathology which deals with the study of disease in a tissue section. A 4 mm diameter punch biopsy of one of the lesions on the ankle was taken and sent for histo pathology at the local hospital. Vitiligo is a disease of melanocytes characterized by achromic lesions in the skin, affecting the epidermis and the pilosebaceous follicle.
The main ultra structural finding is a morphological alteration of lamellar odland bodies. The results showed that the pathological manifestations of hemocytes were similar among moribund shrimps infected via injection, feeding and by nature. Flegels disease usually develops in the fourth or fifth decade. Many conditions affect the human integumentary systemthe organ system covering the entire surface of the body and is composed of skin, hair, nails, and related muscle and glands. Histopathological differences between early and old lesions of hyperkeratosis lenticularis perstans flegels disease. Lichenoid drug eruption flegels disease co dermnet. Flegel disease yamini krishna jessica white tapati sinha arti bakshi abstract hyperkeratosis lenticularis perstans flegel disease is a rare, benign hyperkeratotic skin disorder which typically occurs in lower extremities of caucasian middleaged patients. Kyrle disease should exhibit an impressive keratin plug and inflammatory material perforating through the epidermis. A 73yearold white woman presented to the dermatology clinic with a mildly pruritic, slowly progressive rash on the shins and calves of 10 years duration. A rare and unusual hyperkeratotic disorder journal of. Bsd self assessment session edinburgh 2018 leeds virtual. A clinicopathological study of flegels disease hyperkeratosis lenticularis perstans. This benign disorder is characterized by multiple asymptomatic 1 to 5mm keratotic papules in a symmetric distribution favoring the dorsal aspects of the feet and distal extremities in adults.
A clinicopathological study of flegels disease hyperkeratosis. Aquaculture asia october 2012 network of aquaculture. The permeability of epidermis lacking normal membranecoating granules. The variability of features with time and treatment and difficult differential diagnostic problems will be discussed. Acquired perforating dermatosis in a patient with chronic. At birth, the skin of the individual is entirely covered with thick, horny, armourlike plates. Early enthusiasm for liver transplantation as therapy for patients with malignancy was tempered by high rates of tumor recurrence, particularly in patients with advanced tumors. The term hyperkeratosis is often used in connection with lesions of. Hafner c, di martino e, pitt e, stempfl t, tomlinson d, hartmann a, landthaler m, knowles m, vogt t. Recent studies have revealed that rapamycin activates autophagy in human chondrocytes preventing the development of osteoarthritis oa like changes in vitro, while the systemic injection of rapamycin reduces the severity of experimental osteoarthritis in a murine model of oa in vivo. Hyperkeratosis lenticularis perstans flegel disease hyperkeratosis lenticularis perstans, also known as flegel disease, is a rare dermatosis first described by flegel 1 in 1958.
Since the systemic use of rapamycin is associated with numerous side effects, the goal of the current study. Histopathology showed a small focus of acanthotic epidermis forming a small spire. A clinicopathological study of flegels disease hyperkeratosis lenticularis perstans br j dermatol. Jan 17, 2020 hyperkeratosis lenticularis perstans flegel disease is a rare, benign hyperkeratotic skin disorder which typically occurs in lower extremities of caucasian middleaged patients. No uniformly effective treatment agent is available for the dermatosis although emollients, topical 5. The case was then referred to the regional dermatology grand round for specialist opinion.
The secondary form of the disease is known as acquired perforating dermatosis apd. Hyperkeratosis lenticularis perstans flegel disease is a rare, benign hyperkeratotic skin disorder which typically occurs in lower extremities of caucasian middleaged patients. In all cases we noted alterations in keratinocytes, langerhans cells, and melanocytes. An ultrastmctural tracer study of kyrleflegel disease. Histopathology showing basket weave normal epidermis sharply. Multiple minute digitate hyperkeratosis a peculiar entity as a paraneoplastic syndrome, resolving after successful treatment of the accompanying laryngeal carcinoma. When the adherent scale above the papules is removed, pinpoint bleeding can be seen on the surface.
Nov 01, 2008 flegels disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized by small, red. The clinical and histopathological features of 12 female patients with flegels disease were studied. Tim flegels presentation research progress on bacterial and viral causes of ahpns, and prof. Levers histopathology of the skin, 2nd edition free ebook download as pdf file.
Fruit and food eponyms in dermatology jindal n, jindal p. Clinical and histopathological correlation confirmed the diagnosis of hyperkeratosis lenticularis perstans hlp or flegels disease. Macdonalda clinicopathological study of flegel s disease hyperkeratosis lenticularis perstans br j dermatol, 116 1987, pp. Schindler disease kanzaki disease, alphanacetylgalactosaminidase deficiency schinzelgiedion syndrome scleroatrophic syndrome of huriez huriez syndrome, palmoplantar keratoderma with scleroatrophy, palmoplantar keratoderma with sclerodactyly, scleroatrophic and keratotic dermatosis of the limbs, sclerotylosis. Erythematous scaly papules on the shins and calves. Flegels disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized by small, red. It develops in mid to late adult life and in some cases has an autosomaldominant inheritance.
Clinical and histopathological correlation confirmed the diagnosis of hyperkeratosis lenticularis perstans hlp or flegel s disease. Other forms of hyperkeratosis occur as part of the skins defence against chronic inflammation, infection and the radiation of sunlight or irritating. Hyperkeratosis lenticularis perstans flegels disease researchgate. Five species of palaemonid shrimp, macrobrachium rosenbergii, m. Geboes and others published histopathology of crohns disease and ulcerative colitis find, read and cite all the research you need on researchgate. Sep 28, 2012 flegel s disease usually develops in the fourth or fifth decade. Hyperkeratosis lenticularis perstans also known as flegel s disease is a cutaneous condition characterized by rough, yellowbrown keratotic, flattopped papules 639. Patient was prescribed oral vitamin a 5000 iu daily for 15 days, topical tretinoin cream 0. It involves the clumping of keratin filaments freedberg et al, 2003. Some of each species were artificially infected with yellow head virus yhv by injection and then monitored by rtpcr and by immunohistochemistry using monoclonal antibodies specific to 116 kda. S ir, flegels disease fd is a rare dermatosis characterized by 15 mm, asymptomatic, reddish. Certain authors have suggested that flegels disease forms part of a.
Fgfr3 and pik3ca mutations in stucco keratosis and dermatosis papulosa nigra. Hyperkeratosis lenticularis perstans also known as flegel disease, hyperkeratosis lenticularis perstans is a cutaneous condition characterised by rough, yellowbrown keratotic, flattopped papules of irregular outline measuring 15mm in diameter and up to 1mm in depth 32. We performed an ultrastructural analysis of biopsy specimens from four patients with noninflammatory, stable vitiligo of long duration three had generalized vitiligo and one had segmental vitiligo. Flegel disease, or hyperkeratosis lenticularis perstans hlp. The clinical and histopathological features of 12 female patients with flegel s disease were studied. Spiky papules on the dorsal feet mdedge dermatology. For example, we can start our morning with corn flake sign reminding us of kyrles and flegels disease while enjoying mango we can remember mango dermatitis caused by toxicodendron. Histopathology of crohns disease and ulcerative colitis k. The pathological changes of hemocytes in the haemolymph and hepatopancreas were examined in experimentally and naturally wssv white spot syndrome virus infected fenneropenaeus chinensis. The skin weighs an average of four kilograms, covers an area of two square meters, and is made of three distinct layers. Authoritative facts about the skin from dermnet new zealand. The system of correlating things will eventually help us to permanently ingrain in our minds these difficult signs and syndromes. A 4 mm diameter punch biopsy of one of the lesions on the ankle was taken and sent for histopathology at the local hospital.
The condition may be sporadic or inherited as an autosomal dominant condition. Clinical input is often very helpful in making a diagnosis histopathologically. Hematological changes in white spot syndrome virus. Mar 01, 2002 s ir, flegels disease fd is a rare dermatosis characterized by 15 mm, asymptomatic, reddish.
Pdf differences in susceptibility of palaemonid shrimp. Hyperkeratosis lenticularis perstans flegel disease diagnostic. The diagnosis is based on the clinical appearance in association with the typical histologic features of orthohyperkeratosis and a subepidermal band. You can help by expanding it with reliably sourced entries. List of skin conditions wikimili, the best wikipedia reader. Flegels disease treated with psoralen ultraviolet a. Commonly seen in middle aged women autoimmune disease characterized by hyperthyroidism due to circulating autoantibodies against thyrotropin tsh receptor that activates the receptor, leading to increased thyroid hormone synthesis and secretion and growth of the thyroid gland. This is an incomplete list, which may never be able to satisfy particular standards for completeness. Pdf histopathology of crohns disease and ulcerative colitis. Electron microscopy shows reduction in membranecoating granules odland bodies within the lesional keratinocytes.