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Background
This is a rare dermatosis also known as hyperkeratosis lenticularis perstans. It is characterized by asymptomatic hyperkeratotic papules ranging from 1-5 mm occurring predominately on the dorsa of the feet and lower legs. A rare disseminated variants has been reported.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Hyperkeratosis lenticularis perstans INCIDENCE/
PREVALENCEMay be sporadic or inherited as an autosomal dominant condition AGE SEX GEOGRAPHY EPIDEMIOLOGIC ASSOCIATIONS
DISEASE ASSOCIATIONS CHARACTERIZATION
PATHOGENESIS CHARACTERIZATION GENERAL
- Hyperkeratosis lenticularis perstans (Flegel's disease). In situ characterization of T cell subsets and Langerhans' cells.
Buchner SA.
Department of Dermatology, University of Basle, Switzerland.
Acta Derm Venereol. 1988;68(4):341-5. Abstract quote
We report a patient with hyperkeratosis lenticularis perstans (HLP) manifesting as multiple reddish-brown hyperkeratotic papules on the lower extremities.
Typical histologic features of HLP include hyperkeratosis, thinning or absence of the granular layer and a band-like infiltrate in the upper dermis underlying an atrophic epidermis. In order to determine the cellular composition of the infiltrate, skin biopsy specimens were studied immunohistochemically using a series of commercially available monoclonal antibodies.
The dermal infiltrate consists predominantly of helper/inducer T cells (Leu-4+, Leu-3a+). Suppressor/cytotoxic T cells (Leu-2a+) were fewer at the periphery of the infiltrate. The majority of T cells were activated as they expressed HLA-DR-antigen. Large numbers of Leu-6+ Langerhans' cells were observed at the dermo-epidermal interface. Few natural killer cells (Leu-11b+) were noted within the dermal infiltrate.
These findings support the hypothesis than an active cellular immune reaction involving the epidermis is of pathogenic importance for HLP.
LABORATORY/
RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC LABORATORY MARKERS
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL VARIANTS GENERALIZED
- An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease).
Miljkovic J.
Department of Dermatology, Maribor Teaching Hospital, Maribor, Slovenia.
Wien Klin Wochenschr. 2004;116 Suppl 2:78-80. Abstract quote
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.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
- Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease).
Ando K, Hattori H, Yamauchi Y.
Department of Dermatology, Japanese Red Cross Nagoya First Hospital, Nagoya, Japan.
Am J Dermatopathol. 2006 Apr;28(2):122-6. Abstract quote
A case of hyperkeratosis lenticularis perstans (Flegel's disease) was studied histopathologically comparing early and old lesions. The age of the lesions were judged by both the patient's history and direct observation.
The histopathologic and immunophenotypic features of a new lesion were essentially in accordance with previous findings. However, the old lesion had obviously different pathologic features. These included the absence of epidermal atrophy and infiltrate in the upper dermis under the lesion.
Ultrastructural studies revealed that the presence of many normal-appearing membrane-coating granules in the keratinocytes of the old lesion, whereas the organelles were not found in the keratinocytes of the early lesion.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE ELECTRON MICROSCOPY LAMELLAR BODIES
- Lamellar bodies in hyperkeratosis lenticularis perstans.
Tidman MJ, Price ML, MacDonald DM.
J Cutan Pathol. 1987 Aug;14(4):207-11. Abstract quote
An ultrastructural study of lesional skin from 4 subjects with hyperkeratosis lenticularis perstans (Flegel's disease) demonstrated variable numbers of lamellar bodies of normal size and architecture within cells of the stratum granulosum and the upper stratum spinosum.
Their characteristic lamellate inclusions were frequently observed within the intercellular spaces.
These observations suggest that the lamellar bodies are qualitatively normal in Flegel's disease.
- Hyperkeratosis lenticularis perstans (Flegel's disease). A light and electron microscopic study of involved and uninvolved epidermis.
Kanitakis J, Hermier C, Hokayem D, Schmitt D.
Dermatologica. 1987;174(2):96-101. Abstract quote
Lesional as well as uninvolved skin were studied by light and electron microscopy in 1 case of hyperkeratosis lenticularis perstans (Flegel's disease).
The main ultrastructural finding was a morphological alteration of lamellar (Odland) bodies.
A review of the literature on the ultrastructure of Flegel's disease is made, and the significance of the ultrastructural findings is briefly discussed.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
PROGNOSIS CHARACTERIZATION
TREATMENT CHARACTERIZATION GENERAL 5-FLUOROURACIL
- Hyperkeratosis lenticularis perstans (Flegel's disease)--lack of response to treatment with tacalcitol and calcipotriol.
Blaheta HJ, Metzler G, Rassner G, Garbe C.
Department of Dermatology, Eberhard Karl University, Tubingen, Germany.
Dermatology. 2001;202(3):255-8. Abstract quote
Hyperkeratosis lenticularis perstans (HLP) or Flegel's disease is a rare dermatosis characterized by asymptomatic hyperkeratotic papules predominantly located on the lower extremities.
Lesional and non-lesional epidermis samples were studied by light- and electron-microscopic examination. The main ultrastructural finding was the presence of structurally altered Odland bodies/membrane-coating granules.
Different therapeutic options for HLP have been reported, but none of the treatments was shown to be consistently effective.
Here, we report on a patient with Flegel's disease who did respond to topical 5-fluorouracil, whereas topical vitamin D(3) synthetics were ineffective.
J Am Acad Dermatol. 1992 Nov;27(5 Pt 2):812-6. Abstract quote
Lesional and perilesional skin samples from a 57-year-old man who had hyperkeratosis lenticularis perstans (HLP) (Flegel's disease) were studied by light and electron microscopic examination. Keratohyalin granules were diminished at the center of a fully-developed lesion.
In contrast, keratohyalin appeared normal and membrane-coating granules were found in reduced numbers at the edges of the HLP lesion and were easily detected in normal numbers in clinically normal, perilesional skin. The inflammatory infiltrate in the HLP lesion was composed of small lymphocytes, which often displayed nuclei with deep infoldings resembling Sezary cells, and larger histiocytic cells, many of which were in close contact with the lymphocytes. Peripheral blood mononuclear cells did not show an abnormal ultrastructural appearance.
Treatment with topical 5-fluorouracil cream led to the disappearance of the HLP lesions, whereas topical tretinoin was ineffective.PUVA
Br J Dermatol. 2000 Feb;142(2):340-2. Abstract quote
Flegel's disease is an uncommon condition which causes asymptomatic keratotic papules on the limbs. It usually develops in the fourth or fifth decade.
Therapeutic options are limited to emollients, topical 5-fluorouracil and retinoids, but none of these treatments is consistently helpful.
We report a patient with Flegel's disease who responded to psoralen ultraviolet A treatment.ZINC PASTE BANDAGES
- Zinc paste bandages and the treatment of Flegel's disease.
Lindsay E.
Thames Valley University.
Br J Community Nurs. 2005 Mar;10(3):S14-9. Abstract quote
Such is their prevalence in the population that community nurses are very likely to encounter unfamiliar dermatological problems during their practice, many of which will have been self-treated for some time before professional help is sought.
This article reports a case of a rare congenital condition called Flegel's disease, which leads to the presence of small horny papules on the skin, predominantly on the legs. In this case, the papules had deteriorated and were causing wider problems.
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Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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