Histologically, the eruption shows parakeratosis, orthohyperkeratosis, atrophy of the malpighian layer, and a lichenoid histiocytic and lymphocytic dermal infiltrate. Apr 14, 2017 keratosis lichenoides chronica klc is a rare mucocutaneous eruption, with just greater than 70 cases reported in the literature. Parapsoriasis and pityriasis lichenoides fitzpatricks dermatology. The disease was originally described by kaposi as lichen ruber verrucosus et reticularis but was named after nekam who reported a typical case in 1938. It is a progressive disease characterized by the linear and reticulate appearance of. Yuval ramot, liran horev, abraham zlotogorski created date. Keratosis lichenoides chronica is characterized by violaceous, papular and nodular lesions typically arranged in a linear and reticulate pattern, most marked on the hands and feet, and accompanied by a seborrhoeic dermatitislike eruption of the face. The mucous membranes, genitalia, nails, palms, and soles may also be affected. This cutaneous eruption appears to be previously unreported, and we propose the term keratosis lichenoides chronica.
A case of pediatric keratosis lichenoides chronica with. Keratosis lichenoides chronica successfully treated with isotretinoin. Keratosis lichenoides chronica successfully treated with. Pdf keratosis lichenoides chronica in a child antonio. Sep 01, 2003 keratosis lichenoides chronica is a rare dermatosis that typically presents in adolescene or early adulthood with symmetric lichenoid keratotic papules, forming warty plaques.
Adult keratosis lichenoides chronica klc, also known as nekams disease, is a very rare chronic condition mostly seen in adults. Interhospital dermatology conference 2016 case 19 a 50 yearold. Keratosis lichenoides chronica is characterised by brownishpurple, thick scaly papules or small nodules on the trunk and extremities. Keratosis lichenoides chronica klc is a rare disorder, with fewer than 50 cases reported in the world literature. The clinical, histological and therapeutic information on 71 patients with klc retrieved through a pubmed search plus one. Wikiproject medicine rated redirectclass this redirect is within the scope of wikiproject medicine. Nekams disease aruna c, ramamurthy d, neelima t, bandaru.
Background keratosis lichenoides chronica klc is a rare chronic keratinisation disorder. Keratosis lichenoides chronica mimicking verrucous secondary. Parapsoriasis and pityriasis lichenoides fitzpatricks. Keratosis lichenoides chronica klc, also known as nekams disease, is a rare and chronic disorder of keratinization of unknown etiology 1,2. In 1938, nekam called the disease porokeratosis striate lichen, after seeing the acrosyringeal hyperkeratosis in the case published by kaposi, despite the absence of coronoid lamella. Keratosis lichenoides chronica has not always been a clearly defined entity in the literature. Two cases of pediatric keratosis lichenoides chronica with. Pityriasis lichenoides et varioliformis acuta after influenza vaccine. Histopathology shows findings between atrophy and acanthosis. The clinical picture, histopathology and the criteria for the diagnosis of this rare dermatosis are discussed. It is clinically characterized by the presence of hyperkeratotic papules spread through the trunk and extremities adopting a linear andor reticular pattern. Esophageal pityriasis lichenoides chronica american journal of. Results of direct immunofluorescence of perilesional skin and mucosa and indirect immunofluorescence were normal. Keratosis lichenoides chronica klc is a rare chronic hyperkeratotic disorder that typically affects patients aged 20 to 50 years.
Lichen nitidus, lichen sclerosus et atrophicus, benign lichenoid keratoses, lichen aureus, pityriasis lichenoides, and keratosis lichenoides chronica. Keratosis lichenoides chronica jama dermatology jama. Margolis, cooper, and johnson proposed the new term keratosis lichenoides chronica for an unusual previously unreported cutaneous eruption. This pdf is available for free download from a site hosted. We describe a case of keratosis lichenoides chronica that improved with systemic retinoids. Jun 01, 2002 summary keratosis lichenoides chronica klc is a rare chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhea. The mean single dose, the mean number of irradiations and the mean total dose were 70. It is not known what causes the reaction, but triggers include minor trauma such as friction, drugs, dermatitis, and sun exposure.
Klc presents mainly in adults, between 20 and 40 years of age with a slight predominance in males. Although the clinical variability for all the conditions on the differential is notable, our patient had a history of consuming raw shiitake mushrooms, denied taking. A 20yearold african man presented with clinical and histopathological features of keratosis lichenoides chronica. The widely used term keratosis lichenoides chronica was introduced. In 1899, juliusberg delineated the chronic form and named it plc. Feb 01, 1998 keratosis lichenoides chronica klc is a rare disorder, with only 50 cases reported in the world literature. Response of keratosis lichenoides chronica to efalizumab therapy. Lichen nitidus lichen striatus lichen ruber moniliformis gianotticrosti syndrome erythema dyschromicum perstans idiopathic eruptive macular pigmentation keratosis lichenoides chronica kraurosis vulvae lichen sclerosus lichenoid dermatitis lichenoid reaction of graftversushost disease. It is chronic and progressive usually affecting individuals aged 2040 years. Case of keratosis lichenoides chronica with atypical.
Keratosis lichenoides chronica is a rare keratinization disease with characteristic findings. In addition, patients usually have a seborrheic dermatitislike eruption of the scalp and face and may have involvement of the mucosa, nails, and vocal cords. Multiple eruptive lichenoid keratoses in sunexposed sites are also described. Queratosis liquenoide cronica tratada con calcipotriol topico.
Original article efficacy of uva1 phototherapy in 230. Keratosis lichenoides chronica is a rare dermatosis characterized by violaceous papular and nodular lesions, often arranged in a linear and reticulate pattern on the dorsal hands and feet, extremities, and buttocks. Patients with hyperpigmented mf were identified by a file search of all patients with mf who had attended a cutaneous. On further questioning, the patient indicated that he had consumed raw shiitake mushrooms 3 days before the onset of the rash. Differences of prevalence between genders and races have been reported. Keratosis lichenoides chronica is an uncommon cutaneous disorder of unknown ethiopathogenesis. The etiology of klc is unknown, but it may be associated with internal diseases such as hypothyroidism, glomerulonephritis and lymphoproliferative disorders.
Their case is remarkably similar to one presented by the new york university, school of. Please visit the project page for details or ask questions at wikipedia talk. It presents with erythematous or violaceous keratotic papules on the lower extremities that form linear andor reticulated plaques, and a facial eruption that resembles seborrheic dermatitis. Oct 26, 2006 since 1972 the term keratosis lichenoides chronica, suggested by margolis, has been generally accepted for this condition, except in france, where it is named lichenoid tri keratosis 1. It has been a subject of controversy whether keratosis lichenoides chronica klc is a distinctive inflammatory disease of the skin or whether it represents a manifestation of another wellknown disease, such as lichen planus, lupus erythematosus, or lichen simplex chronicus. Jan 22, 2016 a 30 year old otherwise healthy woman presented with a 15 year history of widespread pruritic rash and slow clinical progression. Keratosis lichenoids chronica klc was first described by kaposi in 1895, who named the disease as lichen ruber acuminatus morbilliform disease. The etiology of klc is unknown, but it may be associated with internal diseases such as hypothyroidism, glomerulonephritis an. Queratosis liquenoide cronica poroqueratosis liquenoide. These lesions are most commonly found in a symmetrical distribution on the trunk and extremities and display a unique linear andor reticulate pattern.
No treatment is incomplete without any side effects. Keratosis lichenoides chronica, lichenoid dermatitis, criteria for diagnosis, histopathology, lichen ruber, lupus erythematosus, prurigo simplex, moritz kaposi search for similar articles you may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search. Keratosis lichenoides chronica or nekams disease is a rare mucocutaneous dermatosis characterized by keratinization. Keratosis lichenoides chronica mimicking verrucous. Keratosis lichenoides chronica klc, also known as nekam. Keratosis lichenoides chronica with tiny keratotic papules that tend to be. Characteristically, klc presents as violaceous, keratotic, lichenoid papules with a linear or reticulated arrangement and symmetrical distribution on the extremities and the trunk. A clinical and histolopathological study of pityriasis lichenoides. Pistoni f1, peroni a1, colato c2, schena d1, girolomoni g1. The main reason to bathe in asss milk for help because it can be more about please search of natural oils.
Nekam s disease with clinical manifestation simulating. Erythema ab igne with histological features of keratosis. The papules are generally arranged in a linear or reticular pattern and are symmetrically distributed. Klc is commonly considered either as a distinct entity or as a rare manifestation of lichen planus. Introductionkeratosis lichenoides chronica klc is a rare chronic cutaneous disease characterized by violaceous, papular and nodular keratotic lesions typically arranged in a linear and reticular pattern. Keratosis lichenoides chronica klc is a rare and chronic disorder of keratinization of unknown etiology. Keratosis lichenoides chronica klc is a rare chronic disorder of keratinization, characterized by the progressive development of erythematosquamous plaques and violaceous lichenoid hyperkeratotic papules, nodules and ridges. Media in category keratosis lichenoides chronica the following 3 files are in this category, out of 3 total. Keywords nekams disease keratosis lichenoides chronica dariers disease introduction ekams disease is a rare disorder, with only 50 cases reported in the world literature until 1998. Keratosis lichenoides chronica jama dermatology jama network. What is lichenoid keratosis lichenoid keratosis is usually a small, solitary, inflamed macule or thin pigmented plaque. Keratosis lichenoides chronica, lichenoid dermatitis, criteria for diagnosis, histopathology, lichen ruber, lupus erythematosus, prurigo simplex, moritz kaposi search for similar articles you may search for similar articles that contain these same keywords or you. Their colour varies from an initial reddish brown to a greyish purplebrown as the lesion resolves several weeks or months later lichenoid keratosis is also known as benign lichenoid keratosis. Although the clinical variability for all the conditions on the differential is notable, our patient had a history of.
Keratosis lichenoides chronica klc is a rare chronic disease characterized by violaceous, papular and nodular lesions typically arranged in a linear and reticulate pattern. Keratosis lichenoides chronica no treatment is incomplete without any side effects. Pityriasis lichenoides is an uncommon inflammatory skin condition of unknown cause that ranges from mild chronic form to a more severe acut. Keratosis lichenoides chronica klc is a rare chronic progressive dermatosis of unknown aetiology characterized by lichenoid hyperkeratotic papules typically arranged in a linear or reticulate pattern on the extremities or on the trunk, warty erythematosquamous plaques and seborrhealike dermatitis on the face. Keratosis lichenoides chronica klc is a rare chronic progressive dermatosis of unknown aetiology characterized by lichenoid hyperkeratotic papules typically arranged in a linear or reticulate pattern on the extremities or on the trunk, warty erythematosquamous plaques and seborrhealike dermatitis on.
The etiology of klc remains unknown and the eruptions tend to be refractory to treatment. Keratosis lichenoides chronica keratosis treatment. Lichenoid keratosis, also known as, keratosis lichenoides chronica is a condition where, usually, a solitary brown lesion turns red and becomes itchy. Pityriasis lichenoides pl represents a group of inflammatory cutaneous. Keratosis lichenoides chronica is a rare, chronic, progressive, dermatosis that is much more common in adults than in pediatric patients. It is a progressive disease characterized by the linear and reticulate appearance of erythematoustoviolaceous keratotic and lichenoid papules over the trunk and limbs and a seborrheiclike dermatitis over the face 2,3. A facial eruption, suggestive of seborrheic eczema, is frequently associated. Keratosis lichenoides chronica nekam disease, lichen ruber. Keratosis lichenoides chronica klc is a rare dermatosis with a chronic progressive course. Keratosis lichenoides chronica is a rare, acquired dermatosis of young adults. Pdf case of keratosis lichenoides chronica with atypical. Keratosis lichenoides chronica in childhood, clinical.
Pediatriconset klc is rare with different clinical characteristics. Similar good results were shown in scleroderma with. These lesions usually appear in an area that is exposed to the sun. Pdf pityriasis lichenoides is an uncommon, benign skin disorder with two major variants. Out of almost 60 published cases of klc, only two report its occurrence in siblings. They are usually asymptomatic but can be itchy in 20% of cases. Papularpurpuric gloveandsock syndrome is a rare, infectious disease, of viral etiology. Keratosis lichenoides chronica klc is a rare dermatological condition characterized by keratotic papules arranged in a parallel linear or reticular pattern and facial lesions resembling seborrheic dermatitis or rosacea. Around 70 cases have been reported in the literature.
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