We describe effective therapy of acquired perforating dermatosis with narrowband uvb in 5 patients. Acquired perforating dermatosis apd is the most common perforating dermatosis, known to often occur in patients with chronic kidney disease or diabetes. Acquired perforating dermatosis in a patient on peritoneal. Both conditions fall within the group of perforating dermatoses. Acquired perforating dermatosis may manifest with several polymorphous skin lesions, such as hyperkeratotic, often umbilicated, millimetric or larger papules or nodules. Acquired reactive perforating dermatosis arpd is a rare disease with characteristic clinical findings which is classified as a primary perforating. It is also rarely associated with liver disease, malignant disease, hypothyroidism and hiv. Acquired reactive perforating dermatosis is a variant of prurigo nodularis article in acta dermatovenereologica 972 june 2016 with 18 reads how we measure reads. The code is valid for the year 2020 for the submission of hipaacovered transactions. Apd affects adults and is associated with systemic diseases, mainly diabetes mellitus and renal failure. Of 22 patients with acquired perforating dermatoses, 72. All lesions disappeared completely after 10 to 15 exposures without.
Diagnostic criteria include central hyperkeratotic or cup. Special attention was paid, if shown in our specimens, to epidermal perforation of collagen and the composition of the covering debris. Mar 25, 2018 the exact cause of acquired perforating dermatosis development is unknown. Acquired perforating dermatosis apd is a debilitating and itchy skin disease.
Acquired perforating dermatosis in patients with chronic. The signs and symptoms of acquired perforating dermatosis include. Acquired reactive perforating dermatosis vashishtha r. Acquired perforating dermatosis classically presents with severely pruritic follicular hyperkeratotic papules, sometimes umbilicated, on the hairbearing limbs of adults, as in our patient.
Acquired perforating dermatosis apd is an uncommon disease characterized by lesions exhibiting transepidermal elimination of collagen or elastic fibers. Treatment of acquired perforating dermatosis with narrowband. Acquired perforating dermatosis associated with endstage. Disseminated acquired reactive perforating collagenosis. Erythematous or nonerythematous lesions can also be found on follicular or nonfollicular regions depending on the apd type. Pdf acquired perforating dermatosis apd is a perforating disease characterized by transepidermal elimination of dermal material 1,2. It is characterised by the presence of hyperkeratotic papules and. Acquired perforating dermatosis associated with endstage diabetic kidney failure in a hemodialysis patient aleksandra wieczorek,1 lukasz matusiak,2 jacek c szepietowski2 acquired perforating dermatosis apd is an uncommon skin disorder seen in majority among patients with chronic kidney disease and also in those with diabetes mellitus. Printed with permission from martins j, rivera m, carrillogijon r, teruel jl, ortuno j. Acquired perforating dermatosis in a peritoneal dialysis patient j martins 1, m rivera 1, r carrillogijon 2, jl teruel 1 and j ortuno 1 1department of nephrology, hospital ramon y cajal, madrid, spain. Epidemiology the prevalence and incidence of this rare dermatosis are unknown 1, 7. The 2 distinct forms of are an inherited form that manifests in childhood and an acquired sporadic form that occurs in adulthood. Patients with renal disease or diabetes mellitus often have an acquired perforating disease of the skin develop that is characterized by hyperkeratotic papule.
Acquired perforating dermatosis diabetes care american. Response to treatment lipy gupta 1, archana singal 1, deepika pandhi 1, sonal sharma 2 1 department of dermatology, university college of medical sciences and gtb hospital, university of delhi, delhi110 092, india 2 department of pathology, university college of medical sciences and gtb. Treatment of acquired perforating dermatosis with cantharidin. Acquired perforating dermatosis associated with primary. Acquired perforating dermatosis in a patient with chronic renal failure.
The term acquired perforating dermatosis was first used by rapini et al. Acquired perforating dermatosis apd is an uncommon cutaneous perforating disorder. Reactive perforating collagenosis pathology dermnet nz. Acquired perforating dermatosis is a rare skin disorder which develops in individuals undergoing dialysis. Criteria included all parameters previously described by weigelt et al. Perforating dermatoses are a heterogeneous group that is characterized by a papulonodular rash with transepidermal elimination of dermal. It is not well established as to how wide spread it is in patients on peritoneal dialysis pd and its implications in this population have not been well studied. Acquired reactive perforating dermatosis is characterized by umbilicated erythematous papules and plaques with firmly adherent crusts. The inherited form is a very rare condition with less than 50 cases reported in literature worldwide.
It has been described in diabetic andor renal patients. It is typified by presence of numerous papules on the body, especially, on the head, neck and trunk region. The principal diagnosis feature is the presence of a central keratotic core which is overlying a focus of epidermal perforation. Acquired perforating dermatosis associated with metastatic. Acquired perforating dermatosis is clinically and histopathologically similar to perforating folliculitis, also associated with chronic kidney failure, with or without hemodialysis or peritoneal dialysis, andor diabetes mellitus, but not identical to kyrle disease 1462. Case report giant variant of acquired perforating dermatosis in a renal dialysis patient laurenmetterle,bs,cynthiam. Examination revealed multiple discrete, erythematous, crateriform nodules and plaques with central plugs of an escharlike black crust. While used for both the inherited and acquired forms, the name reactive perforating dermatosis is also used for the more common acquired type. Giant variant of acquired perforating dermatosis in a. Several acquired types of perforating dermatoses have been identified, eg kyrles disease, elastosis perforans serpiginosa and reactive perforating collagenosis, the clinical boundary between these can be blurred and as such the term acquired perforating dermatosis is sometimes used to encompass this group of conditions.
This article is within the scope of wikiproject medicine, which recommends that medicinerelated articles follow the manual of style for medicinerelated articles and that biomedical information in any article use highquality medical sources. Treatment of acquired reactive perforating dermatosis a. The follicular epithelium is disrupted with dermal collagen entering the perforation. The exact prevalence and incidence of acquired perforating dermatosis are unknown. The exact cause of acquired perforating dermatosis development is unknown. B in the lumbar region, erythematous papules, some following a linear path, suggesting koebner phenomenon arrows. Many treatments have been proposed for acquired perforating dermatosis apd, with varying success. In the acquired form in adults, it is frequently associated with diseases such as diabetes and chronic renal failure. The purpose of this study was to clarify the clinical and histopathological features of apd, and evaluate. Acquired perforating dermatosis in a peritoneal dialysis. Its diagnosis is based on biopsy and the treatment is not very clear. Acquired perforating dermatosis includes perforating dermatoses arising in adults previously named. Reactive perforating collagenosis rpc is a very rare skin disorder that can manifest during early childhood or adulthood. This reflects that there is overlap with the other acquired perforating disorders of perforating folliculitis and kyrle disease.
The prevalence, incidence and the exact pathogenesis of this rare dermatosis are unknown 1. Skin biopsy confirmed the clinical impression of acquired perforating dermatosis, a perforating disorder strongly associated with diabetes mellitus and chronic renal disease. Giant variant of acquired perforating dermatosis in a renal. Acquired perforating dermatosis in patients with chronic renal failure and diabetes mellitus acquired perforating dermatosis apd is a skin disorder occurring in the patients with chronic renal failure crf, diabetes mellitus dm or both. There are rare reports of apd in association with liver disease or thyroid disease. Acquired perforating dermatosis associated with metastatic colon cancer hana jeon ab 1, g peter sarantopoulos md 2, nima m gharavi md phd 3, emma taylor md 3, melvin w chiu md mph 3 dermatology online journal 17 11. Acquired reactive perforating collagenosis and chronic hbv infection 2 introduction reactive perforating collagenosis rpc is a rare cutaneous disorder of unknown etiology 1.
Acquired reactive perforating dermatosis arpd is a rare disease with characteristic clinical findings which is classified as a primary perforating dermatosis. Acquired perforating dermatosis in renal and diabetic patients. In patients receiving dialysis, acquired perforating dermatosis occurs in about 10% of patients. Reactive perforating collagenosis is a rare skin disorder characterized by the transepidermal elimination of altered collagen through the epidermis. Apd has been reported to occur in association with various diseases, but is most commonly associated with dialysisdependent chronic renal failure crf or diabetes. Figure 2 skin biopsy specimen of acquired perforating dermatosis shows a dilated follicle with keratinous and necrotic debris. Acquired reactive perforating dermatosis wagner 20 jddg. Acquired perforating dermatosis in a patient on peritoneal dialysis. The pathogenesis is unknown, although scratching appears to play a critical role.
Acquired perforating dermatosis apd, which encompasses kyrle disease, acquired rpc, acquired pf, and acquired eps and is often associated with underlying chronic renal failure or diabetes mellitus picture 4ab and table 1. Acquired perforating dermatosis consists of papulonodular skin lesions characterized by transepidermal elimination of some components of the dermis. Oct 27, 2009 acquired perforating dermatosis classically presents with severely pruritic follicular hyperkeratotic papules, sometimes umbilicated, on the hairbearing limbs of adults, as in our patient. In general, treatment is unsatisfactory and definitive resolution of existing lesions is difficult to achieve. Here we present a case of apd that developed in a patient on pd. Dermatological disease in patients with ckd american. Reactive perforating collagenosis rpc is a very rare skin disorder that can manifest during early childhood or. A clinicopathologic study of thirty cases of acquired perforating dermatosis in korea seo wan kim, mi sun kim, june hyunkyung lee, sookja son, kui young park1, kapsok li1, seong jun seo1, tae young han department of dermatology, eulji general hospital, eulji university.
Acquired perforating dermatosis apd, eps see separate chapter and pediatric rpc are sufficiently different to be considered independent clinical entities. Acquired perforating dermatosis apd is strongly associated with pruritus, chronic renal failure, andor diabetes mellitus. Acquired perforating dermatosis apd is an uncommon skin eruption of unclear etiology that most often is associated with diabetes mellitus or chronic renal insufficiency. Acquired perforating dermatosis is clinically and histopathologically similar to perforating folliculitis, also associated with chronic kidney failure, with or without hemodialysis or peritoneal dialysis, andor diabetes mellitus, but not identical to kyrle disease. Some experts believe that it is due to the formation of abnormal mineral deposits resulting from a metabolic imbalance. In a retrospective study of patients diagnosed with apd between 2002 and 2014, included 33 patients. Acquired perforating dermatosis may manifest with several polymorphous skin lesions, such as hyperkeratotic, often umbilicated, millimetric or larger papules or. In addition, clinical and microscopic features of epidermal perforation identical to those in acquired reactive perforating dermatosis ardp were observed. Acquired reactive perforating collagenosis acquired elastosis perforans serpiginosa kyrlesdisease perforating folliculitis may represent different stages of development lesions have different morphologies in the same patient. Garciamalinis aj, del valle sanchez e, sanchezsalas mp, et al.
A clinicopathologic study of thirty cases of acquired perforating dermatosis in korea article pdf available in annals of dermatology 262. Wikiproject medicine dermatology rated stubclass, midimportance this article is within the scope of wikiproject medicine, which recommends that medicinerelated articles follow the manual of style. Acquired perforating dermatosis in patients with chronic renal failure. The classical morphology and histopathology of the lesion and demonstration of collagen fibres in the crater contributed to the diagnosis of rpc in the present case. Acquired perforating dermatosis apd is a perforating disease characterized by transepidermal elimination of dermal material 1,2. Acquired perforating dermatosis in a patient with chronic renal failure 11 figure 3. We report a case of typical and aggressive apd in a 70yearold man. Acquired reactive perforating dermatosis is a variant of. Acquired perforating dermatosis usually occurs during the fifth decade of life.
Acquired perforating dermatosis is a chronic disease, usually associated with diabetes mellitus or renal failure or both. Mar 07, 2017 reactive perforating collagenosis is a rare skin disorder characterized by the transepidermal elimination of altered collagen through the epidermis. Acquired perforating dermatosis is difficult to treat. Acquired reactive perforating dermatosis is a variant of prurigo nodularis. Indian j dermatol venereol leprol serial online 1993 cited 2020 mar 18. Acquired reactive perforating collagenosis is more common and occurs in approximately 10% of patients receiving maintenance haemodialysis. Coexistent acquired perforating collagenosis and lepromatous leprosy with erythema nodosum leprosum. Division of dermatopathology, department of pathology and laboratory medicine, david. Oct 26, 2017 examination revealed multiple discrete, erythematous, crateriform nodules and plaques with central plugs of an escharlike black crust. Typical apd cases can be diagnosed at a glance, and clinicians should be aware of the characteristic eruptions of apd. Perforating dermatoses are a group of skin diseases characterized by transepidermal elimination of dermal material. Acquired perforating dermatosis apd is the most common. Reactive perforating collagenosis clinical presentation.
Acquired perforating dermatosis perforating dermatoses. What are the signs and symptoms of acquired perforating dermatosis. The differential diagnosis includes prurigo nodularis, folliculitis, arthropod bites, multiple keratoacanthomas, psoriasis and lichen planus. Intensive light microscopical analysis of biopsied lesions was performed. Perforating dermatoses primary care dermatology society uk. Statistical comparison with previous results for prurigo nodularis pn showed that up is clearly a subtype of pn. Acquired perforating dermatosis in a peritoneal dialysis patient. Clinically the acquired perforating dermatoses are characterised by. A case of arpd in association with chronic renal failure and dialysis is reported here. The dose was started at 400 mjcm 2 and increased to a maximum of 1500 mjcm 2. A clinicopathologic study of thirty cases of acquired. Apr 02, 2007 acquired perforating dermatosis consists of papulonodular skin lesions characterized by transepidermal elimination of some components of the dermis.
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