Perforating Disorder Research Articles

Perforating folliculitis in Down’s syndrome − a rare case report

Perforating folliculitis is a primary perforating disorder, characterized by the phenomenon of transepidermal elimination. It is associated with various disorders such as psoriasis, juvenile acanthosis nigricans, diabetic mellitus, renal failure, cardiac failure, and malignant neoplasia. Other primary perforating dermatoses are Kyrle’s disease, reactive perforating collagenosis, and elastosis perforans serpiginosa. Among the association of perforating dermatoses with genetic diseases like Down’s syndrome, elastosis perforans serpiginosa and very rarely reactive perforating collagenosis have been reported. We report the first case of an 11-year-old boy who is a known case of Down syndrome presented with perforating folliculitis.

Elastosis perforans serpiginosa- A rare case report

Elastosis perforans serpiginosa is a rare primary perforating disorder. In EPS, there is elimination of altered elastic fibres from papillary layer of dermis to epidermis which is described as transelimination disorder. It is characterized as multiple hyperkeratotic umbilicated papule which are arranged in annular or serpiginous pattern with raised edge and central plug. These are more commonly distributed over back, sides of neck and extremities in bilateral symmetrical pattern. It is common in age group of 5 to 20 years with male predominance. Lesions are generally self limiting in nature. Isotretinoin is used in penicillamine induced disease. A 45 year old diabetic female presented with chief complaints of asymptomatic dark coloured raised lesions on face, sides of neck and upper extremities in bilateral symmetrical pattern for last four years. On cutaneous examination multiple asymptomatic hyperpigmented keratotic papular lesions, coalescing to form plaques were arranged in annular, serpiginous and polycyclic pattern over face, sides of neck, chest and forearms distributed in bilaterally symmetrical pattern with central sparing and atrophy. Patient underwent general and systemic examination followed by routine and histopathological examination. Routine investigations were normal except random blood sugar and HBA1C levels were raised. Histopathological examination revealed hyperkeratotic and acanthotic epidermis with transepidermal channels having nuclear debris and elastic fibres. Patient was explained about course and prognosis of disease and was stared on topical and oral retinoids. Later, she was lost follow up.

Perforating disorder secondary to leflunomide and review of the literature of medications associated with perforating disorder

The perforating dermatoses are a group of disorders characterized by transepidermal elimination of a material from the upper dermis. The most widely accepted classification consists of four primary perforating disorders that are defined by the type of material eliminated and the type of epidermal disruption. Pathogenesis of the perforating dermatoses is poorly understood, but some appear to have a genetic component. There are also acquired forms, which have been associated with underlying systemic diseases and the use of certain drugs. In this report, we describe a perforating disorder that occurred secondary to leflunomide therapy. To our knowledge, this is the first case in which this has been reported. We also review the recent literature on medications associated with perforating disorders.

Bullous pemphigoid arising in a patient with acquired perforating dermatosis.

A middle-aged Japanese man who had been on haemodialysis treatment for diabetic nephropathy developed multiple itchy papules and nodules, which were histopathologically diagnosed as acquired perforating dermatosis. Two years later he developed oral lesions and subsequently numerous erosive plaques with necrotic crusts on the trunk and extremities. Histopathology of a papule showed a parakeratotic plug intermingled with basophilic, necrotic debris and collagen bundles, along with penetration of collagen bundles across the epidermis and subepidermal blister. Immunoblotting studies revealed IgG autoantibodies in the patient's serum, which reacted with the C-terminal and the NC16a domains of bullous pemphigoid (BP)180, indicating presence of BP. We searched the literature and found no other cases of an autoimmune blistering disease occurring in association with a perforating disorder. Possible injury to the basement membrane zone induced during the process of transepidermal elimination might be involved in the pathogenesis of the pemphigoid disease.

D-Penicillamine induced elastosis perforans serpiginosa with involvement of glans penis

Elastosis perforans serpiginosa (EPS) is an unusual perforating disorder characterized by extrusion of altered elastic fibers through the epidermis. Clinically, it presents as keratotic papules with a tendency for serpiginous or annular distribution that most commonly involves the sides of the neck and the back. However, involvement of the penis has rarely been reported. We present a case of EPS involving the neck, axilla, and glans penis in a 42-year-old man who had received long-term d -penicillamine treatment for Wilson disease. Skin biopsy revealed perforating channels containing numerous altered elastic fibers, with a characteristic “bramble brush” or “lumpy-bumpy” appearance as demonstrated by an elastin stain. The latter is thought to be pathognomonic for penicillamine-induced degenerative elastosis. These degenerative changes occurring in glans penis have rarely been described in the literature. Prompt recognition of the rare presentation could lead to early discontinuation of the offending drug, to prevent further sequelae.

Effectiveness of topical maxacalcitol for acquired perforating disorder

Effectiveness of topical maxacalcitol for acquired perforating disorder

Buschke–Ollendorff syndrome mimicking pseudoxanthoma elasticum with a perforating disorder

Buschke–Ollendorff syndrome mimicking pseudoxanthoma elasticum with a perforating disorder

FDG PET/CT Findings in Acquired Perforating Dermatosis

Acquired perforating dermatosis (APD) is an uncommon cutaneous perforating disorder. We report a patient on hemodialysis who developed skin eruption and jaundice. He underwent FDG PET/CT under suspicion of biliary malignancies. PET/CT showed no significant abnormal uptake except of multiple FDG-avid nodules in the skin. The eruption he had was histopathologically diagnosed as APD by skin biopsy. His case suggests that APD should be considered as a differential diagnosis when multiple cutaneous FDG accumulations are found in a patient on hemodialysis. To the best of our knowledge, this is the first report showing the FDG PET/CT findings of APD.

Periumbilical perforating pseudoxanthoma elasticum ‐ an acquired perforating disorder

International Journal of DermatologyVolume 51, Issue 4 p. 439-441 Morphology Periumbilical perforating pseudoxanthoma elasticum - an acquired perforating disorder Anil Budania MBBS, Anil Budania MBBS Dermatology, Venereology and LeprologySearch for more papers by this authorDipankar De MD, Dipankar De MD Dermatology, Venereology and LeprologySearch for more papers by this authorUma N. Saikia MD, Uma N. Saikia MD Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaSearch for more papers by this authorAmrinder J. Kanwar MD, FAMS, Amrinder J. Kanwar MD, FAMS Dermatology, Venereology and LeprologySearch for more papers by this authorRahul Mahajan MD, Rahul Mahajan MD Dermatology, Venereology and LeprologySearch for more papers by this authorSavita Yadav MD, Savita Yadav MD Dermatology, Venereology and LeprologySearch for more papers by this author Anil Budania MBBS, Anil Budania MBBS Dermatology, Venereology and LeprologySearch for more papers by this authorDipankar De MD, Dipankar De MD Dermatology, Venereology and LeprologySearch for more papers by this authorUma N. Saikia MD, Uma N. Saikia MD Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaSearch for more papers by this authorAmrinder J. Kanwar MD, FAMS, Amrinder J. Kanwar MD, FAMS Dermatology, Venereology and LeprologySearch for more papers by this authorRahul Mahajan MD, Rahul Mahajan MD Dermatology, Venereology and LeprologySearch for more papers by this authorSavita Yadav MD, Savita Yadav MD Dermatology, Venereology and LeprologySearch for more papers by this author First published: 21 March 2012 https://doi.org/10.1111/j.1365-4632.2011.05276.xCitations: 4 Dipankar De, mdDepartment of Dermatology Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh 160012 India E-mail: dr_dipankar_de@yahoo.in Conflicts of interest: None. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume51, Issue4April 2012Pages 439-441 RelatedInformation

Treatment of Acquired Perforating Dermatosis With Cantharidin

A 65-year-old woman presented with a 6-month history of pruritic, hyperpigmented papules with central, hyperkeratotic plugs on her bilateral lower extremities (Figure 1A). There were no papules on her trunk, head, upper extremities, volar surfaces, or mucous membranes. Her medical history was significant for chronic renal failure treated for the past 4 years with peritoneal dialysis, a 30-year history of insulin-dependent diabetes mellitus, hypercholesterolemia, hypertension, coronary artery bypass, hypothyroidism, and hyperparathyroidism. There was no family history of similar skin lesions. A biopsy specimen from a lesion on the left leg revealed keratotic plugs filled with laminated orthokeratotic and parakeratotic keratin and debris. Beneath the plug, the epidermis was thinned and the dermis showed a neutrophilic infiltrate and foci of fibrosis (Figure 2A). Trichrome stain showed rare focal collagen extruding through the base. The surrounding skin showed features of lichen simplex chronicus. The clinical and histopathologic features supported the diagnosis of an acquired perforating disorder associated with renal disease.

Acquired perforating dermatosis associated with pregnancy

Acquired perforating collagenosis (APC) is a rare condition in which altered collagen and elastin fibres undergo transepidermal elimination. The pathogenesis is unknown but it is usually associated with underlying systemic disease, most commonly diabetes mellitus and renal impairment. We describe a case of an acquired perforating disorder occurring in pregnancy. APC is a diagnosis that can easily be missed, especially in atypical cases such as ours, and clinicians and histopathologists need to be aware of this condition to avoid unnecessary patient morbidity.

Necrotizing infundibular crystalline folliculitis.

We describe a 22-year-old woman with a background of acne who developed multiple folliculocentric facial papules associated with sharply demarcated waxy, keratotic plugs. Multiple skin biopsies showed umbilicated craters that were filled with dispersed bundles of eosinophilic filaments embedded in a pale amorphous matrix forming a plug. The plugs bulged into the upper dermis. Serial sections showed vacuolar and filamentous destruction of the infundibular and adjacent perifollicular epithelium and a close relationship of the crystalline necrosis to follicles. Electron microscopy revealed that the filamentous bundles were tonofilaments. No fresh material was available for polarization and the paraffin sections failed to polarize. The clinical and pathological findings of the lesions in our patient were identical to those reported as a new perforating disorder with urate-like crystals. Our case indicates that the process may represent crystalline folliculocentric necrosis rather than a primary perforating disorder. The nature and basis of the crystals that have a urate-like appearance remain to be determined.

Fibronectin and the extracellular matrix in the perforating disorders of the skin.

Despite detailed microscopic descriptions and clinical observation, little is known regarding the pathogenesis of the perforating disorders of skin, which have traditionally been subdivided into numerous microscopic entities associated with various clinical settings. An increasing body of evidence now suggests that the perforating disorders of skin are akin, and may constitute an expanded single pathologic entity. Each of the classic perforating disorders of skin, including elastosis perforans serpiginosa, perforating folliculitis, reactive perforating collagenosis, Kyrle's disease, and perforating disorder of uremia, have been shown to extrude collagen, elastin, and related extracellular matrix components through the epidermis. Considering a shared pathogenic mechanism among these entities, we explored the possible role of the extracellular matrix, in particular fibronectin, in perforating disorders of skin. Using immunohistochemical and serum determinations of extracellular matrix constituents, including fibronectin, collagen type IV, laminin, and tenascin, we showed consistent serum elevation and/or deposition of fibronectin, in each case, without a commensurate increase in laminin, collagen type IV, and tenascin. We propose that elevated serum and tissue concentrations of fibronectin may be responsible for inciting, in a physiologically aberrant manner, increased epithelial migration and proliferation culminating in perforation.

Surgical Pearl: Surgical planing in the treatment of chondrodermatitis nodularis chronica helicis of the antihelix

Chondrodermatitis nodularis chronica helicis (CNCH) is a common condition involving the auricle in middle-aged and elderly patients. It typically presents as a solitary, tender, erythematous nodule, usually with central crusting and ulceration, on the helical rim in men and the antihelix in women. 1 A biopsy specimen reveals epidermal acanthosis with a central, crested ulcer containing necrotic dermal debris and perichondral thickening. 2 Degenerative changes in the underlying cartilage may also be present. CNCH has been described as both a transepidermal perforating disorder 3 and a necrobiotic granuloma with secondary epithelial changes. 4 The anatomy of the auricle makes it susceptible to environmental and mechanical injury. Relative ischemia as a result of pressure may occur because of the small dermal blood vessels supplying the epidermis, dermis, perichondrium, and cartilage. The lack of a thick cushioning subcutaneous layer in the auricle further predisposes it to pressure-induced ischemia and to trauma. 5 Various treatments, both medical and surgical, have been described for CNCH. Medical approaches include cryotherapy, topical corticosteroids, intralesional corticosteroids, and intralesional injection of collagen. 5-7 Surgical therapies include wedge resection with suturing, curettage and electrocauterization, carbon dioxide laser ablation, and excision of skin followed by scalpel resection or curettage of cartilage. 8-13 The method described by Lawrence 14

Acquired perforating dermatosis in a British dialysis population

A perforating disorder of the skin developing in association with chronic renal failure and often also diabetes, acquired perforating dermatosis (APD), affects up to 10% of patients receiving maintenance haemodialysis in North America. The prevalence of this condition in British dialysis patients has not yet been ascertained. We have undertaken a skin survey of our dialysis population (n = 80) to determine the prevalence and clinical presentation of APD, with subsequent assessment of disease management and outcome. Of 72 patients who participated in the survey, eight were found to have APD, seven of whom were also diabetic. Skin lesions had developed pre-dialysis in two patients, on commencement of dialysis in one, and after 1-3 years on dialysis in the remaining five. Patients typically presented with pruritic dome-shaped papules with central crusts arising on the trunk and extensor limb surfaces. Histological examination of biopsy specimens revealed two types of lesion, typified by either narrow or broad ulcer craters, each showing perforation of both collagen and elastic fibres. Topical/intradermal steroid or topical retinoid were effective therapies in certain of our patients. Clinical clearance was achieved after 3-12 months of treatment in five patients with improvement in the remaining two patients who received treatment. Of the four patients who were alive at 2-year review, three remained clear, while one patient continued to develop new lesions. We report an 11% prevalence of APD in our dialysis population, suggesting the disorder to be as prevalent in patients with chronic renal failure in Britain as in North America. An association of the disorder with long-standing diabetes was confirmed.

Acquired Perforating Dermatosis: Comparison of an Acquired Perforating Dermatosis and Perforation as an Incidental Histologic Finding

A 66-year-old Japanese woman with a rare acquired perforating disorder, usually called adult-type reactive perforating collagenosis, is reported. The patient had poorly controlled diabetes mellitus with retinopathy under oral diabetic medication. She was found to have multiple papules and umbilicated nodules on the trunk and four extremities when she was admitted and examined for the origin of jaundice and severe pruritus of sudden onset. In the biopsy specimen, collagen fibers were observed to be eliminated from the dermis through epidermal tunnel-like perforations. No elastic fibers were eliminated, and serial sectioning of the specimen could not prove follicular perforation. Adenocarcinoma of the biliary duct was found to be the cause of the jaundice with pruritus. Although such cases are usually classified as acquired reactive perforating collagenosis of adult onset, proposed reclassification for acquired perforating disorders is discussed. Another case which also showed perforation and transepithelial elimination of both collagen and elastic fibers as an incidental histologic finding is described. Such elimination seems to be a not uncommon step in the formation of pruriginous eruptions. Therefore, these cases should be differentiated from acquired-type characteristic perforating disorders.

Acquired perforating disease in oil field workers

Eight cases of an acquired perforating disease are reported in oil field workers who were exposed to drilling fluid. Analysis of the drilling fluid showed it to contain many components, including calcium chloride. Histopathologic study of patient lesions demonstrated transepidermal elimination of calcium. An in vivo mouse model was developed to study this phenomenon, which may represent a calcium-mediated perforating disorder.