Acquired Reactive Perforating Collagenosis Occurring in Association With Nonred Ink Tattoo.

ABSTRACT: Perforating dermatosis is a group of skin conditions in which there is transdermal elimination of collagen, elastic fibers, or other dermal connective tissue. Perforating dermatosis can be genetic or acquired, known as acquired perforating dermatosis (APD). When collagen is the primary extruded material in acquired cases, the disease is designated as acquired reactive perforating collagenosis (RPC). We report a case of acquired RPC occurring in a new tattoo. One week after having a new tattoo placed on the left forearm, a 38-year-old gentleman presented to the emergency room with pruritic, crusted plaques and erosions in the regions of red and green inks of the tattoo. Histopathologic examination of the biopsy revealed an ulceration with transepidermal elimination of collagen bundles accompanied by basophilic debris, scattered dermal tattoo pigment, and a superficial to deep perivascular lymphohistiocytic infiltrate with scattered neutrophils and eosinophils. There have been 2 reported cases of tattoo-associated RPC, both in association with red tattoo ink. This present case is the first reported APD to occur in association with nonred tattoo ink. This case reaffirms the conclusions of others in recognizing APD as a potential tattoo-associated complication.

Acquired reactive perforating collagenosis: A case report and review of the literature.

INTRODUCTION: Acquired reactive perforating collagenosis (ARPC) is a rare skin disorder, which is associated with various internal diseases and even malignant neoplasms. A comprehensive knowledge of the concomitant diseases in ARPC patients is helpful to decrease the misdiagnosis. Although the treatment of ARPC is challenging, systemic assessment of existing regimens is not available. PATIENT CONCERNS: A 50-year-old woman was admitted to the hospital due to cutaneous pruritus and papules all over the body. DIAGNOSIS: Physical examination showed various sized papules on the lower limbs, buttocks, back, chest, and upper arms with keratotic plugs in the center. Histopathology showed typical collagenous fiber perforation. The diagnosis of ARPC was made according to histopathology, onset age and typical skin lesions. Type 2 diabetes mellitus (T2DM), chronic renal failure (CRF), and hypothyroidism simultaneously presented in this patient. INTERVENTIONS: This patient was initially treated with topical corticosteroids and oral antihistamines for the skin lesion and pruritus. Medications for glucose control and recovery of renal and thyroid functions were also applied. On the second admission, the combined therapy of topical retinoic acid, Chinese medicinal herb-Qingpeng ointment, and Zinc oxide ointment was added. OUTCOMES: Papules and pruritus were improved significantly after the second hospitalization. CONCLUSION: We present a case of ARPC associated with T2DM, CRF, and hypothyroidism, which has rarely been described. There is no standardized treatment for ARPC. Co-administration of two or more agents for dermatologic interventions and treatment for associated diseases may help to improve skin symptoms.

Roles of the procollagen C-propeptides in health and disease.

The procollagen C-propeptides of the fibrillar collagens play key roles in the intracellular assembly of procollagen molecules from their constituent polypeptides chains, and in the extracellular assembly of collagen molecules into fibrils. Here we review recent advances in understanding the molecular mechanisms controlling C-propeptide trimerization which have revealed the importance of inter-chain disulphide bonding and a small number of charged amino acids in the stability and specificity of different types of chain association. We also show how the crystal structure of the complex between the C-propeptide trimer of procollagen III and the active fragment of procollagen C-proteinase enhancer-1 leads to a detailed model for accelerating release of the C-propeptides from procollagen by bone morphogenetic protein-1 and related proteinases. We then discuss the effects of disease-related missense mutations in the C-propeptides in relation to the sites of these mutations in the three-dimensional structure. While in general there is a good correlation between disease severity and structure-based predictions, there are notable exceptions, suggesting new interactions involving the C-propeptides yet to be characterized. Mutations affecting proteolytic release of the C-propeptides from procollagen are discussed in detail. Finally, the roles of recently discovered interaction partners for the C-propeptides are considered during fibril assembly and cross-linking.

Reactive perforating collagenosis: An important differential diagnosis in hemodialysis patients.

This is a case report of a 68-year-old hypertensive, diabetic woman who was on regular thrice weekly hemodialysis (HD). She presented with gradually worsening left lower limb pain and swelling. Clinical examination revealed significant edema over the left calf and ankle joint with significant calf tenderness. Extensive workup including magnetic resonance imaging of the lower limb and venous Doppler failed to show any significant abnormality. In view of developing papular lesions over the area, skin biopsy was performed, which finally confirmed reactive perforating collagenosis. This diagnosis ensured reassurance to the patient with halting of further extensive and probably expensive testing. Simple conservative management and symptomatic relief improved the pain over the next few days. This case report highlights the importance of keeping a wide differential for calf tenderness in diabetic HD patients and projects reactive perforating collagenosis as one of the important but frequently missed entities in such a scenario.

Dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid in a patient with acquired reactive perforating collagenosis.

Bullous pemphigoid (BP) is a common autoimmune blistering disorder with unknown etiology. Recently, increasing numbers of BP cases which developed under the medication with dipeptidyl peptidase-4 inhibitors (DPP4i), widely used antihyperglycemic drugs, have been reported in published works. Here, we report a case of DPP4i (teneligliptin)-associated BP that developed in a 70-year-old Japanese man. Interestingly, the patient had acquired reactive perforating collagenosis (ARPC), which is also known to be associated with the onset of BP. In the present case, clinical, histopathological and immunological findings suggested that DPP4i rather than ARPC was associated with the onset of BP.

Morphologic changes of cerebral veins in hypertensive rats: venous collagenosis is associated with hypertension.

BACKGROUND: The aims of this study were to determine whether arterial hypertension could affect the venous system of brain and to find out the consequent pathologic changes of cerebral veins. METHODS: Thirty male Sprague-Dawley rats were divided into 2 groups: a sham-clipped group and a stroke-prone renovascular hypertensive rat group. A 2-kidney 2-clip rat model was used to induce renovascular hypertension in the hypertensive group. Systolic blood pressure was measured by tail cuff once each week. Susceptibility-weighted imaging (SWI) was performed at 12, 16, and 20 weeks after surgery. All the rats were sacrificed after the SWI examination at 20 weeks after surgery. The brains were extracted and embedded in paraffin for histologic examination. Masson trichrome staining was performed to identify venous collagenosis. RESULTS: The sham group demonstrated less prominence of cerebral veins compared with hypertensive groups (P < .01); the hypertensive group showed significant venous collagenosis in cerebral venous walls compared with the sham group (P < .01). CONCLUSIONS: The increased visibility of cerebral veins on SWI as a sign of venous hypertension and the thickened cerebral venous walls (venous collagenosis), which may play a role in cerebral ischemia and/or infarction, are both consequences of long-term hypertension in hypertensive rats.

Low-energy fractures without low T-scores characteristic of osteoporosis: a possible bone matrix disorder.

BACKGROUND: Osteoporotic fractures commonly occur after low-energy trauma in postmenopausal women with reduced bone quantity documented by low bone mineral density (BMD). Low-energy fractures, however, have also been reported to occur in premenopausal women with normal or near-normal BMD, suggesting the existence of a bone quality abnormality. METHODS: Bone quality and quantity were evaluated in a cross-sectional study of three groups of premenopausal white females: (1) twenty-five subjects with low-energy fracture(s) and BMD in the normal range (t-scores > -2.0), (2) eighteen subjects with low-energy fracture(s) and BMD in the osteoporotic range (t-scores ≤ -2.5), and (3) fourteen healthy volunteers (controls). Bone quality was assessed with use of Fourier transform infrared spectroscopy and histomorphometry in iliac crest bone samples obtained from all subjects; bone quantity was assessed by dual x-ray absorptiometry and histomorphometry. RESULTS: The collagen crosslinking ratio in the non-low-BMD subjects with fractures was 13% greater than the ratio in the low-BMD subjects with fractures and 14% greater than the ratio in the controls (p < 0.001 for both). Cancellous bone volume was 29% greater (p < 0.01) and trabecular separation was 31% less (p < 0.01) in the non-low-BMD subjects with fractures than in the low-BMD subjects with fractures; the values in the non-low-BMD subjects did not differ from those in the controls. Bone turnover did not differ among the groups, and osteomalacia was not present in any subject. Thus, the non-low-BMD subjects with fractures maintained bone quantity, but the collagen crosslinking ratio, a parameter of bone quality, was abnormal. In contrast, the low-BMD subjects with fractures did not have this collagen crosslinking abnormality but did have abnormal bone quantity. CONCLUSIONS: This study highlights a collagen crosslinking abnormality in patients with low-energy fractures and nonosteoporotic t-scores. Reports have indicated that altered collagen crosslinking is associated with subnormal fracture resistance. A finding of nonosteoporotic bone mass in a patient with low-energy fractures would justify assessment of bone material quality, which currently requires a bone biopsy. Further studies are needed to search for possible noninvasive tests to diagnose abnormal crosslinking. Since no specific therapies for abnormal collagen crosslinking are currently available, studies are also needed to explore novel therapeutic modalities to reverse the underlying collagen crosslinking abnormality. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Allopurinol in the treatment of acquired reactive perforating collagenosis.

Acquired reactive perforating collagenosis is a perforating dermatosis usually associated with different systemic diseases, mainly diabetes mellitus and/or chronic renal insufficiency. Different therapies have been tried but treatment is not standardized yet and remains a challenge. In the last few years, allopurinol has been reported as a good therapeutic option for acquired reactive perforating collagenosis. We describe the case of a 73-year-old man affected by acquired reactive perforating collagenosis associated with diabetes type 1 and chronic renal failure with secondary hyperparathyroidism. The patient was successfully treated with allopurinol 100mg once/day p.o..

[Acquired reactive perforating collagenosis following herpes zoster as isotopic response?]. / Erworbene reaktive perforierende Kollagenose nach Herpes zoster als isotopische Antwort?

Reactive perforating collagenosis is a disease whose pathogenesis is still not fully understood. Histological findings are degenerated collagen bundles which are arranged in vertical direction penetrating the epidermis into a dome-shaped crater. Usually diabetes mellitus and renal failure can be found among patients with reactive perforating collagenosis. To date, there have been five cases described where the eruption of reactive perforating collagenosis followed herpes zoster infection. This could be a form of Wolf's isotopic response, a term that is used for dermatoses which arise after the healing of a preexisting dermatosis. We report the sixth case of a herpes zoster-associated reactive perforating collagenosis and discuss the current literature.

The spectrum of lung involvement in collagen vascular-like diseases following allogeneic hematopoietic stem cell transplantation: report of 6 cases and review of the literature.

Multisystem autoimmune diseases occurring after allogeneic hematopoietic stem cell transplantation are infrequent, late-onset manifestations that resemble well-defined collagen vascular disorders. Because the lung is frequently involved in the course of connective tissue disorders, we focused on lung manifestations occurring in autoimmune diseases following allogeneic stem cell transplantation. In the present series, we report 6 patients with systemic lupus erythematous, mixed connective tissue disease, Sjögren syndrome, polymyositis, and ANCA-positive vasculitis who presented with a spectrum of pulmonary manifestations affecting the airways, lung parenchyma, and probably respiratory muscles. We identified 3 different histopathologic patterns of interstitial pneumonia consistent with the underlying autoimmune disorder: lymphocytic interstitial pneumonia and non-specific interstitial pneumonia in 2 patients with Sjögren syndrome and diffuse alveolar damage in 1 patient with ANCA-positive vasculitis. These lung manifestations had poor prognoses. Further studies are needed to determine the optimal therapy for these complications.

Colagenosis perforante reactiva adquirida: Caso clínico / Acquired reactive perforating collagenosis: Report of two cases

Acquired reactive perforating collagenosis is a perforating dermatosis characterized by transepidermal elimination of collagen. It is frequently associated to diabetes mellitus and chronic renal insuffciency, but it is also related to other systemic diseases. The lesions tend to resolve once the underlying condition is treated. We report two patients with the condition. A 65 year-old diabetic female on hemodialysis consulted for multiple itching cutaneous ulcers lasting one year. On physical examination, hyperpigmented papules and nodules were observed. A 65 year-old female with chronic renal failure in hemodialysis consulted for itching lesions in hands, forearms and arms. On physical examination, hyperpigmented lesions with ulcers, erosions and crusts were observed. In both cases, the pathological study of the lesions disclosed a reactive perforating collagenosis.

Acquired reactive perforating collagenosis: current status.

Acquired reactive perforating collagenosis is a unique perforating dermatosis, characterized clinically by umbilicated hyperkeratotic papules or nodules and histologically by a focal hyperkeratosis in direct contact with transepidermal perforating dermal collagen. Several inflammatory or malignant systemic diseases may coexist with acquired reactive perforating collagenosis. The possible biochemical or immunological mechanisms of the systemic diseases, potentially responsible for the development and appearance of acquired reactive perforating collagenosis, are still under investigation. Several topical treatments, ultraviolet B phototherapy and allopurinol p.o. administration may be effective.

Acquired reactive perforating collagenosis associated with papillary thyroid carcinoma: a paraneoplastic phenomenon?

Acquired reactive perforating collagenosis (ARPC) is an uncommon dermatosis characterized by transepidermal elimination of altered collagen. It is commonly seen in patients with diabetes mellitus and/or chronic renal insufficiency. Rarely, it has been reported in association with malignancy and other conditions. We report a 30-year-old woman with insulin-dependent diabetes mellitus who presented with multiple, discrete, violaceous, hyperkeratotic papules on the extensor aspects of both legs, characteristic of ARPC. Four months later, metastatic papillary thyroid cancer was diagnosed. This case further supports the possibility that ARPC may represent a paraneoplastic phenomenon.