Primary Cutaneous Amyloidosis Research Articles

Pathology of extranodal marginal zone lymphoma at different anatomic sites

: Extranodal marginal zone lymphomas arise within acquired lymphoid tissue at a variety of extranodal sites. They have characteristic architectural and morphological features that recapitulate those seen in constitutive lymphoid tissue in extranodal locations that is best exemplified by the mucosa (gut) associated lymphoid tissue (MALT) present as Peyer’s patches in the terminal ileum. The morphology of these MALT lymphomas is constant across the various extranodal sites in which they are encountered although there is some variation between cases in the detailed cellular morphology with variable proportions of cells with centrocyte-like, monocytoid and plasmacytoid/plasmacytic appearances. Architecturally there is inter-case variation in the degree and type of follicular colonisation. The appearance of lymphoepithelial lesions varies between sites. There is variable association with light chain deposition disease and nodular amyloidosis which appear to be more frequently associated with a pulmonary origin. The immunophenotype is uniform across sites with a rather non-specific B-cell antigen expression with aberrant CD43 expression in a proportion of cases. There is infrequent expression of CD5 and this appears more frequent in lymphomas arising in the ocular/ocular adnexal region.

Treatment of lichen amyloidosis with fractional CO2 laser and topical steroid: a preliminary study of 10 cases.

Treatment of lichen amyloidosis with fractional CO2 laser and topical steroid: a preliminary study of 10 cases.

Genetic testing identifies the potential risk of multiple endocrine neoplasia in a Vietnamese family

Multiple endocrine neoplasia type 2A (MEN2A) is a rare syndrome characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, hyperparathyroidism and sometimes cutaneous lichen amyloidosis. This syndrome is caused by a germline activation mutation in the rearranged during transfection (RET) proto-oncogene transmitted by an autosomal dominant inheritance. In this study, we reported a rare case of a 44-year-old man from Vietnam with medullary thyroid carcinoma and pheochromocytoma as the symptom of MEN2A. Genetic testing indicated a nucleotide substitution located in exon 11 of the RET proto-oncogene (c.1900T>C, p.C634R), which was reported as a known pathogenic mutation of MEN2A. Further genetic tests on the other family members found the same mutation in his daughter (currently 14 years-old) and his son (currently 8 years-old). Although these 2 children do not yet have any manifestations of MEN2A, this data emphasizes their high risks of this disease. Therefore, this case draws attention to the importance of genetic counselling in C634R carriers, as well as rigorous follow-up appointments to reduce incidence and mortality since the mutation is classified as a high-risk group within the medullary thyroid carcinoma guidelines.

A study on efficacy of high fluence Q-switched neodymium doped yttrium aluminium garnet laser in macular amyloidosis

<p class="abstract"><strong>Background: </strong>Macular amyloidosis (MA) typically presents as small, dusky-brown or greyish pigmented macules, treatment of which remains challenging with topical and systemic therapies, however Q-switched neodymium-doped yttrium aluminium garnet (Nd:YAG) laser has proven to be an effective treatment modality to reduce hyperpigmentation. The aim of the study is to study the efficacy of Nd:YAG laser in the management of MA.</p><p class="abstract"><strong>Methods: </strong>30 female patients were treated with Q-switched Nd:YAG laser at 1064 nm with a spot size of 3 mm at a fluence of 6-7 J/cm<sup>2</sup>, frequency of 2 Hz, for 350-500 pulses. Total number of pulses decreased with each treatment session as the intensity of the hyperpigmentation decreased. The procedure was repeated at one-month intervals for a total of 6 treatment sessions.</p><p class="abstract"><strong>Results: </strong>After completion of treatment sessions with Nd-YAG laser 36.66% patients showed >50% improvement in the pigmentation. 66.6% patients were satisfied with the laser treatment. 33.3% patients showed <25% improvement and unsatisfied with the treatment.</p><p class="abstract"><strong>Conclusion: </strong>MA remains an enigma and a source of concern for the suffering patients and physicians. Q-switched Nd-YAG laser 1064 nm has shown positive response in the reduction of pigmentation in MA.</p>

A clinical study of primary cutaneous localized amyloidosis

<p class="abstract"><strong>Background:</strong> Amyloidosis refers to abnormal extracellular tissue deposition of one of the biochemically unrelated proteins that share certain characteristic staining properties such as apple green birefringence of congo red stained preparations under polarized light. Amyloid deposition may occur in many organs of the body (systemic amyloidosis) or may be restricted to one tissue site (localized amyloidosis).</p><p class="abstract"><strong>Methods:</strong> Observational study was conducted in the Department of Dermatology, Narayana Medical College and Hospital, Nellore. In this study of 70 patients a complete history and clinical data was taken including details of presenting complaints, age, site, duration, course of the disease was recorded. Special importance was given to the friction history using different scrubs, family history and photo exposure.<strong></strong></p><p class="abstract"><strong>Results:</strong> Out of 70 patients of cutaneous amyloidosis 55 patients (78.6%) were of macular amyloidosis. Male:female ratio was 1:2.3. Majority of the patients were housewives (42.8%) and 28.5% of agriculturists. Majority of the patients (40) with cutaneous amyloidosis had disease duration between 2-5 years. In 70 patients of cutaneous amyloidosis 64.3% were symptomatic. 92% patients give history of using scrub. 50% of patients had history of photo exposure. Positive family history was seen in 7 patients (10%) and most common site involved was extensor aspect of arm.</p><p class="abstract"><strong>Conclusions:</strong> From our study it can be concluded that cutaneous amyloidosis is more common among females, most patients are symptomatic, majority have history of using scrub and photo exposure and most common site involved is extensor aspect of arm.</p><p class="abstract"> </p>

PROGRESSIVE PIGMENTATION OF WHOLE BODY MIMICKING HEREDITARY RETICULATE PIGMENTATION IN A YOUNG GIRL: A RARE PRESENTATION OF AMYLOID CUTIS DYSCHROMICA

Primary cutaneous amyloidosis is a chronic, progressive disorder of skin, because of the amyloid deposition in the skin with no systemic involvement. Amyloid cutis dyschromica (ACD) is considered a rare variant of primary cutaneous amyloidosis with around 50 cases reported so far. Most cases are reported from Asia, majority having the family history. Autosomal recessive inheritance in GPNMB encoding glycoprotein non metastatic gene B has been reported in many cases with few case reports of semidominat inheritance. It is usually asymptomatic condition as opposed to other types of primary cutaneous amyloidosis like macular or lichenoid variant which are associated with moderate to severe pruritus and photosensitvity. Chief complaint in majority of cases is cosmetic concern only. In view of the very few cases reported from India, we hereby report the case of a 28 years old female having similar dyspigmentation in one sibling.

A CASE REPORT OF PRIMARY AMYLOIDOMA OF THE LUNG

SESSION TITLE: Fellows Lung Pathology Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Primary amyloidoma of the lung is rare and is usually found incidentally on chest radiographs in asymptomatic, elderly patients. Amyloid nodules may be solitary or much more commonly multiple. We present a case of primary amyloidoma of the lung. CASE PRESENTATION: A 79-year-old man, former smoker, with a history of COPD, DM, HTN, CKD, GERD, and adenocarcinoma of the colon presented with a few days of history of cough, dyspnea, and lethargy. Four days prior he was treated as an outpatient for pneumonia with levofloxacin and metronidazole. CT chest showed mild emphysema, RUL lung mass, and multiple bilateral non-calcified lung nodules. CT guided biopsy of RUL mass was consistent with amyloidoma. TTE did not show evidence of cardiac involvement. Immunoglobulin levels were unremarkable. SPEP and UPEP revealed no evidence of monoclonal protein. This was thought to be a localized process rather than diffuse parenchymal amyloidosis and decision was made to manage conservatively with surveillance rather than surgical excision. DISCUSSION: The term amyloid was first used by Rudolf Virchow in 1854.1 Amyloidosis is a heterogeneous group of diseases characterized by the deposition of misfolded insoluble proteins in extracellular tissue. Amyloidosis can be primary or secondary, and the deposition of amyloid proteins can be localized or systemic. Localized pulmonary amyloidosis was first described by Lesser in 1877. ¬¬2 It is divided into three main anatomical areas; tracheobronchial, parenchymal, and mediastinal.3 Tracheobronchial is the most common form of localized pulmonary amyloidosis.4 Patients can be asymptomatic or present with cough, dyspnea, and hemoptysis. A chest radiograph is nondiagnostic. CT findings ranges from a solitary nodule, multiple nodular lesions to more diffuse disease, with no pathognomonic features. Solitary amyloidoma of the lung may be confused with primary lung carcinoma. Multiple lesions may be difficult to differentiate from metastatic disease. Demonstration of apple-green birefringence with Congo red stain under polarized light is the gold standard for diagnosis. Isolated pulmonary amyloidoma is diagnosed by excluding other organ involvement. The treatment of pulmonary amyloidosis ranges from observation to bronchoscopic or surgical resection depending on the severity of the disease. Localized amyloidosis is usually treated with local interventions. Excisional therapy is the standard approach for tracheobronchial amyloidosis. Nd: YAG laser can be used to treat some nodular forms of tracheobronchial amyloidosis. Radiotherapy can be a viable option for the treatment of solitary pulmonary amyloidoma. CONCLUSIONS: Solitary pulmonary nodular amyloidosis is a rare diagnosis. Exclusion of systemic involvement is crucial in the management of these patients. The treatment ranges from observation to bronchoscopic or surgical resection based on the severity of the disease. Reference #1: 1. Saleiro S, Hespanhol V, Magalhães A. Endobronchial amyloidosis. J Bronchol. 2008;15:95–99. Reference #2: 2. Gillmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax. 1999;54:444–51. Reference #3: 3. Lachmann HJ, Hawkins PN. Amyloidosis and the lung. Chron Respir Dis. 2006;3:203–14. 4. Sugihara E, Dambara T, Okamoto M, Sonobe S, Koga H, Inui A, et al. Clinical features of 10 patients with pulmonary amyloidosis. J Bronchol. 2006;13:191–3. DISCLOSURES: No relevant relationships by Moises Cossio, source=Web Response No relevant relationships by Domingo Franco-Palacios, source=Web Response No relevant relationships by Kushagra Gupta, source=Web Response No relevant relationships by Shahzad Hussain, source=Web Response No relevant relationships by Katie Kennedy, source=Web Response No relevant relationships by Toribiong Uchel, source=Web Response

Fractional Carbon Dioxide Laser is Effective in Amelioration of Pruritus in Primary Cutaneous Amyloidosis: A Clinical and Biochemical Study.

Primary cutaneous amyloidosis (PCA) is a pruritic disease characterized by amyloid deposition in the skin. Interleukin-31 (IL-31) is a pruritus-mediating cytokine. Fractional carbon dioxide (CO2 ) laser has shown efficacy in the treatment of PCA regarding the clinical appearance, histological pattern, and pruritus. The aim of this study is to assess the effect of fractional CO2 laser on pruritus associated with PCA, and analyze whether this effect is related to IL-31 and IL-31 receptor (R) expression. The study included 24 patients with PCA and 24 healthy controls. Each patient received four fractional CO2 laser sessions, 4 weeks apart, using the superficial ablative mode. Skin biopsies were taken from patients before and after treatment, as well as controls, for assessment of IL-31 and IL-31R by real-time polymerase chain reaction. Treatment resulted in significant improvement of all clinical parameters, including pruritus (P < 0.001). Patients before treatment had significantly higher IL-31 and IL-31R than controls (P = 0.000 for both). In addition, there was a statistically significant decrease in IL-31 and IL-31R after treatment than their values before treatment (P = 0.000 for both). This study confirms the therapeutic efficacy of fractional CO2 laser in treatment of PCA. Reduction of IL-31 and its receptor seems to be one of the involved mechanisms; however, its relation to improvement of pruritus is still not clear. Lasers Surg. Med. © 2020 Wiley Periodicals LLC.

Multiple endocrine neoplasia type 2A with cutaneous lichen amyloidosis

A 33-year-old woman presented with a decade-long history of a painless, gradually increasing neck swelling not associated with any compressive symptoms. She also complained of intermittent unprovoked episodes of headache,...

伴双手麻木的结节性皮肤淀粉样变一例

伴双手麻木的结节性皮肤淀粉样变一例

Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosedante mortemby pulmonologists

BackgroundAnte mortem diagnosis of amyloidosis of the respiratory system is rare. Few data are available regarding clinical presentation, precursor proteins, diagnostic procedures, comorbidities, complications, and outcome. We assessed clinical features of a series of patients with amyloidosis of the respiratory system in two Japanese centres.MethodsMedical records of 16 patients with amyloidosis of the respiratory system were retrospectively analysed. Amyloid was diagnosed by polarisation microscopy using Congo red-stained tissue specimens and classified immunohistochemically.ResultsMedian patient age was 71 years, and median follow-up period was 5 years. Immunoglobulin light-chain (AL)-λ amyloidosis was found in eight and AL-κ in five patients. Two patients harboured wild-type transthyretin and one harboured serum amyloid A-derived amyloid. Five different forms of amyloidosis of the respiratory system were observed: nodular pulmonary amyloidosis (seven patients), diffuse alveolar-septal amyloidosis (five), mediastinal lymph node amyloidosis (three), tracheobronchial amyloidosis (one), and pleural amyloidosis (one). One patient had diffuse alveolar-septal amyloidosis and mediastinal lymph node amyloidosis. Three of five patients with diffuse alveolar-septal amyloidosis were diagnosed by transbronchial lung biopsy as having concurrent diffuse alveolar haemorrhage or pneumocystis pneumonia. Two of three patients with mediastinal lymph node amyloidosis were diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration.ConclusionsNot only nodular pulmonary amyloidosis, diffuse alveolar-septal amyloidosis, and tracheobronchial amyloidosis but also mediastinal lymph node amyloidosis and pleural amyloidosis should be considered in the differential diagnosis of amyloidosis of the respiratory system. Useful diagnostic methods include transbronchial lung biopsy for diffuse alveolar-septal amyloidosis and endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal lymph node amyloidosis.

A clinical study of cutaneous manifestations in diabetes mellitus

Background: Diabetes mellitus (DM) is the most common endocrine disorder1.At least 30% of diabetics have some cutaneous involvement during the course of their disease 1. Type II patients do develop more frequent cutaneous infections, whereas type I patients develop more autoimmune- type cutaneous lesions 2,3. Objective: To evaluate the prevalence and pattern of skin manifestations among diabetic patients. Methods: 100 patients of diabetes mellitus with skin manifestations who consecutively attended DVL outpatient department and willing to participate in the study were enrolled in this study. Skin examination was done and findings entered to computer database for analysis. Results: Among 100 patients, there were 56 males and 44 females the youngest patient being 19 years old and eldest was 77 years old. Cutaneous infections were the most commonly observed dermatoses (51%), followed by dermatoses more commonly associated with diabetes mellitus (36%), non specific dermatoses (23%), neuropathic and ischemic diabetic skin disease (5%), due to microangiopathy (4%), metabolic disorders (3%) and cutaneous reactions to therapy (3%).Among cutaneous infections, fungal infections (38%) are the most common dermatoses followed by bacterial infections (11%) and viral infections (2%). Tinea corporis was the most common fungal infection and furunculosis (3%) was most common bacterial infection. Among the common dermatoses associated with diabetes mellitus (36%) majority presented with pruritus secondary to xerosis (12 patients, 12%) followed by 11 cases (11%) of psoriasis, 4 cases of vitiligo, 2 patients each (2%) of acrochordons, lichen planus and macular amyloidosis, 1 patient each (1%) of acanthosis nigricans, alopecia universalis and kyrles disease. Among patients with non specific dermatoses, eczema was most common followed by pemphigus, acne vulgaris and hidradenitis suppurativa. Conclusion: Infections were the most common cutaneous manifestations in diabetes, followed by dermatoses most commonly associated with diabetes. Cutaneous manifestations are more common in patients who have overall poor glycemic control. Cutaneous manifestations can heighten the suspicion of a physician regarding the diagnosis of diabetes and help to prevent systemic derangements by early institution of appropriate treatment.

Localized cutaneous nodular amyloidosis in a patient with primary Sjögren syndrome: A revisit of autoimmunity‐modifying clinicopathology through an updated literature review

Localized cutaneous nodular amyloidosis in a patient with primary Sjögren syndrome: A revisit of autoimmunity‐modifying clinicopathology through an updated literature review

Nodular localized cutaneous amyloidosis in an adult patient with Sjögren’s syndrome

Nodular localized cutaneous amyloidosis in an adult patient with Sjögren’s syndrome

Comparison of Immunostaining with Hematoxylin-Eosin and Special Stains in the Diagnosis of Cutaneous Macular Amyloidosis

BackgroundAlthough macular amyloidosis is a relatively rare disease, it is a common cutaneous disease in Asia and the Middle East. On hematoxylin and eosin (H&E) stained slides, early lesions could easily be missed without the use of special stains and/or immunohistochemistry.MethodsWe enrolled 42 patients with the clinical impression of macular amyloidosis who had undergone two 4-mm punch biopsies from 2015 to 2016 at a dermatology clinic affiliated to Shiraz University. Besides, 14 cases with a clinical diagnosis other than macular amyloidosis were selected as the negative control group. Congo red, crystal violet, and immunohistochemical (IHC) staining of CK5 and high molecular weight keratin (HMWK) were performed for each specimen.ResultsH&E slides showed globular depositions in 15 (35.7%) out of 42 patients. None of the patients showed apple-green birefringence with Congo red stain. Evaluation of crystal violet stained sections revealed purplish violet amyloid deposits in 15 (35.7%) patients. IHC study showed expression of CK5 in 52.4% and HMWK in 50% of the patients, which was not a significant difference (p = 0.715). The findings of both IHC markers had a significant difference with H&E stains (p = 0.039) and crystal violet (p = 0.008). Additionally, we found that two punch biopsies from two sites in the involved area did not have a significant preference over one punch biopsy. All of the cases in the control group were negative for amyloid deposition in H&E, special stains, and IHC stained slides as expected.ConclusionsIHC evaluation using CK5 and HMWK might be a useful tool for diagnosing macular amyloidosis.

P47 A case of parenchymal nodular amyloidosis with Sjögren's syndrome mimicking a bronchogenic tumour

Abstract Background Amyloidosis is a disorder characterised by the misfolding of extracellular proteins. Patients with Sjögren’s syndrome frequently have a range of lung manifestations. Parenchymal nodular amyloidosis has been reported in association with Sjögren’s syndrome although occurrences are rare. Methods We present a 77 year old woman with a longstanding history of exertional breathlessness on a background of mild asthma since childhood, a cardiac pacemaker for atrial fibrillation and sick sinus syndrome. She has never smoked tobacco and has a good performance status. She presented with an episode of breathlessness and cough requiring hospital admission, receiving treatment for a lower respiratory tract infection. Results Chest X-rays had suggested two benign nodules in the right upper lobe which seemed to have progressed over the last ten years. Amongst her inpatient investigation, she underwent a CT-pulmonary angiogram which did not demonstrate a pulmonary embolism but found three lesions in the right upper lobe. The largest of the lesions measured 2.4cm in size and was suspicious for malignancy. A PET-CT scan showed two of these to be calcified lesions in the right upper lobe, and the other an irregular soft tissue density nodule with low-grade FDG uptake, suspicious of a primary bronchogenic tumour. Lung function showed mild airflow obstruction. Bronchoscopy and endobronchial ultrasound-guided biopsy did not yield a diagnosis. As surgery could be potentially curative, she underwent a right upper lobectomy and systematic nodal sampling. The histology on the lesions has shown abundant eosinophilic concretions with peripherally prominent foreign body giant cell reaction. This was congophilic and showed green birefringence. Further staining suggested AL amyloid. Histological appearances and the clinical picture seemed to represent parenchymal nodular amyloidosis. Further investigation revealed ANA was 1:640 speckled pattern and anti-Ro antibody positive. On further questioning, she reported sicca symptoms affecting eyes and mouth, fatigue and generalised arthralgia particularly affecting knees, ankles, wrist and shoulder. Schirmer’s test suggested severely dry eyes. A diagnosis of primary Sjögren’s was made and hydroxychloroquine was started. Conclusion Parenchymal nodular amyloidosis has been associated with Sjögren's syndrome but is a rare manifestation. However, there are other chronic inflammatory or haematological condition has been described. Parenchymal nodular amyloidosis is associated with multiple nodules, rarely a single nodule. Isolated pulmonary amyloidosis has four distinct patterns of presentation: diffuse alveolar septal, lymphatic, trachea-bronchial and nodule-parenchymal (typically found incidentally on routine imaging). These nodules remain stable over long periods of time. There is no curative treatment of nodular amyloid, but successful treatment has been documented with resection or laser therapy. In our patient, the presence of AL amyloidosis prompted us to investigate for an underlying cause and clinical features and immunological testing suggested primary Sjögren's and should be considered in the pulmonary manifestation of this condition. Disclosures K. Halai None. O. Savanovic-Abel None. T. Howell None.

Efficacy of topical dimethyl sulfoxide (DMSO) 50% solution vs tretinoin 0.5% cream in treatment of patients with primary macular amyloidosis: A split-side single-blinded randomized clinical trial.

Primary localized cutaneous amyloidosis (PLCA) is a recalcitrant sporadic dermatological condition and most treatments have failed so far. We studied the efficacy of topical dimethyl sulfoxide (DMSO) 50% solution in comparison with tretinoin 0.5% cream in treatment of macular amyloidosis. In this split-side within-person single-blinded randomized clinical trial, 18 patients with bilateral macular amyloidosis received topical DMSO 50% solution and tretinoin 0.5% cream either on their right or the left side. The colorimetry, pruritus scoring, and photography were done. A significant pigmentation decline per each follow-up was observed in DMSO group compared to the tretinoin group (tretinoin: -1.31 vs DMSO: -7.34; difference in slopes: -6.03 [95% confidence interval: -12.06 to -0.01], PInteraction = .049). An insignificant diminution trend in pigmentation was observed for both treatments (Ptretinoin = .672, PDMSO = .092). Also, both treatments relived itchiness, but DMSO completely dispatched itchiness from the first follow-up (P = .003 for tretinoin and <.0001 for DMSO). In conclusion, our results showed DMSO and tretinoin cream have the positive effect on the both pigmentation and itchiness in PLCA. DMSO may be more beneficial than tretinoin, since DMSO was significantly better in reducing itchiness. More investigations are warranted to provide sufficient evidence.

MULTIPLE ENDOCRINE NEOPLASIA IN PRACTICE OF PRIMARY CARE AND FAMILY PHYISICIANS

Multiple endocrine neoplasia is characterized with a predisposition to tumors involving two or more endocrine glands. The four main forms of the disease are inherited as an autosomal dominant syndrome or may occur sporadically. In addition to these four forms, six other syndromes are associated with the presence of multiple endocrine and other neoplasms of the organs: hyperparathyroidism − jaw tumors, Carney complex, von Hippel−Lindau disease, neurofibromatosis type 1, Cowden syndrome and McCune − Albright syndrome. The diagnosis of multiple endocrine neoplasia syndrome can be established in humans by one of the three available criteria: clinical features, family history, genetic analysis. Mutation analysis during these syndromes is useful in clinical practice to confirm the clinical diagnosis; identifying family members who tolerate the mutation and need to be screened, and identifying family members who do not tolerate the mutation. Syndrome of multiple endocrine neoplasia (Wermer syndrome) is characterized by the presence of a triad of tumors, including tumors of the parathyroid glands, pheochromocytoma and tumors of the parathyroid gland. It occurs less frequently in combination with Hirschsprung's disease, caused by the absence of vegetative ganglion cells in the intestine terminal parts, that leads to colonic enlargement, severe constipation and obstruction. This syndrome may be associated with cutaneous lichen amyloidosis, the clinical manifestations of which are pruritus and lichenoid lesions, usually located in the upper back. A clinical case of MEN2 syndrome in a 52−year−old patient is presented. It is noted that for such patients, in addition to timely syndromic rather than component diagnosis of this endocrine multipathology, the spread of neoplastic process in medullary thyroid cancer to its capsule and surrounding tissues, as well as the presence of metastases in peripheral lymph nodes are important. As a rule, such patients cannot be timely cured. Key words: multiple endocrine neoplasia, endocrine tumors, genetic analysis, family history.

Clinical resolution of generalized lichen amyloidosis with dupilumab: a new alternative therapy

Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis characterized by deposition of amyloid protein in the skin without visceral involvement. Although it is usually limited to localized areas of the body, it rarely can present in a generalized fashion and is severely pruritic. The limited form is treated with skin directed therapies such as topical or intralesional corticosteroids or topical tacrolimus but the generalized type is more difficult to treat. We present a patient with generalized primary cutaneous lichen amyloidosis successfully treated with dupilumab.

A case report and differential diagnosis of pruritic pretibial skin lesions

Pretibial pruritic papular dermatitis (PPPD) is a clinical entity first described in 2006. The etiology is uncertain; however, gentle chronic rubbing is likely to be the reason for the skin reaction. Pretibial pruritic lesions may reflect many different systemic diseases and dermatoses. We present a 61-year-old patient with a 2-year history of pruritic pretibial xerosis, keratotic erythematous to brownish papules, and excoriations. Differential diagnosis excluded papular mucinosis, myxoedema, stasis dermatitis, lichen simplex chronicus, prurigo nodularis, lichen amyloidosis, and lichen planus. Regarding clinical-histological correlation, we confirmed a diagnosis of PPPD.