Mucin Deposition Research Articles

Papulonodular mucinosis: a systematic review on clinicopathologic characteristics, course and treatment options.

Papulonodular mucinosis (PNM) is a rare cutaneous mucinosis recognized as a rare variant of lupus erythematosus. There are no large-scale cohort studies characterizing its clinicopathological features or evaluating the effectiveness of various treatment options. This study aimed to review the literature on PNMs to identify its clinical and histological features and treatment outcomes. The search includes studies from the PubMed, Embase, and Cochrane Library databases up to the March 1, 2024. A total of 37 studies, encompassing 44 patients, were included in the review. Of these, 20 were males and 24 females, with a mean age at diagnosis of 34.9 ± 1.92years. PNM lesions are characterized by generalized papules, plaques, and nodules, and histopathologically, they show abundant mucin deposition throughout the dermis. The temporal relationship between PNM and SLE is variable. There is no evidence that SLE patients with PNM are more prone to organ involvement or increased disease activity. A combination of medical history, clinical features, and histopathological findings is essential for diagnosis; however, misdiagnosis is possible until lupus erythematosus (LE) becomes evident. Hydroxychloroquine and systemic glucocorticoids are the current mainstays of treatment for PNM, with most patients achieving partial or complete remission.

7854 Ablation Aberration: A Case of Thyroid Dermopathy Exacerbation in a Filipino Male Following Radioactive Iodine Ablation and Total Thyroidectomy Improved by Weekly Intradermal Long-Acting Glucocorticoid Injection

Abstract Disclosure: K.T. Lim: None. O.C. Dampil: None. Ablation Aberration: A Case of Thyroid Dermopathy Exacerbation in a Filipino Male Following Radioactive Iodine Ablation and Total Thyroidectomy Improved by Weekly Intradermal Long-Acting Glucocorticoid Injection Introduction: Thyroid dermopathy (TD) represents an unusual extrathyroidal presentation of Graves' disease (GD), impacting about 0.5–4% of patients. RAI treatment for hyperthyroidism is known to progress Graves' ophthalmopathy. RAI-induced TD exacerbation, however, is rare in literature. Here, we present a case of worsening TD and ophthalmopathy in a Filipino patient with GD who underwent definitive therapy for persistent hyperthyroidism. Clinical Case: A 44-year-old man with GD had bilateral lower extremity indurated, mildly erythematous plaques for 4 years. Hyperthyroidism persisted despite methimazole compliance, so he underwent RAI ablation two years earlier, total thyroidectomy a year later, and has been on levothyroxine since. Despite definitive treatment, his extrathyroidal symptoms worsened. A punch biopsy of the skin lesions revealed upper and mid-lower dermis mucin deposition, with occasional spindle-shaped and stellate fibroblasts, and mast cells. These histopathologic findings were consistent with pretibial myxedema. We assessed his thyroid function and found he was euthyroid with normal TSH (3.1 μIU/mL; reference range 0.27-4.20) and an elevated TRAb (35.73 IU/L; reference range <1.75). Physical examination revealed thickened skin, cobblestone-like plaques, and non-pitting edema on the pretibial, ankle, and pedal areas. A 3.5-cm-diameter, circumscribed, orange-peel-textured, indurated plaque covered his left ankle. Fluocinolone acetonide 0.025% w/w ointment was initiated. After 3 weeks, his cutaneous symptoms improved by 35%, albeit his left ankle circumscribed plaque improved marginally. Triamcinolone acetonide 40 mg/mL was injected into this and other nodular lesions at 4mg (0.1 mL) per 1-cm-diameter circular region. Each dose was equally dispersed by multipoint intradermal injections using a 1 mL syringe with a 0.5”x 25G needle. A week after intralesional triamcinolone injection and continuous topical steroid application on the non-nodular lesions, a 70% improvement in cutaneous manifestations was observed (from baseline) with the plaques becoming thinner and softer. After three weekly intradermal steroid injections, the circumscribed plaque had flattened (with residual hyperpigmentation), and the other plaques and nonpitting edema dissipated. Conclusion: Our experience with this patient highlights a paradoxical TD exacerbation following RAI ablation and definitive surgery for persistent hyperthyroidism in GD. It also reveals that weekly administration of multiple subcutaneous injections of a long-acting glucocorticoid alleviates lesions where topical ointment does not. Presentation: 6/1/2024

Chondroid Syringoma of scalp- A Case Misdiagnosed Clinicoradiologically as Dermoid Cyst

Chondroid syringoma (CS) is a rare tumor of the apocrine or eccrine glands. CS of the scalp is rare, and its clinical manifestations are similar to those of dermoid cyst, which is a common misdiagnosis for this disease. A 26-year-old woman presented with a 2-month history of an asymptomatic subcutaneous mass on the scalp. The lesions increased progressively over time. Dermatological examination showed that there was a subcutaneous mass, ranging from 2x2 cm in diameter, with a clear boundary. The surface of the skin was smooth without ulceration or scaling. Histopathologic examination was consistent with the diagnosis of CS. CS is a rare tumor of the apocrine or eccrine glands. It usually presents as a wellcircumscribed and single subcutaneous masses. Histopathology showed the tumor was located in the dermis, with nests, sheets, and cords of basal-like cells, mucin deposition, and chondroid structures. We herein report a case of CS located in the scalp. CS of the scalp is rare, and its clinical manifestations are similar to those of dermoid cyst, for which it is commonly misdiagnosed.

Clinically amyopathic dermatomyositis: Clinical, laboratory, and histopathological features.

Despite the advancements in the categorization of clinically amyopathic dermatomyositis (CADM), the classification and diagnosis of its subtypes are still challenging. The aim of our study was to describe the clinicopathological features of CADM and assess the differences between amyopathic dermatomyositis (ADM) and hypomyopathic dermatomyositis (HDM). This retrospective study included 43 patients with CADM diagnosed at our institution from 2016 to 2020. Patients were subclassed into ADM (n = 30) and HDM (n = 13) groups to assess their clinicopathological differences. All included patients had characteristic cutaneous manifestations of dermatomyositis; 67.4% had myositis-associated auto-antibodies, including ANA (32.6%), RNP (14.0%), anti-Ro52 (9.3%), anti-p155/140 (7.0%), rheumatoid factor (7.0%), anti-NXP-2 (4.7%), anti-MDA5 (2.3%), and anti-Jo-1 (2.3%) antibodies. One patient had associated interstitial lung disease, and another patient had oral squamous cell carcinoma. The histopathological findings included mucin deposition (69.8%), telangiectasia (65.1%), lymphocytic infiltrate (48.8%), vacuolar interface dermatitis (46.5%), and epidermal atrophy (14.0%). Compared to patients with HDM, ADM patients were significantly less likely to have epidermal atrophy, 3.3% versus 38.5% (p = 0.006), and more likely to have mucin deposition, 80.0% versus 46.2% (p = 0.028). We described the clinicopathological features of CADM and highlighted the distinctions between ADM and HDM dermatopathologic findings. This information may prove helpful in diagnosing ambiguous lesions.

A Case of a 50-Year-Old Female with Dermatomyositis Secondary to a Paraneoplasm (Infiltrating Ductal Carcinoma, Grade 2)

Introduction: Dermatomyositis is an autoimmune disorder with an incidence of 5 to 10 per 1 million per year. 10 to 20% of patients with adult-onset Dermatomyositis have an underlying malignancy. There are currently no specific cutaneous markers for Paraneoplastic Dermatomyositis. This case highlights a patient with uncommon features of paraneoplasm after breast malignancy. Case Report: A 50-year-old female with Infiltrating Ductal Carcinoma, presented with patches on the face, neck, chest, upper back, outer arms, dorsal hands, thighs, hair loss and weakness. Histopathology revealed widened basal layer vacuolization with widened spaces between collagen bundles filled with mucin deposition, and pigment incontinence in the papillary dermis. Direct immunofluorescence showed granular deposits of IgG, C3, IgM and IgA in the basement membrane zone, which are consistent with Dermatomyositis. The patient was prescribed with Clobetasol ointment mixed equally with petrolatum on the body and Mometasone cream on the face. She was subsequently started on Prednisone at 0.5 mkd with eventual tapering, Omeprazole, and Calcium + Vitamin D tablet daily. Hydroxychloroquine 200 mg/tab daily was added. Referral to Rheumatology for co-management and Surgery for management of the breast mass was also done. Conclusion: This case emphasizes the rare occurrence of a Paraneoplastic Dermatomyositis following a diagnosis of breast malignancy. A careful review upon diagnosis may detect occult malignancies especially in patients who present primarily with cutaneous Dermatomyositis. Clinicopathologic correlation and a multi-disciplinary approach, with Surgery, Oncology, Rheumatology and Dermatopathology are needed to identify the underlying cause and facilitate successful treatment of a neoplasm followed by improvement of Dermatomyositis.

Differential diagnosis of sclerosing skin diseases: scleredema adultorum of Buschke

Scleredema adultorum of Buschke is an important differential diagnosis in sclerosing diseases. Diagnosis is based on the typical histology with mucin deposits and grossly increased dermal width and aclinical presentation of diffuse non-pitting induration of the skin starting at the nape of the neck and interscapular region extending to shoulders and upper thorax, causing dysmobility due to dermal stiffness. Even though the pathogenesis remains unclear, three subtypes can be distinguished: association with infections, paraproteins, or most frequently with diabetes mellitus. Management of the disease includes physiotherapy, physical therapies such as ultraviolet (UV) or ionizing irradiation, intravenous immunoglobulins and interdisciplinary treatment directed at associated diseases. Optimizing diabetes therapy and thereby decreasing insulin use may confer significant improvement.

Plaque-like cutaneous mucinosis associated with pain: A case report

Plaque-like cutaneous mucinosis (PCM) is a rare atypical subtype of Lichen myxedematosus, characterized by hyperpigmented plaques typically found on the midline trunk. We report the case of a 26-year-old woman with a 1-year history of painful, pruritic, hyperpigmented plaques over her spine. Physical examination revealed soft plaques in the midline of the back, and histopathology showed dermal mucin deposition, consistent with PCM. The presented case is notable as the first reported instance of PCM presenting with lesional pain. We propose that mechanical stress, likely due to repeated friction at the site of spinous processes, contributed to the development of pain. The fact that pain lessened after the use of a lumbar support pillow and gaining weight supports our hypothesis. Our case demonstrates the importance of considering PCM in the differential diagnosis of hyperpigmented plaques and highlights the need for further research to elucidate its underlying pathogenesis and optimize treatment strategies.

Regenerative potential of platelet derived growth factor in nicotine induced intervertebral disc degenerative model – In vivo study

Intervertebral disc (IVD) degeneration is an age-related disease affecting the elderly population worldwide. The disease is contributed by degeneration of the cartilage tissue in the vertebral column. The current study explores the possibility of exploiting platelet derived growth factor (PDGF), a growth factor secreted by platelets, as a therapeutic agent against the IVD degeneration. BALB/c mice were exposed to nicotine at higher concentrations to induce IVD degeneration. Three different concentrations of PDGF (1 ng/mL, 2 ng/mL, 3 ng/mL) were used in the study. Oxidative stress parameters were assessed through Lipid peroxidation (LPO), Myeloperoxidase (MPO) and Nitric oxide (NO). Histopathology was done for assessing the extent of degeneration, collagen II, mucin, and calcium deposition by Haematoxylin and Eosin staining, immunohistochemistry, Alcian blue and Alizarin red staining respectively. Gene expression studies were carried out by quantitative real-time Polymerase chain reaction (RT-PCR) for Aggrecan, alkaline phosphatase, RUNX2, Collagen I alpha and osteocalcin. IVD degeneration was prominent and PDGF treatment restored the tissue as evidenced by H&E staining. Oxidative stress was induced by the nicotine treatment and all the biomarkers of oxidative stress were restored back to normal by PDGF in a dose dependent manner. Biomarkers of endochondral bone formation (aggrecan, alkaline phosphatase, RUNX2, Collagen I alpha and osteocalcin) was observed to be normalized by exposing to PDGF in mRNA level though RT-PCR. Mucin, calcium contents were also brought back to normal by PDGF treatment. In conclusion, current work strongly supports that PDGF could be used as a therapeutic agent for the treatment of IVD degeneration.

Reticular Erythematous Mucinosis: A Case Report and Brief Review

Reticular erythematous mucinosis is a rare entity characterised by mucin deposition in the skin. It has been reported in association with a few autoimmune diseases such as type 1 diabetes mellitus, systemic lupus erythematosus, systemic sclerosis, Hashimoto’s thyroiditis and idiopathic thrombocytopenic purpura. It is commonly misdiagnosed and treated as photodermatitis due to its subtle presentation. The case is being reported due to its rarity.

Morphological features of the late-embryo-stage gastrointestinal tract of free-roaming light ecotype Nigerian indigenous chicken.

Morphological changes in the late-embryo-stage gastrointestinal tract of Nigerian indigenous chicken were investigated using anatomical techniques. The paraffin-embedded sections were stained with haematoxylin and eosin and periodic acid Schiff-Alcian blue (pH 2.5) stains. During the late pre-hatch period, the framework of the stomach was already established, but glands were yet poorly developed. Randomly oriented pre-glandular cells in the lamina propria mucosae at embryonic day (ED) 14 became organized into elongated simple tubular glands at ED 19 and assumed adult morphology at post-hatch day (PD) 1. Acidic and neutral mucin deposits were associated with the glandular cells. In the small intestine, enterocytes of the tunica epithelialis mucosae transformed from cuboidal-shaped cells (at ED 14) to tall columnar cells (at PD 1). Short crypts of Lieberkühn, goblet cells, microfold cells and enteroendocrine cells were evident at ED 19, while the lamina propria mucosae and submucosa contained mesenchymal cells, reticular cells and isolated lymphoblasts. Similarly, the crypts, lymphoblasts, mesenchymal and reticular cells were also associated with the lamina propria mucosae and submucosa of the caecum and colorectum at ED 19. It was inferred from these findings that extensive cellular and tissue modifications occur in the gastrointestinal tract within the narrow window of the late pre-hatch period. The definitive gut tunics, epithelium, glandular tissues, immune-competent cells and tissues are formed as a result. Thus, the embryonic gut of the bird is made capable of assuming its necessary functions of food digestion, nutrient absorption, water and ion re-absorption, immune surveillance, antibody production and immune responses at hatch.

Nodular-Type Lichen Myxedematosus: A Rare Variant With Exclusive Involvement of the Hands-Report of Two Cases With a Comprehensive Review of the Literature.

Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition.

Abstract 11919: Cardiac Conundrum: A Novel Case of Isolated Cardiac Scleromyxedema

Case Presentation: A 41-year-old woman with history of diabetes, inferior STEMI with RCA PCI, and HFmrEF was transferred to our hospital with worsening cardiogenic shock on ionotropic support. She was admitted to an outside facility one month prior with worsening dyspnea on exertion, peripheral edema, paroxysmal nocturnal dyspnea, pre-syncope, and weakness. TTE demonstrated LVEF of 30-35% (40-45% previously) with severe inferior and posterior wall hypokinesis. Cardiac MRI revealed LVEF of 23%, prior RCA infarct, full-thickness late gadolinium enhancement in the left ventricular basilar wall, global abnormal parametric mapping parameters of both native T1, T2 and ECV, and severe biventricular dysfunction concerning for infiltrative cardiomyopathy. Left heart catheterization revealed nonobstructive CAD. Right heart catheterization showed severely elevated right sided filling pressures and endomyocardial biopsy revealed frequent deposits of interstitial mucin (Figure 1), later confirmed by electron microscopy; a Congo red stain was negative for amyloid deposition. Additional work up included serum protein electrophoresis, which was negative for monoclonal proteins. She had no dermatologic disease. A diagnosis of isolated cardiac scleromyxedema was made. Discussion: Scleromyxedema is a rare mucinous disorder typically characterized by cutaneous mucin deposits and fibroblastic proliferation. It is commonly associated with monoclonal gammopathies, specifically IgG lambda; however, there are rare cases without paraproteinemia. Systemic mucinous infiltration has been reported and cardiac involvement specifically has been described in up to 20% of patients, although it has never been reported without cutaneous manifestations or monoclonal gammopathy as demonstrated with our patient. The patient received IVIg treatment, however required two additional hospitalizations over the next four months for decompensated heart failure.

Recurrence after cytoreductive surgery and HIPEC for pseudomyxoma peritonei: A single-center retrospective cohort study.

Pseudomyxoma peritonei (PMP) is a rare disease characterized by progressive build-up of mucinous deposits inside the abdominal cavity. The aim of this study was to investigate the effect of disease recurrence on overall survival in patients with PMP after cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). One-hundred thirty-two consecutive PMP patients treated with CRS + HIPEC at Helsinki University Hospital between 2008 and 2017 were included. The impact of clinicopathological and treatment-related characteristics on recurrence and overall survival was evaluated. The median follow-up time in the study was 5.04 (range = 0.05-11.60) years. In 121 (91.7%) patients, the disease was classified as low grade and 11 (8.3%) had high-grade disease. In the low-grade group, 26 (21.5%) patients developed a recurrence during follow-up compared to 6 (54.5%) patients in the high-grade group. In the low-grade group, cumulative survival was 98.2%, 91.4%, and 91.4% at 3, 6, and 8 years, respectively. In the high-grade group, cumulative survival was 90.0% and 78.8% at 3 and 6 years, respectively. In patients with recurrent disease, the cumulative survival was 100%, 84.6%, and 84.6% at 3, 6, and 8 years in the low-grade category and 80.0% and 60.0% at 3 and 6 years in the high-grade category, respectively. In the low-grade group, a statistically significant correlation with recurrence but not with overall survival was identified with peritoneal cancer index (PCI), carcinoembryonic antigen (CEA), and the number of affected regions. The recurrence of low-grade PMP does not significantly affect overall survival of patients. Disease extent may not be a prognostic indicator after curative CRS and HIPEC in low-grade PMP.

P22 Will the real scleroderma please stand up?: a case of a rare scleroderma mimic in ED

P22 Will the real scleroderma please stand up?: a case of a rare scleroderma mimic in ED

Effect of ciclosporin on clinical signs of degenerative mucinotic mural folliculitis in a young cat

AbstractA 1.5‐year‐old, spayed, female, mixed‐breed cat that had been housed completely indoors presented with a chief complaint of facial hair loss. Alopecia extended from the face to the outside of the auricle, and was accompanied by thickening of the dorsal eyelid and scales on the dorsal chest. Histopathological examination revealed infiltration of lymphocytes targeting the hair follicle wall and deposition of mucin in the isthmus of hair follicles. A diagnosis of degenerative mucinotic mural folliculitis was obtained based on the clinical and histological findings. The cat was treated with ciclosporin (9.3 mg/kg) administered once daily. The alopecia and thickening resolved, and the general condition improved. The cat was clinically well for 10 months. The cat has survived 14 months since treatment was started. This case demonstrates the efficacy of high‐dose ciclosporin in treating degenerative mucinotic mural folliculitis.

Increased histological severity and chronicity of interface inflammation in anti-MDA5 dermatomyositis-A comparative dermatopathological analysis.

Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis is characterized by serological detection of anti-MDA5 antibody and rapidly progressive interstitial lung disease. In this study, the largest cohort of skin biopsies to date of anti-MDA5 dermatomyositis was reviewed and compared with cases of dermatomyositis with negative serology. Findings contribute to the histological diagnosis and evaluation of the severity of cutaneous inflammation in anti-MDA5 dermatomyositis. Skin biopsies collected over a 7-year period from individuals with clinically and histologically confirmed dermatomyositis with anti-MDA5 serology were reviewed. A total of 46 cases with 17 anti-MDA5 positive cases were retrieved. Patients with positive antibody were younger (53.7 vs. 60.6 years, p = .013). No differences in epidermal changes (p > .05) were observed. Pertaining to interface changes, anti-MDA5 dermatomyositis showed a higher degree of pigmentary incontinence (p = .014), suggesting increased and sustained cutaneous inflammation. Periodic acid-Schiff (PAS) stain demonstrated a greater degree of basement membrane thickening (p = .045). Other parameters, including dermal inflammation, dermal mucin deposition and vasculitic/vasculopathic features did not show statistical difference between anti-MDA5 positive and negative dermatomyositis (p > .05). Findings suggest increased cutaneous inflammation for anti-MDA5 dermatomyositis. In skin biopsies, marked pigmentary incontinence or basement membrane thickening should raise suspicion of anti-MDA5 dermatomyositis.

Patterns of Mucin Deposition in Lichen Planopilaris: A Journey From Follicular "Bubbles" to Perifollicular Fibroplasia.

Lichen Planopiolaris (LPP) is a scarring alopecia characterised by a perifollicular lymphoid cell infiltrate at the level of the infundibulum and isthmus. While perifollicular mucinous fibroplasia is an established finding in LPP, intrafollicular mucin deposition has not been previously reported. We describe two cases with this histopathology and suggest it may represent a helpful clue to the diagnosis of LPP, in the appropriate clinical setting.

Incidental finding of appendiceal mucinous neoplasms

Low-grade appendiceal mucinous neoplasms (LAMN) are rare and are diagnosed in at least 0.13% of appendectomies in Germany, although significant underreporting is likely. Perforation of the tumors can result in abdominal mucinous collections, so-called pseudomyxoma peritonei (PMP). The challenge in the treatment of LAMN is the adequate approach to the incidental finding of these tumors. If a mucinous neoplasm is preoperatively suspected in cases of an acute condition, usually appendicitis, it must be weighed up whether aconservative approach is justifiable or whether immediate appendectomy is necessary. If this is the case, an intraoperative perforation of the appendix must be avoided and the complete abdominal cavity must be inspected for mucin deposits. If conservative treatment is possible, further treatment should take place at aspecialized center. If the neoplasm is first found incidentally during surgery, perforation of the appendix should also be avoided and the entire abdominal cavity should be inspected for aPMP. If a PMP is present cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) should be performed in a specialized center. If LAMN are found during the postoperative histological work-up, it should be evaluated whether aperforation was present and mucin collections are noted in the surgical report. In the case of LAMN without evidence of a PMP, appendectomy is the adequate treatment. In cases of intra-abdominal mucinous collections, samples should be taken and further treatment should be performed at a center with sufficient expertise. An ileocecal resection or oncological hemicolectomy is not indicated. After adequate treatment, all patients should receive a follow-up using cross-sectional imaging (preferably magnetic resonance imaging, MRI) and determination of the tumor markers CEA, CA 19-9 and CA 125.

CPC04 A rare rupioid rash

Abstract A 59-year-old man was referred to an urgent skin cancer dermatology clinic with a 4-month history of multiple skin lesions on his trunk, arms and face. The lesions were painful, increasing in size, bleeding and oozing serous fluid. The patient reported being otherwise systemically well, with a history of mild eczema and a background of high alcohol intake. On examination, he had a striking rash with numerous large rupioid plaques with thick crusting and surrounding erythema on the trunk and limbs. Smaller plaques were seen on the face and neck. An infective aetiology was favoured with an initial differential diagnosis of ecthyma or atypical mycobacterial infection. A biopsy was taken, as well as screening blood tests. Treponemal antibodies were detected; rapid plasma reagin was reactive (1 : 32), as was Serodia Treponema pallidum particle agglutination test (1 : 1280), suggesting active syphilis infection. His biopsy demonstrated granulomatous inflammation, associated with neutrophils, plasma cells and increased mucin deposition. Immunohistochemical staining for T. pallidum confirmed corkscrew-like organisms within the basal epidermis and upper dermis, consistent with secondary syphilis; screening for concurrent HIV infection was negative. The patient was referred to a local genitourinary clinic for treatment with benzylpenicillin and for full sexual health screening. The word ‘rupioid’ describes oyster or limpet shell-shaped thick keratotic lesions and is derived from the Greek word rhupos, meaning ‘filth’. Differential causes for rupioid lesions include cutaneous T-cell lymphoma, psoriasis, scabies and syphilis. Rupioid syphilis is also termed ‘malignant syphilis’ or ‘Lues maligna’, describing well-demarcated plaques with thick, lamellate and adherent crusts. It is most frequently encountered in HIV-positive patients; in HIV-negative patients, the associated comorbidities are diabetes, alcoholism, drug abuse, psoriasis and hepatitis. Typically, there is rapid clinical improvement once antibiotic treatment is administered, but episodes of Jarisch Herxheimer reaction are reported. Syphilis diagnoses have tripled over the last 10 years, with cases in the heterosexual population rising most rapidly. Dermatologists have a pivotal role to play in diagnosing primary and secondary syphilis, thereby preventing the long-term sequelae of tertiary syphilis and controlling the rising prevalence of the disease in the general population. This case highlights a striking and rare manifestation of secondary syphilis which, once seen, will not be forgotten.

Clinical and histopathological features of rostrolateral nasal alar arteriopathy of German shepherd dogs.

Dermal arteritis of the nasal philtrum (DANP) has been described in large-breed dogs. To characterise clinically distinct, discrete fissures of the dorsolateral nasal alae associated with severe bleeding in German shepherd dogs (GSDs). Fourteen privately owned GSDs with linear rostrolateral nasal alar fissures and a histopathological diagnosis of nasal vasculopathy. Retrospective analysis of medical records and histological slides. Mean age of onset was 6 years. Before biopsy, episodic arteriolar bleeding was noted in 11 of the 14 (79%) dogs. Slide analysis revealed enlarged nasal arterioles with expanded vascular tunics and luminal stenosis beneath ulcers. Histopathological lesions consistent with mucocutaneous pyoderma and/or facial discoid lupus erythematosus were present in 5 of the 14 (36%) dogs. Enlarged arterioles stained blue with Alcian blue and Masson's trichrome stains, consistent with deposition of mucin and collagen, respectively. Immunohistochemical stains (neutrophil myeloperoxidase, IBA1, CD3) were performed. CD3 was negative for all dogs, whilst neutrophil myeloperoxidase and IBA1 occasionally demonstrated intramural neutrophils (3 of the 14 dogs, 21%) or histiocytes (1 of the 14 dogs, 7%) in altered vessels, respectively. All dogs underwent medical management and/or surgical excision. Treatments included tacrolimus, prednisone, ciclosporin-modified, pentoxifylline, antimicrobials and doxycycline/niacinamide. No dogs were treated with antimicrobials alone. For seven dogs with long-term follow-up, treatment response was complete in five (71%) and partial in two (29%), with six of the seven (86%) receiving immunomodulatory treatments to maintain remission. Nasal alar arteriopathy of GSDs shares histopathological changes with DANP. It has characteristic clinical and histopathological features and appears amenable to immunomodulation.