Multiple Erythematous Plaques Research Articles

Retrospective study of the clinical significance of the neutrophil-to-lymphocyte ratio in 79 patients with palmoplantar pustulosis.

Palmoplantar pustulosis (PPP) is a chronic relapsing inflammatory skin disease characterized by multiple vesicles, pustules, and erythematous plaques on the palms and soles. The exacerbation of PPP is strongly associated with focal infections, such as tonsillitis, dental infections, and sinusitis, in Japan. Recently, the neutrophil-to-lymphocyte ratio (NLR) has been widely used as a convenient and useful marker for clinical conditions and various diseases; however, an association between PPP and NLR has not yet been established. We retrospectively analyzed 79 patients with PPP from our hospital to evaluate the clinical significance of the NLR. The average NLR value in patients with PPP was significantly higher than that in healthy controls (2.30 ± 1.02 vs 1.69 ± 0.45, P < 0.001). A comparative analysis of patients with PPP with and without infectious complications showed that there was a statistical difference in the NLR between patients with PPP with and without focal infections, whereas no significant difference was found for metal allergy, smoking, and pustulotic arthro-osteitis. Multivariate analysis indicated that the NLR was significantly associated with focal infections (odds ratio = 18.38, 95% confidence interval 3.86-87.35, P < 0.001). The NLR was also significantly correlated with C-reactive protein levels (P = 0.013, r = 0.2857). Interestingly, after symptom improvement, the NLR significantly decreased from the baseline levels. Furthermore, statistical analysis using the Youden's index revealed that an NLR of 2.28 or higher was associated with the risk of any focal infections in patients with PPP. These results suggest that the NLR has potential applications as a biomarker of the presence of focal infections in patients with PPP.

Risky Behavior of Men who have Sex with Men (MSM) Increases the Risk of HIV and Syphilis Infection

The incidence of sexually transmitted infections in man who have sex with man (MSM) is increased among human immunodeficiency virus (HIV) patients. HIV and syphilis are two sexually transmitted diseases that frequently interact and modify the symptoms and signs of the disease. A case of a 27-year-old male with generalized multiple erythematous plaques including palms and soles since 4 days ago was reported. The patient has been diagnosed with HIV since 2017. The patient is sexually active with a male sex partner and rarely uses condoms. Diagnosis of syphilis is made based on anamnesis, physical examination, and laboratory tests. The treatment was benzathine penicillin G 2.4 million units in 3 doses. The combination of HIV and syphilis needs longer therapy and requires serological follow-up for up to 24 months.

Palmoplantar Erythrodysesthesia: An Unusual Side Effect of Lenvatinib

Palmoplantar erythrodysesthesia also known as hand-foot syndrome is an intense, painful erythema of the palms and soles due to chemotherapeutic drugs. The most commonly implicated drugs are multi-kinase inhibitors such as sorafenib, sunitinib, doxorubicin, cytarabine, and docetaxel. The most effective management is withdrawal or reduction of drugs. Oral and topical corticosteroids and dimethyl sulfoxide are other treatment options. A 67-year-old male patient of hepatocellular carcinoma taking tablet lenvatinib presented with multiple tender erythematous plaques and patches over the bilateral palms and dorsum of the fingers after 1 month of initiation of therapy. The patient was successfully treated with dose reduction of lenvatinib and topical corticosteroids and is maintained on medium potency corticosteroid.

A mismanaged case of post herpetic nasal stenosis: a salvage story

Acquired nasal stenosis is due to loss of vestibular lining because of scar contracture or direct injury to the lobule-ala-columella complex. We present a patient of Herpes zoster mismanagement with scarring of the right nasal cavity who was treated by placing a stent to achieve nasal patency. A 47-year-old male, previously diagnosed to have Herpes zoster, presented with complaints of right nasal obstruction, discolouration of skin and burning sensation over the right cheek and nose. He had applied an herbal paste given by quacks which led to scarring and hypopigmentation over the face. He tried peeling the scab off which resulted in a skin contracture and right nasal obstruction. Local examination revealed multiple erythematous hypopigmented plaques over the right maxillary region, scarring over the right lateral aspect of the dorsum, ala and tip and a skin contracture causing stenosis of the right anterior nares. The stenosed area was excised and diagnostic nasal endoscopy showed collapse of the ala and the vestibular region in the right nasal cavity. A red rubber catheter was used as a stent and sutured in place at the ala. Postoperatively, nasal cavity patency was maintained. Herpes zoster is usually self-limiting and treatment with analgesics suffices. Antiviral medication and corticosteroids may alleviate severe pain. However, mismanagement of the lesions by taking local herbal paste and medicines can lead to exacerbation of the lesions and complications like nasal stenosis.

A case of chromoblastomycosis caused by Fonsecaea pedrosoi: challenge in diagnosis

Link of Video Abstract: https://youtu.be/9CxOCuDn4f4 Background: Chromoblastomycosis (CBM) is a chronic, granulomatous mycosis of the skin and subcutaneous tissue produced by the traumatic inoculation of various dematiaceous fungi. CBM lesions are clinically polymorphic and often misdiagnosed as multiple infectious and noninfectious diseases. Early diagnosis and prompt treatment of CBM are important to prevent further complications. This case study aims to evaluate the chromoblastomycosis caused by Fonsecaea pedrosoi as a challenge in diagnosis. Case Presentation: A male, 47 years old, came to the dermatovenereology clinic with a chief complaint of a wound on his right leg 17 years ago. The skin appeared thicker and scaly and felt rough. He also complained of pain and itch on the wound. Dermatological examination revealed multiple verrucous erythematous plaques, sized 10x20 cm, irregular with a well-defined and elevated border and black punctum. Results of potassium hydroxide, culture, and histopathology examination confirmed CBM with F. pedrosoi as the etiological agent. The patient was treated with a favorable outcome with oral itraconazole, local thermotherapy, and CO2 laser. Conclusion: The diagnosis of Chromoblastomycosis remains a challenge because lesions are clinically polymorphic and often misdiagnosed as various infectious and noninfectious diseases. Physicians should raise awareness of CBM as one of the differential diagnoses for chronic wounds. This case also showed that the combination treatment of itraconazole with adjuvant thermotherapy and CO2 laser had a favorable outcome.

Dermoscopy of xanthelasmoid mastocytosis

A one-year-old boy presented with a 3-months history of pruriginous cutaneous lesions on his trunk and limbs. Dermatological examination revealed multiple erythematous and pigmented plaques and nodules varying in size (Figure 1A).

The Enigma of a Keratitis-Ichthyosis-Deafness with Silence, Scaling, and Related Syndrome

Abstract Ichthyosis is a group of genetic disorders characterized by generalized dry skin and scaling, and it can be frequently associated with erythroderma. Here, we report a case of ichthyosis associated with a rare syndrome. A 6-year-old congenitally deaf female presented with severe scaling and generalized erythema for 6 months which began with a scaly plaque over the face at the age of 18 months and gradually covered the whole body. Three similar episodes happened after that with a gap of 6 months. There were multiple scaly erythematous plaques present over the trunk and limbs and thick hyperkeratotic plaques were present in axillary and inguinal folds. The scalp hairs were sparse. Histopathology showed psoriasiform changes. Salivary sample sent for genetic analysis revealed an heterozygous mutation c.42C&gt;G (p.Asn14Lys) in GJB2 gene that is known to harbor pathogenic mutations associated with keratitis-ichthyosis-deafness (KID) syndrome. KID syndrome presents with the triad of keratitis, progressive erythrokeratoderma, and hearing loss.

Rescue therapy in methotrexate toxicity: case report

Methotrexate (MTX) is widely used for the treatment of psoriasis. It has an antiproliferative action by inhibiting dihydrofolate reductase enzyme, therefore interferes with folic acid synthesis. MTX toxicity is rarely reported and the most common cause of it is accidental overdosage by the patient. This is a case report of a 51-year-old male, a known case of psoriasis, who presented with multiple erythematous tender plaques associated with burning sensation. He self-medicated with tablet MTX 5 mg daily for 10 days when the lesions got exacerbated and was found to be leucopenic. On skin biopsy, diagnosis of psoriasis with MTX induced epidermal necrolysis was established. Patient was treated with filgrastim 300 μg which was given subcutaneously 1 day apart. On follow up, lesions subsided and repeat counts were within normal limits. Patients should be explained about the selected treatment regime and discouraged from self-medicating.

DP22 Cutaneous lymphoid hyperplasia: clinicopathological correlation and a therapeutic option

Abstract Cutaneous reactive lymphoid hyperplasia, also known as cutaneous pseudolymphoma, is an uncommon entity in which there are skin lesions that histologically, and sometimes clinically, mimic cutaneous lymphoma but are not, both morphologically and immunohistochemically. Correlating clinical, pathological, immunohistochemical and, occasionally, molecular findings is essential for the diagnosis (Romero-Perez D, Blanes Martinez M, Encabo-Duran B. Pseudolinfomas cutaneous. Actas Dermosifiliogr 2016; 107:640–51). Although the majority of cases can be regarded as idiopathic, certain infections and inflammatory conditions can precipitate cutaneous reactive lymphoid hyperplasia, examples of which include Borrelia infection, insect bite reactions and folliculitis (Mitteldorf, C, Kempf, W. Cutaneous pseudolymphoma – a review on the spectrum and a proposal for a new classification. J Cutan Pathol 2020; 47:76–97). The first case was an 18-year-old woman who presented with deep dermal and subcutaneous nodules for the last 5 years that started on her limbs and were slightly itchy. The nodules were firm and deep, with minimal surface changes (hyperpigmentation and adherent scale) but did not ulcerate. She also had nodules involving her buccal mucosa, inner lips and soft tissue in the nasal bridge and a couple of deep nodules on the cheeks. Pathological examination showed a dense lymphocytic infiltrate with plasma cells admixed with other inflammatory cells involving the deep dermis with an extension to the dermal/subcutaneous junction. The pathological picture was in the spectrum of deep tumid lupus/lupus panniculitis overlap and cutaneous lymphoid hyperplasia. Immunohistochemical stains showed a mixture of inflammatory cells with CD3+ and CD20+ cells. Lip mucosal biopsy showed florid reactive lymphoid follicular hyperplasia. Her condition was resistant to multiple systemic treatments. Eventually, she underwent treatment with rituximab and had satisfactory results. The second patient is an elderly female who had multiple erythematous papules, plaques and nodules on her face. Pathological examination showed a dense dermal lymphocytic infiltrate with periadnexal predilection and reactive follicles that also were highlighted by CD20, with Bcl2 and CD3+ cells around germinal centres. She was diagnosed as having cutaneous reactive lymphoid hyperplasia. Topical calcineurin inhibitors were initiated, with a good response. In conclusion, this paper highlights the term cutaneous pseudolymphoma/cutaneous reactive lymphoid hyperplasia, presenting a few cases with clinicopathological correlation and exploring some uncommon treatment options.

Mycosis Fungoides: A Diagnostic Challenge

Background: Mycosis fungoides (MF) is the most frequent type of cutaneous T-cell lymphomas (CTCL), a heterogeneous group of non-Hodgkin lymphoma of T-cell origin. CTCLs accounts for about 4% of all non-Hodgkin lymphoma. MF mainly involves the skin but in advanced cases it may involve the lymph, blood and other organs. It mimics other skin disorders like erythema necroticans, leprosy, psoriasis etc which leads to delayed diagnosis and subsequent treatment. Case report: A 61 year old male patient visited the OPD of a tertiary care hospital in Eastern India with multiple reddish elevated lesions for last 6 months which later became dome shaped and then ulcerated. He did not have any fever or itching. He informed that he had been taking Homeopathic medicine previously, but the identity of the said medicine could not be confirmed. Physical examination ruled out the presence of pallor, jaundice, oedema and cyanosis. Pulse and BP were within normal range. Systemic examination was unremarkable except mild hepatomegaly. Cutaneous examination revealed multiple erythematous indurated plaques, papules and nodules over chest, abdomen, back, limbs and face. Few of the plaques present on the trunk and left arm had developed ulcers having a necrotic floors. Cervical, axillary and inguinal lymph nodes were palpable, being firm in consistency and mobile in nature. Hair, nail and mucosal examination revealed normal results. Complete blood count (CBC), peripheral blood smear(PBS), serum urea and creatinine level did not deviate from normal findings. Ultrasonography of whole abdomen showed a mildly enlarged liver. A prominent lymph node in the aortocaval groove could be appreciated on the computed tomography(CT) scan of whole abdomen. CT scan of chest revealed bilateral axillary lymphadenopathy. Erythema necroticans, sarcoidosis and CTCL were considered as the differential diagnoses. Lesional biopsy revealed irregular epidermal hyperplasia, parakeratosis and epidermotropism in the absence of spongiosis.There was infiltration of atypical lymphocytes in the epidermis, forming well developed Pautrier’s micro abscesses. The lymphocytes were tagging the dermo-epidermal junction and within the epidermis showing surrounding halo, convoluted nuclei and variable nuclear pleomorphism. Band like papillary dermal lymphoid infiltrate, dermal lymphoid fibroplasia and nodular lymphoid aggregates could be appreciated in the deep dermis. Immunohistochemical tests were performed and the immunophenotypic profile revealed positivity for CD2, CD3, CD5, CD7, CD 8 and TIA1 and negative for CD4, CD20, CD30, CD56 and granzyme B. Based on clinical presentation, histopathology and Immunohistochemical tests, a final diagnosis of MF was made. The patient was then started on systemic antibiotic therapy, due to increased risk of bacterial super infection of lesions due to skin barrier disruption. He was then referred to Oncology department for further treatment. Conclusion: In this case, the male patient was suffering from mycosis fungoides. In the background of its clinical features being similar to other skin disorders like erythema necroticans, clinicians may face a diagnostic dilemma to correctly diagnose it. Timely diagnosis and treatment improves the prognosis in most cases. Histopathological evaluation still remains the investigation of choice.

Parvovirus B19 infection in a patient of juvenile arthritis: A case report

Abstract Parvovirus B19 infection, or fifth disease or erythema infectiosum, is a self-limiting viral exanthem and can occur along with other connective tissue disorders. Occasionally, children may have preceding chronic joint complaints suggestive of juvenile idiopathic arthritis. A 7-year-old female patient presented to the outpatient department with a rash over her face, upper and lower limbs, and trunk, which was preceded by fever. The patient is a known case of juvenile arthritis. On examination, there were multiple erythematous and skin-colored plaques over cheeks; few pustules over lips; multiple erythematous papules over upper limbs, lower limbs, and trunk; multiple erythematous macules over palms; and swelling over interphalangeal joints of bilateral hands, elbow and knee joints with genu valgum deformity of limbs. The patient was given topical antibiotic and steroid combination cream and oral antihistaminic for her cutaneous complaints.

Recurrent cutaneous eosinophilic vasculitis characterized by annular purpuric lesions: A case report

A 71-year-old woman presented with a persistent, intensely pruritic cutaneous eruption localized on the palmoplantar regions, lips and palate. The histological findings allowed to make the diagnosis of recurrent cutaneous eosinophilic vasculitis, a very rare cutaneous vasculitis characterized clinically by multiple erythematous or purpuric erythematous papules or plaques or angioedema with a relapsing course in the absence of systemic involvement and histologically by a necrotizing vasculitis of the dermal small vessels with a dominant eosinophilic infiltration. The patient was treated with oral methylprednisolone and pentoxifylline which led to a rapid resolution of the cutaneous lesions.

Spurious Presentation of Pulmonary Kaposi Sarcoma as Unresolved Pneumonia Led to Fatal Outcome.

Acquired immunodeficiency syndrome (AIDS)-associated Kaposi sarcoma (KS) is an angioproliferative neoplasia caused by infection with human herpesvirus 8 (HHV-8). It typically presents with mucocutaneous involvement, but it can be disseminated. Initial presentation with primarily pulmonary KS is rare. We present a case of a 32-year-old male with untreated human immunodeficiency virus (HIV) diagnosed 1 year before presentation who developed progressively worsening cough and shortness of breath for 6 months. He was hospitalized twice and treated for unresolved pneumonia in an outside hospital. The patient concomitantly developed purplish nodules on his face, then the upper trunk, back, chest, and thighs bilaterally that gradually increased in size and number. Histopathology findings from skin lesions were consistent for KS. Bronchoscopy found multiple erythematous plaques throughout the tracheobronchial tree with telangiectasias and inflammation suggestive of pulmonary KS. His imaging findings and positive serum HHV-8 polymerase chain reaction (PCR) were consistent with disseminated KS. He started antiretroviral therapy (ART) to treat his HIV infection, followed by liposomal doxorubicin chemotherapy. But both ART and chemotherapy were interrupted due to adherence and insurance issues. The patient was readmitted with acute respiratory failure requiring mechanical ventilation with multiple vasopressors that led to the patient's demise. The late recognition of KS diagnosis and delayed treatment can lead to worse outcomes.

Non-pustular annular psoriasis with signs of high disease activity

Background: Non-pustular annular psoriasis is a rare condition in which a type of plaque psoriasis appears with ring-like lesions and a clearer central appearance. The disease activities in psoriasis may be evaluated clinically. It is essential to provide a more meaningful approach to treatment management. Case illustration: A 33-year-old woman complained of a one-year history of multiple erythematous, scaly, and annular plaques with central clearing. Some lesions were surrounded by discrete pinpoint papules on several parts of her body. The lesions worsened during seven weeks of topical and systemic therapy. Discussion: Psoriasis is a chronic, multisystem inflammatory disease with predominantly skin and joint involvement. High disease activity or unstable psoriasis was defined as recent exacerbations showing pinpoint papules developing around established and erythematous borders of the lesions. The active severe disease may present with systemic involvement due to the production of pro-inflammatory chemokines and cytokines in the skin lesions and circulation. Conclusion: A complete clinical history and physical examination can help the clinician diagnose non- pustular annular psoriasis. Several factors may cause worsening disease activity in a patient with psoriasis. Distinguishing between stable and unstable plaque psoriasis is essential to obtain information on psoriasis pathomechanism related to systemic involvement and managing therapeutic strategies. A multidisciplinary approach must be conducted in the management of a patient with high disease activity.

1427. Correlation of Dermoscopic and Histopathologic Patterns in Leprosy-a Pilot Study

Abstract Background Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae. It is diagnosed based on clinical features and confirmed on the histological findings and peripheral slit skin smear staining. Dermoscopy is a handy easily accessible tool to diagnose this granulomatous disease and classify patients based on the immunological and clinical response. Methods A total of 50 patients in different poles of leprosy were evaluated as a single spot observational analysis. The clinical and histological findings were correlated with the characteristic dermoscopy findings. A Lepromatous leprosy showing xerosis and scaling along leg as part of glove and stocking distribution of lesions Dermoscopic pattern in lepromatous leprosy showing partial loss of hair follicles, dry xerotic skin, and white characteristic scaling (Heine Delta® 20 T dermatoscope; 16×; polarizing) Results The dermoscopy findings correlated with the clinical and histological findings. Tuberculoid poles of leprosy classically showed loss of hair and skin pigment along with absence of white dots as sweat glands in dermoscopy. Lack of blood vessel changes ruled out any lepra reaction. Lepromatous pole of leprosy on the other hand showed characteristic xerosis and white scaling on dermoscopy in the background of hypotrichosis and hypopigmentation. leprosy reactions were characterized by blood vessel changes and arborizing blood vessels were characteristic in erythema nodosum leprosum and a diffuse erythema was a clue towards diagnosing type I lepra reaction. Interestingly clofazimine induced pigmentation was picked up characteristically on dermoscopy as a ‘honey comb pattern’. Borderline tuberculoid leprosy with a hypoaesthetic patch on the left side of face with a thickened greater auricular nerve Dermoscopic pattern in borderline tuberculoid leprosy showing white areas with loss of hair follicles. In between yellow globules can be seen which was the hallmark apart from white areas demonstrating hypopigmentation. Absence of blood vessels rules out lepra reaction (Heine Delta® 20 T dermatoscope; 16×; polarizing) Conclusion Dermoscopy is certainly a handy tool in aiding the diagnosis of leprosy, lepra reactions and course of therapy. Characteristic patterns during the course of leprosy would certainly facilitate a quick and definitive diagnosis of patients suffering from leprosy. Also patient drug compliance particularly to Clofazimine can also be picked up objectively on Clofazimine. Multiple erythematous plaques on the back depicting type 1 lepra reaction Dermoscopic features of type I lepra reaction showing characteristic diffuse erythematous background and branching telangiectatic vessels along with sparse hair follicles and scaling (Heine Delta®20 T dermatoscope; 16×; polarizing) Disclosures All Authors: No reported disclosures.

Multiple Annular Erythematous Plaques

A 59-year-old man was admitted to the medical ward with multiple annular erythematous plaques and polyarthralgia of several months’ duration. His medical history included low-grade stage IIA follicular lymphoma diagnosed 6 months prior to presentation, substance abuse with opiates and cocaine, coronary artery disease, ascending aortic aneurysm, and chronic lower back pain.

The Expansion of Lyme Disease: A Case of Infection in the Absence of Known Exposure

Lyme disease is a systemic infection caused by the spirochete Borrelia burgdorferi often carried the Ixodes tick. It is associated with dermatologic manifestations, most notably the “bullseye rash” of erythema migrans. The disease can progress in stages to involve other organs such as joints, heart, and the nervous system. Lyme disease is continuing to spread, with West Virginia cases increasing dramatically in the last 2 decades. As a result, physicians are likely to encounter this disease more often, making recognition and early treatment a top priority in order to prevent potentially dangerous sequelae. We present a case of a 60-year-old man who presented with multiple erythematous annular plaques and was subsequently diagnosed with Lyme disease. He had no known exposure to ticks, was treated for Lyme Disease, and subsequently developed a Jarisch- Herxheimer reaction (JHR).

Diagnosis and Treatment of Multibacillary Leprosy Borderline Lepromatous Type: A Case Report

Background: Leprosy is a chronic granulomatous infectious disease caused by Mycobacterium leprae. This disease has a high transmission ability and can cause physical disability and have an impact on the social life of the sufferer because of the negative stigma about leprosy. This study aims to describe the examinations performed in the diagnosis and treatment of patients with multibacillary leprosy, borderline lepromatous type.&#x0D; Case presentation: A 43-year-old woman with a chief complaint of redness on the arm since 1 year ago. The patches are not itchy, painless, stiff, and numb. Since 3 months ago, the patient complained of red patches increasing and spreading to the trunk and legs. Dermatological examination found in the facial region et truncus anterior et posterior et superior, and inferior extremities bilateral showing multiple erythematous plaques, well-demarcated with scales in several parts. Sensory function examination revealed a decrease in lesions on the face, superior and inferior extremities, and anterior and posterior trunks. Negative AFB examination and biopsy results support the diagnosis of borderline lepromatous type (BL) multibacillary leprosy (MB).&#x0D; Conclusion: Clinical findings in the form of hypopigmented lesions that feel numb is a cardinal signs of leprosy. Asymmetrical distribution of lesions with a number of more than 5 lesions accompanied by impaired nerve function in the form of decreased sensibility is a characteristic of MB leprosy. Histopathological features support the diagnosis of MB type BL leprosy even though the results of the skin slit smear examination were negative.

OA14 A case of scleromyxedema with muscle involvement including myotonia and dysphagia

Introduction/BackgroundScleromyxedema is a rare, para-neoplastic, progressive condition of the Lichen myxedematous family. It is clinically characterized by generalized papules with skin infiltration and underlying systemic disorders, which may be fatal as it still constitutes a therapeutic dilemma.Description/MethodWe are reporting a rare case of scleromyxedema presented to the rheumatology department mainly with dysphagia, weight loss, lower limb weakness and myotonia under dermatological and haematological review.Case description: In January 2011, a 49-year-old man who was diagnosed year earlier with papular mucinosis and paraproteinemia-M band 8g/l (Monoclonal gammopathy of undetermined significance (MGUS)) presented with dysphagia mainly for solid, weight loss about 10 kg over the period of three months without other B symptoms and stiffness mainly in the hands and hips when walking or opening a jar described as slow relaxation following contraction.On examination he looked well with full upper limb muscle strength. Scleromyxedmatous skin lesion in the form of multiple painful erythematous plaques and grouped papules involving his knees, elbows, dorsum of the hands and feet were appreciated. There were no telangiectasias or calcinosis. Neurological examination was unremarkable without muscle wasting or fasciculations, but there was a slow release of grip. Chest, heart, and abdominal examination was normal.There was marked improvement of his skin lesion on 50mg thalidomide up to 150 mg daily, however his condition, including the skin lesions fluctuated in severity. Overall, he exhibited a benign course. The myotonia seemed to improve over time.Discussion/ResultsInvestigations showed raised creatine kinase at 1200 with normal full blood counts, normal renal, liver, calcium, CRP, ANA, ANCA and thyroid function test, normal Anti GAD, normal anti-voltage-gated potassium channel antibodies, normal barium swallow, unremarkable CT scan of chest, abdomen, and pelvis. Bone marrow biopsy ruled out plasma cell proliferation, M band always stable at 8 to 10 g/l. There were typical myotonic discharges on EMG with normal NCS. Normal MD and PROMM at ZNF9 genes.Muscle biopsy showed several groups of very small atrophic fibres and patchy fibrosis with attempts of regeneration with PAS positive vacuoles deposition within the muscle fibres, replacing in places the majority of sarcoplasm with evidence of increased fibroblastic activity.Over three to four years post diagnosis he developed only mild weakness in shoulder abduction and neck flexion MMT8 77/80, his CK remained high with a range of 900 to 3000 U/L. His weight stabilized.Discussion: We present a rare case of scleromyxedema-associated with dysphagia, myotonia and muscle weakness. These symptoms in combination with cutaneous lesions raise the alternate diagnostic possibilities of systemic sclerosis-associated myositis, dermatomyositis, and myxedema. The diagnosis of scleromyxedema can be missed in this setting owing to the rarity of the disease. Histology can help make a definitive diagnosis; in our case mucinous material were demonstrated in the muscles as well as the skin lesions. Although cases of scleromyxedema remain rare, a better understanding of its mechanisms of action could have implications for the research and treatment of autoimmune conditions. As our case several authors have reported rheumatic features in association with scleromyxedema in the form of erosive seronegative rheumatoid like arthropathy, sicca complex, dysphagia with oesophageal aperistalsis and sclerodactyly with acrolysis.Key learning points/ConclusionThe pathogenesis of systemic findings in scleromyxedema remains unknown although aggressive therapy mainly in the form of corticosteroid, thalidomide and IVIG may provide disease control, the prognosis remains guarded due to the systemic complications and high relapse rate. We need further studies to look at the link between scleromyxedema and systemic features.

Morbus Hansen Multibacillary Type Mid Borderline: A Case Report

Background: Morbus Hansen (MH) is a granulomatous infectious disease caused by an obligate intracellular pathogenic bacterium, Mycobacterium leprae (M. leprae). Cardinal signs of Hansen's morbus (MH) include patches of numb skin, thickening of the peripheral nerves, and acid-fast bacteria (AFB) on a skin smear or biopsy. This case report aims to establish the diagnosis of mid borderline (BB) MH with clinical and histopathological examination.&#x0D; Case presentation: A 44-year-old woman presented with red patches on her face and body 1 month ago. Madarosis and multiple erythematous plaques confluent, poorly demarcated, raised edges, atrophic center with some smooth shiny and some rough surfaces found in the facial region and superior extremities et al. bilateral inferior with bilateral auricular infiltrates. A sensory examination of the lesion showed decreased sensitivity to pain and temperature. Skin-slit smear examination showed AFB with bacterial index (IB) +2 and morphological index (IM) 30%. On histopathological examination with hematoxylin and eosin staining, Grenz zone appeared, epithelioid granuloma affecting the epidermis, no lymphocytes, and no giant Langhans cells were found, and nerves were surrounded by lymphocytes and granulomas. On fite-faraco staining, BTA was obtained with IB +3 and IM 30%. The diagnosis of MH type BB was established, and the patient was given multidrug therapy (MDT) multibacillary (MB).&#x0D; Conclusion: Morbus Hansen type BB has a typical histopathological picture of granulomas composed of mature epithelioid cells, Grenz zone, AFB, enlarged and swollen nerves surrounded by lymphocytes or infiltrated with granulomas without damage. After 3 months of therapy, there was a decrease in IB and IM on AFB examination.