A 68-year-old female patient developed pale hyperaemic, few millimeters large confluent papules with a shiny surface forming large plaques, localized on the face, neck, trunk and the proximal part of the upper limbs. Based on the clinical symptoms, differential diagnoses of papular mucinosis, amyloidosis, interstitial granulomatous dermatitis, granuloma annulare, photocontact eczema, systemic lupus erythematosus, dermatomyositis, mycosis fungoides, and polymorphic light exanthema arose. Diagnosis of interstitial granulomatous dermatitis was made on the base of, mucin positivity in the dermis in association with granulomatous inflammation, Apart from myelodysplastic syndrome, no other underlying disease or medicinal conditions could be proven. Local powerful corticosteroid, PUVA cream therapy did not result in sufficient results. With systemic corticosteroid and acitretin, the skin symptoms improved. In this case report, the authors describe in detail the currently accepted classification of mucinoses and review the rare papular mucinosis and interstitial granulomatosus dermatitis.

As in other organs, the diagnosis of endogenous cutaneous overload diseases is based on histopathological analysis of the lesions using special stainings, even if the clinical appearance is sometimes very suggestive. The lesions are sometimes very subtle and can be included in the group of "invisible" dermatoses, such as primary macular cutaneous amyloidosis or calciphylaxis. Superficial dermal melanosis or pigmentary incontinence generally reflects the post-inflammatory stage of a chronic or recurrent interface dermatitis. Section levels should be systematically performed to look for active lesions of diagnostic interest: Alcian blue staining to identify dermal mucinosis (connectivitis) and pan-T markers (fixed pigmented erythema, lichenoid mycosis fungoides, and vitiligo). Some pathologies have a prognostic impact, either because they reflect an underlying disease, monoclonal gammopathies, in particular myeloma, being one of the most common conditions in this context (AL amyloidosis, xanthoma and xanthogranuloma, scleromyxedema), or because they can be associated with visceral damage (AL amyloidosis, scleromyxedema). The clinical-pathological comparison is mandatory to rule out differential diagnoses, especially for life-threatening diseases: nodular amyloidosis and primary cutaneous amyloidosis versus systemic AL amyloidosis, papular mucinosis versus scleromyxedema and calcific panniculitis versus calciphylaxis.

ABSTRACT HIV/acquired immunodeficiency syndrome has significant systemic implications, with dermatological manifestations often being the earliest and most visible indicators of infection. These skin conditions can severely impact patients’ quality of life and esthetic health. This review explores a broad range of dermatological changes associated with HIV, including HIV-specific dermatoses such as papular pruritic eruption, xerosis, eosinophilic folliculitis, prurigo nodularis, and papular mucinosis. It also covers common dermatoses such as seborrheic dermatitis, psoriasis, pityriasis rubra pilaris, and hidradenitis suppurativa. In addition, the review examines skin infections (fungal, bacterial, and viral), HIV-related malignancies (Kaposi’s sarcoma and cutaneous lymphomas), and antiretroviral therapy (ART)-related skin changes including exanthematous reactions and lipodystrophy. Special attention is given to the management of these conditions, which often involves a combination of topical and systemic treatments along with ART. The phenomenon of immune reconstitution inflammatory syndrome and its dermatological manifestations are also discussed, highlighting the complexities of managing skin reactions as the immune system recovers. Furthermore, the review addresses ectoparasitic infestations and skin complications arising from intravenous drug use, which are prevalent in HIV patients. Effective management of these dermatological issues is crucial not only for improving physical health but also for addressing the profound esthetic concerns that affect patients’ psychological well-being. Early recognition and appropriate treatment strategies can lead to better patient outcomes and enhanced quality of life. Advances in ART and dermatological therapies continue to improve the care provided to HIV patients, ensuring a comprehensive approach that considers both medical and esthetic aspects of their health. This review underscores the importance of a multidisciplinary approach in managing the diverse and complex skin conditions associated with HIV.

The generalized and sclerodermic form of lichen myxedematosus, known as scleromyxedema (SMX), is a chronic mucinosis that manifests cutaneously and has multiple systemic comorbidities. There are few available treatment options and no established therapeutic guidelines. We describe a 48-year-old man who had intravenous immunoglobulins (IVIg), oral corticosteroids, and methotrexate (MTX) for the treatment of SMX, monoclonal gammopathy, and arthritis. Because of its effectiveness and high level of tolerance, IVIg is the most often used first-line therapy for SMX and has been used for an increasing range of skin conditions. In our instance, better control of skin disease and extracutaneous manifestations was made possible by combining IVIg with oral prednisone and MTX. To the best of our knowledge, this is the first instance of SMX treatment that has combined therapeutic approaches with a favorable safety profile.

Although rare, small lymphocytic lymphoma can present as chronic lip swelling and papules, thus mimicking the features of orofacial granulomatosis, a chronic inflammatory disorder characterized by subepithelial noncaseating granulomas, or papular mucinosis, characterized by localized dermal mucin deposition of mucin. When assessing lip swelling, one must carefully consider the clinical clues and have a low threshold to perform a diagnostic tissue biopsy, preventing delays in treatment or progression of the lymphoma.

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.

Background: Papular mucinosis is a variety of mucinosis characterized by excessive production of mucin by fibroblasts and deposition in the dermis. It manifests itself with fleshy papules or plaques in different sites of the body and taking different clinical morphological cutaneous features. Objective: The objective is to report and evaluate the different clinical and histopathological features of the disease in Iraqi patients. Patients and Methods: This is a case series and a clinical descriptive study in which ten patients with papular mucinosis were reported during the period from 2012 through 2019. The age ranged from 4 to 56 years, with seven females and three males. Clinical evaluation regarding histories of the disease and examination was carried out. General investigation was done and skin biopsy for histopathological assessment was conducted. Results: Nine patients were adults, with their age ranging from 20 to 56 years, a mean of around 35 years, and only one 4-year-old child. It is a disease with a female predominance, as observed in 7 (70%) females. The common sites of involvement were the face but the rash may extend to affect the neck and upper arms. The rash appeared in the form of skin-colored or red fleshy papules and plaques or in diffuse erythematous orange peel-like forms. The rash was asymptomatic in most patients. The pathology of the disease clarified the diffuse deposition of mucin in the dermis, as was demonstrated with H&E staining. Conclusion: Papular mucinosis is a rare disease characterized by mucin deposition in the skin affecting mostly adult females. The face is commonly involved together with other areas such as the neck and upper arms, but the trunk and lower limbs are spared. It manifests itself with different clinical morphological cutaneous features. Papular granuloma annulare must be considered as an important differential diagnosis in all cases of papular mucinosis.

Scleromyxedema (SME) is a rare mucinosis associated with monoclonal gammopathy. Several biochemical peculiarities of monoclonal immunoglobulins (Ig) in SME patients were reported in case reports or short series, such as IgGλ over-representation, cationic migration, and partial deletion. Monoclonal immunoglobulins (Ig) from the serum of 12 consecutive patients diagnosed with scleromyxedema (SME) were analyzed using electrophoretic and immunoblotting techniques. All monoclonal Ig from 12 SME were of IgG1 subclass, with an overrepresentation of the lambda-type light chain and a cationic mobility on standard zone electrophoresis, as compared with 21 cases of monoclonal gammopathy of undetermined significance (MGUS) of IgG1 subclass. Reactivity with specific monoclonal antibodies demonstrated no evident deletion of the heavy chain constant domains, which was also confirmed by analysis of Ig heavy chain molecular weight on a purified monoclonal component from one case. Significant isotype restriction of both heavy and light chains, and peculiar biochemical properties suggest that monoclonal Ig might be involved in pathophysiological events of SME.

Acral persistent papular mucinosis

ObjectivesWe describe a case of systemic sclerosis (SS) with acro-osteolysis associated with cutaneous mucinosis, usually characterized by mucin deposition in the skin. The main differential diagnosis was multicentric reticulohistiocytosis due to the presentation of papulonodular skin lesions.Materials and methodsA physical examination, imaging studies and laboratory tests were performed.ResultsDistal bone resorption was evident on plain radiographs, and skin biopsy confirmed mucinosis. The SS diagnosis was based on the clinical features, high levels of antinucleolar antibodies and typical nailfold capillaroscopy findings.ConclusionTo the best of our knowledge, this is the first description of cutaneous mucinosis accompanying SS with acro-osteolysis.LEARNING POINTSCutaneous mucinosis is rarely associated with systemic sclerosis.Systemic sclerosis with cutaneous mucinosis may resemble multicentric reticulohistiocytosis.

Scleromyxedema (SMX) is a rare systemic sclerosis mimic that often responds to intravenous immunoglobulin (IVIG) therapy, yet the resulting clinical and biochemical changes have not been well characterized. To better understand the pathogenesis of the disease and the efficacy of IVIG, we sought to explore whether IVIG would introduce a measurable biologic effect corresponding with clinical improvement. Fifteen patients with SMX were recruited for the study. Clinical information and peripheral blood mononuclear cells for flow cytometry were obtained immediately before and again 1-2 weeks after patients received IVIG therapy. Ten patients also underwent skin biopsies for gene expression analysis both before and after IVIG therapy. Clinical data included measures of skin involvement (modification of the modified Rodnan skin thickness score [MMRSS] and percentage of body surface area) and several patient-reported outcome measures assessing patients' skin. Posttreatment, the average MMRSS score decreased from mean ± SD 13.6 ± 2.6 to 10.3 ± 1.9; P = 0.003. There were also significant improvements in skin flexibility (mean ± SD 5.4 ± 0.8 to 3.2 ± 0.7; P = 0.003) and softening (mean ± SD 4.9 ± 0.9 to 2.6 ± 0.6; P = 0.022). Baseline levels of Tc17 cells (CD8+CCR6+CXCR3+CCR4-) correlated with the extent of skin involvement as measured by MMRSS pretreatment (r = 0.69, P = 0.012) and decreased after IVIG therapy (mean ± SD 3.4% ± 3.2% to 1.3% ± 1.7%; P = 0.008). Posttreatment analysis of RNA in skin tissue revealed a decrease in gene expression of transforming growth factor β (TGFβ) cytokines as well as several interferon-inducible proteins. This open-label study further supports the evidence that patients with SMX respond both objectively and subjectively to IVIG therapy. Biologic studies suggest a role for T lymphocytes in the pathogenesis of the disease and reveal the potential significance of TGFβ and interferon pathways.

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.

Atypical scleromyxedema improved by immunoglobulins intravenous

Scleromyxedema (SM) was previously known to be associated with monoclonal gammopathy. The association of SM and its counterpart lichen myxedematosus (LM) with chronic hepatitis has rarely been reported. We retrospectively reviewed medical records and histopathological reports of consecutive patients who presented at our department with the diagnosis of SM or LM from January 2001 to September 2017. The patients' demographic details, cutaneous presentation, associated underlying diseases and hepatitic profile were studied and compared with previous published cases. In all, 28 patients were enrolled, including one SM, 19 LM and eight atypical LM. Of the patients, 50% (n=14/28) had hepatitis. Of these, 21.4% (n=6/28) had hepatitis C, 10.7% (n=3/28) hepatitis B, 7.1% (n=2/28) concurrent hepatitis B and C, whereas 10.7% (n=3/28) had alcoholic liver disease. The prevalence of hepatitis C in our patients was 6.5-times higher than that of the general population (28.6% vs 4.4%) and the prevalence of hepatitis B was similar (17.9% vs 17.3%). Polyclonal gammopathy was found in 28.6% (n=8/28) of the patients and monoclonal gammopathy was found in 7.1% (n=2/28). The extent of clonality did not correlate with disease severity. Our study did not notice a significant association with monoclonal gammopathy but the prevalence of hepatitis C was found to increase 6.5-times in these patients compared with the general population. We recommend dermatologists to be aware of hepatitis investigations in such patients and future studies are warranted to understand the mechanism behind such association.

Scleromyxedema: An advanced presentation in a patient with MGUS

Papular mucinosis: A report of two cases

A case of scleromyxedema complicated by monoclonal plasma cell proliferation

Scleromyxedema (SM) is a rare disorder which initially presents with waxy skin stiffness and maculopapular lesions. It also has non-dermatologic manifestations, such as serum paraproteinemia and myopathies, and is sometimes associated with other autoimmune disorders. A 52-year-old man was referred for treatment because of torso-dominant skin stiffness. He also had a neglected history of bilateral inflammatory back pain and chronic Helicobacter pylori-negative gastritis. Skin histopathology confirmed a diagnosis of SM. A grade 4 bilateral sacroilitis and a positive human leukocyte antigen B27 led to the patient also being diagnosed with ankylosing spondylitis (AS). Upon further analysis, monoclonal gammopathy of immunoglobulin (Ig) G/kappa (lower than 3 g/dl) and a normal percentage of plasma cells in his bone marrow aspiration sample were discovered. Due to the patient’s IgA deficiency, intravenous immunoglobulin (IVIG) could not be used to treat his SM; due to his positive tuberculin skin test (25 mm) and history of gastritis, anti-tumor necrosis factor alpha and non-steroid anti-inflammatory drugs were also withheld. The patient received a drug regimen of cyclosporine (3mg/kg/day) and high-dose prednisolone (0.5mg/kg/day) which successfully controlled both his SM and AS disorders. In this paper, we report a previously unreported case of SM-associated gammopathy and AS. We also show the efficacy of cyclosporine and high-dose prednisolone in the treatment of both of these conditions.

Systemic sclerosis is usually considered to be the main cause of cutaneous sclerosis, but many other diseases can also manifest as cutaneous sclerosis and tissue fibrosis in clinic, which are easy to be confused with systemic sclerosis. This kind of diseases include a wide spectrum of heterogeneous diseases, ranging from a benign disease with a localized cutaneous involvement, to a widespread systemic, life-threatening disease. This review summarizes several main kinds of cutaneous fibrosing disorders, such as nephrogenic fibrosing dermopathy, scleromyxedema, POEMS syndrome, myeloma, Buschke scleroderma, eosinophilic fasciitis, eosinophilia-myalgia syndrome, carcinoid syndrome, porphyria cutanea tarda, chronic graft-versus host disease, environmentally triggered scleroderma-like disorders. Distinguishing the pathogenesis, clinical and pathological manifestations and therapeutic methods between this group of diseases will improve the understanding of these diseases, and has great clinical significance for their correct diagnosis and proper treatment. Key words: Scleroderma, systemic; Patient simulation; Pathology; Diagnosis; Therapeutic uses

Acral persistent papular mucinosis (APPM): Dermoscopy of an uncommon disease