Papulonodular Lesions Research Articles

Papulonodular Lesions in Photo Exposed Areas as First Manifestation of IgG4-Related Disease

IgG4-related disease (IgG4-RD) or IgG4 syndrome is a chronic inflammatory condition of presumed autoimmune etiology, affecting mainly men in their sixth decade of life. It has a good prognosis following instituted treatment and is characterized by infiltration of IgG4 + plasma cells, predominantly in the pancreas, bile duct, lymph nodes, kidneys, retroperitoneum, lungs and salivary, parotid, submandibular and lacrimal glands. Skin manifestations of IgG4 syndrome are uncommon but should be known to the dermatologist. This report details arare case of IgG4 syndrome involving cutaneous and lymph nodes. A 53-year-old woman described a 15-year history of erythematous and pruritic papulo-nodular lesions, mostly in photoexposed areas of the face, neck and upper limbs. She explained that the lesions evolved with ulceration, healing and residual dyschromia. Furthermore, she reported relapsing lymphadenomegaly 2 years ago, primarily in the cervical, axillary and inguinal chains.

Nail Disorders in Patients Living with HIV Coinfected with Monkeypox: A Clinical Study in 69 Mexican Cases

Introduction: Several infectious diseases can present nail manifestations, which may be useful for diagnosis and prognosis, and only a few reports have been made regarding monkeypox (mpox). The objective of this study was to describe the clinical characteristics of nail alterations in patients living with HIV coinfected with mpox. Methods: A prospective, cross-sectional study included patients living with HIV/AIDS, coinfected with mpox. We examined all 20 nails in search of nail plate alterations. Patients were divided into two groups, with and without nail disease, and the CD4 count was noted according to the Centers for Disease Control and Prevention (CDC) classification. A χ2 or Fisher’s exact test for qualitative variables and Mann-Whitney U and Kruskal-Wallis tests with post hoc Bonferroni test for quantitative variables were used to compare them. Data were analyzed with the SPSS Statistics 25 software. Results: Sixty-nine patients were included. The frequency of nail involvement was 58%. Papulonodular lesions were the most frequent type identified, with 21 cases (30.4%). A significant difference was observed between patients with nail disease versus median CD4 count (160 vs. 700/mm3; p = 0.002) and median HIV viral load (45,000 vs. 900/mL; p = 0.009). When comparing the characteristics of the nail lesions with the CDC Classification System for HIV infection by the CD4 count, a significant difference was observed in foot involvement, splinter hemorrhages, papulo-nodular lesions, anonychia, onychomadesis, acute paronychia, and nail bed ulcer-atrophy (p < 0.05). Conclusions: The frequency of nail lesions is high in patients living with HIV coinfected with mpox. In addition, they tend to be more destructive in patients with lower CD4 counts and higher viral loads.

Rare Skin Diseases: Turban Tumor (Brooke-Spiegler Syndrome)

The Brooke-Spiegler syndrome is a rare autosomal dominant disorder characterized by development of multiple skin tumours including multiple cylindromas and trichoepitheliomas. Here we describe a case of 68-year-old man admitted at a consultative appointment into the surgical department of Siberian State Medical University with numerous asymptomatic, skin-coloured, dense smooth papulonodular lesions from 2 to 5 mm diameter over the face and with multiple pinkish dense smooth dome-shaped nodules from 1 to 5 cm over the scalp. The patient has noted their appearance since childhood. Earlier, similar lesions have been observed in his father and grandmother. Histopathological examination classified facial lesions as trichoepitheliomas and defined scalp lesions as cylindromas, thereby confirming the diagnosis of Brooke-Spiegler syndrome.

Clinical polymorphism of zoonotic cutaneous leishmaniasis: combination of the clinical and the parasitological diagnosis.

Zoonotic cutaneous leishmaniasis (ZCL) is a neglected tropical disease caused by Leishmania (L.) major. This zoonosis is characterized by a broad-spectrum clinical polymorphism and may be underestimated and poorly treated since it is a simulator of various dermatoses. The aim of our study was to analyze the clinical polymorphism of patients with ZCL. A total of 142 patients with confirmed CL based on the microscopic examination of skin lesion biopsies were included in this study. Molecular typing of Leishmania species revealed that all patients were infected with L. major.In total, 14 clinical formswere observed. Six were typical and eight were atypical. The typical ZCL forms are grouped as follows: papular (26.76%), ulcero-crusted (26.05%), ulcerated (13.38%), impetiginous (9.86%), nodular (9.15%), and papulo-nodular (5.63%) lesions. In atypical ZCL forms, we described erythematous (2.81%), erysipeloid (1.4%), sporotrichoid, (1.4%), keratotic (0.7%) lupoid (0.7%), lichenoid (0.7%), psoriasiform (0.7%), and zosteriform (0.7%) lesions. Here, the lichenoid and the keratotic forms caused by L. major were reported for the first time in Tunisia. These findings will help physicians to be aware of the unusual lesions of ZCL that could be confused with other dermatological diseases. For this reason, it will be necessary to improve the diagnosis of CL especially in endemic areas. Such large clinical polymorphism caused by L. major may be the result of a complex association between the vector microbiota, the parasite, and the host immune state, and further studies should be carried out in order to reveal the mechanisms involved in clinical polymorphism of ZCL.

Tuberous Sclerosis Complex in a 20-year-old Female: Delayed Recognition and Life-threatening Outcomes

Tuberous sclerosis complex (TSC) is a rare, autosomal dominant multisystem disorder affecting the brain, heart, kidneys, lungs, and skin leading to significant morbidity and mortality. We report a case of TSC and highlight the need for prompt diagnosis and proper surveillance to minimize life-threatening complications. A 20-year-old female presented with facial and ungual papulonodular lesions 4 years after being diagnosed with epilepsy at the age of eight. No family history of genetic diseases was reported. Eight years later, the patient developed recurrent cough, shortness of breath, and blurring of vision. Biopsy of facial and digital nodule showed angiofibroma and ungual fibroma (Koenen tumor), respectively. Chest computed tomography scan revealed extensive cystic lesions diffusely scattered throughout the entire lung parenchyma suggestive of lymphangioleiomyomatosis. Cranial MRI revealed cortical and subependymal tubers, compatible with TSC. The patient had multidisciplinary management. However, her symptoms progressed, and she eventually succumbed to death. Cutaneous lesions such as facial angiofibromas and ungual fibromas along with multisystemic manifestations should alarm the clinician to TSC. Given its highly variable expressivity, awareness of different TSC-associated signs and symptoms is essential for prompt diagnosis, proper treatment, disease monitoring, and early recognition of TSC complications.

Aleukemic leukemia cutis: a rare case report

Leukemia cutis is a rare case disorder characterised by infiltration of neoplastic leukocytes (myeloid or lymphoid) in clinically identifiable cutaneous lesions. It can occur before the onset of hematological presentation of leukemia or during the disease course. The lesions may be highly variable ranging from flesh coloured to violaceous papules, nodules or plaques. A 52-year-old male patient came to the Dermatology Department with multiple asymptomatic erythematous papulonodular lesions involving the face, trunk as well as the extremities from the past 6 months. Systemic examination and counts were normal. On histopathological examination diffuse dense infiltrate of neutrophils and eosinophils throughout the dermis with extension of infiltrate in the interstitium of reticular dermis. Scattered amidst the infiltrate were several large cells with abundant pale cytoplasm and irregular nuclei. Immunohistochemistry was done, radiotherapy was planned and poor prognosis was explained to the patient. The case is being reported due to its rarity and the role of dermatopathologist in early diagnosis.

P18 Giant Periocular Juvenile Xanthogranulomas Treated with Trametinib

Abstract Juvenile Xanthogranuloma (JXG), the commonest type of non-Langerhans cell histiocytosis, is regarded as a benign self-limiting condition of infancy, characterised by asymptomatic yellow brown papulonodular lesions, usually confined to the skin but occasionally affecting the eyes or internal organs. Skin lesions characteristically regress spontaneously within 3-6 years. We describe an unusually aggressive cutaneous presentation requiring input from paediatric oncology. A 6 year old boy presented with extensive soft yellowish papules, plaques and nodules particularly around the eyes but also over the chest and leg. Biopsy confirmed the clinical diagnosis of JXG. The lesions enlarged progressively with new lesions appearing, causing significant distortion of the upper and lower eyelids with reduced ability to open the eyes, discomfort and distress. Treatment was started with topical steroids, progressing to intralesional and systemic steroids, but with minimal effect. In view of the severity, vinblastine and subsequently cytarabine were added, again with limited benefit. The periocular lesions were surgically debulked and he was commenced on Trametinib (a MEK inhibitor) with a good response. Now aged 9 years, and the patient displayed a favourable clinical response after 3 months of being on Trametinib. JXG rarely requires treatment, but there is no recognised therapy for the occasional problematic lesions. In this case trametinib was effective and well-tolerated. The efficacy of trametinib in histiocytosis has been attributed to the presence of activating BRAF and MAP2K1 mutations. Genetic analysis of JXG may guide future therapies.

Diagnostic challenges in cutaneous leishmaniasis due to atypical Leishmania infantum: pathologists' insights from re-emergence zones.

Leishmaniasis, a parasitic infection affecting both humans and animals, is increasingly spreading across Mediterranean and European regions, largely driven by human migration and environmental changes. In countries like Türkiye and across Europe, which have seen large influxes of migrants, the incidence of cutaneous leishmaniasis (CL) is rising, with cases now appearing in cities where the disease was previously undocumented. In these previously non-endemic areas, physicians unfamiliar with the characteristic lesions may misdiagnose CL, particularly in cases with only cutaneous manifestations. This study aims to evaluate the impact of re-emerging CL on the routine diagnostic practices of pathologists in Türkiye, by retrospectively reviewing cases. We conducted a retrospective analysis of CL cases diagnosed between 2013 and 2022 at a single pathology center in Türkiye, covering multiple provinces. Twelve cases of CL were identified and analyzed based on clinical presentation, pre-diagnosis, histopathological findings, and molecular diagnostics. DNA extraction and PCR were performed on paraffin-embedded tissue samples to identify the Leishmania species involved. Out of the twelve CL cases reviewed, seven exhibited morphological findings strongly suggestive of CL (MFSS of CL), warranting further microbiological evaluation. All patients presented with non-healing skin lesions characterized by central ulceration, crater-like formations, or papulonodular lesions. Notably, CL was included in the clinical pre-diagnosis in only 58.3% of cases, while it was not considered in the remaining 41.7% of cases. Clinicians initially pre-diagnosed skin tumors in six cases (50%), four of which led to wide surgical excision. Histopathological examination in all cases revealed chronic or mixed (acute/chronic) inflammation, predominantly rich in histiocytes. To further investigate the role of Leishmania species in the pre-diagnosis, DNA extraction and PCR were performed on paraffin-embedded tissue samples, identifying L. infantum as the causative agent in 10 cases and L. major in two cases. Notably, L. infantum was the causative agent in all five cases initially misdiagnosed as skin tumors, which were also associated with a granulomatous type of chronic inflammation.

The Unusual Sequence of Lymphomatoid Papulosis Following Hodgkin's Disease: A Case Report

Lymphomatoid papulosis (LyP) is a rare skin disease which is characterised by chronic, recurrent self-healing papulo-nodular skin lesions. It is classified under the group of primary cutaneousCD30+ve lymphoproliferative disorders. It is important to diagnose LyP as it is usually associated with other malignant lymhoproliferative disorders like mycosis fungoides, cutaneous or systemic anaplastic large cell lymphoma and Hodgkin's lymphoma. LyP associated malignancies may occur before, concurrent with or after the onset of LyP. In most of the cases previously described, Hodgkin's disease has developed after, or concurrently with, the onset of LyP. Here we report a case of LyP that developed 5 years after the onset and therapy for Hodgkin's lymphoma.

Histoplasmose africaine chez un enfant en Guinee: a propos d’un cas

African histoplasmosis is a rare deep mycosis caused by two varieties of Histoplasma capsulatum (capsulatum and duboisii). We report a case of histoplasmosis in an immunocompetent child. 6-year-old female patient, hospitalized for poor general condition, fluctuating inflammatory swelling of the lower limb evolving for 4 months. The clinical examination revealed in an altered and febrile patient, papulo-nodular lesions of variable size, fluctuating in places, disseminated umbilical predominantly on the trunk, the root of the upper and lower limbs. There were also axillary and inguinal lymphadenopathy and osteo-articular involvement. X-ray of the right lower limb revealed osteomyelitis. Histopathology of the biopsied nodule confirmed African histoplasmosis. The rarity of African histoplasmosis in our country corroborates current data from the literature, however this could also be due to an underdiagnosis often influenced by self-medication.

Zimmermann-Laband syndrome and infantile systemic hyalinosis: an enigma with two separate terms with overlapping features: a case report

BackgroundZimmermann-Laband Syndrome (ZLS) and infantile systemic hyalinosis (ISH) are rare genetic disorders. They are characterized by various spectrum manifestations. In spite of other case reports, this case with features of both syndromes was reported by oral medicine specialists and oral and maxillofacial surgeons.Case presentationIn this study, we reported an 18-months old female patient with gingival overgrowth. This phenomenon completely embedded all the erupted teeth. In this case, the presence of multiple papulonodular cutaneous lesions is a newly observed aspect that has rarely been reported in the existing literature. Gingival overgrowth was excised under general anesthesia. At six months of follow-up after surgery, mastication and breathing problems were improved. Aesthetic aspects were ameliorated in terms of gingival appearance.ConclusionsTo date, due to the ambiguous presentations, both syndromes remain an enigma for specialists. A timely diagnosis could be crucial for prognosis and preventing severe further surcharge. Dentists could play an important role in the diagnosis of rare disorders.

Annular skin tumors and lymphoproliferative diseases

Determining the shape of a skin lesion may provide a diagnostic clue in dermatology practice, more commonly for inflammatory diseases but also for skin tumors. The annular formation may develop by diverse mechanisms in skin tumors. Annular lesions may occur from the onset of the tumor as sparing the central area or depression and/or ulceration in the center of the tumor or outward expansion of the primary lesion. Clustering of multiple papulonodular lesions sparing the central area or relatively independent processes acting on the central and peripheral components of the tumor may also result in an annular appearance. We have explored a wide variety of benign and malignant skin tumors and lymphoproliferative diseases forming an annular shape.

Eyelid involvement of primary cutaneous lymphomas: A clinical perspective.

Eyelids may be affected in systemic, ocular adnexal and primary cutaneous lymphomas (PCLs). The frequency of eyelid involvement in PCLs is still not well known and it is not a predilection site for any type. While primary cutaneous T-cell lymphomas (CTCLs) are more commonly seen than primary cutaneous B-cell lymphomas (CBCLs), especially mycosis fungoides (MF) as by far the most frequent type, B cell lymphomas are reported to be the commonest type in eyelid localization on the contrary. PCLs may be located on the eyelids, as the sole manifestation or in association with the involvement of other parts of the eye and elsewhere of the body. MF may present with a rich spectrum of clinical features on the eyelids mostly seen in folliculotropic subtype and advanced-stage disease. Erythematous scaly patches or plaques representing the most commonly encountered eyelid MF lesions may mimic many other dermatological conditions. Diffuse thickening, oedema, poikilodermic changes, atrophy and wrinkling are other suggestive findings of eyelid MF. Milia-like papules, madarosis and ectropion are also seen in the folliculotropic variant of MF, as ectropion is more typical for Sezary syndrome. Eyelids are also a typical location for tumoural MF which has been suggested as a poor prognostic indicator in MF. Papulonodular lesions, large tumours, ulceration, diffuse infiltration, oedema and subcutaneous atrophy on the eyelids may also be seen in other types of PCLs. Keep in mind, the rich clinical spectrum of PCLs on the eyelids may be crucial in early diagnosis in this special localization.

Histoid leprosy complicated by erythema nodosum leprosum mimicking connective tissue disease.

Erythema nodosum leprosum (ENL) is an immunological complication of leprosy seen in 50% of lepromatous and 10% of borderline lepromatous leprosy. It usually presents as a multisystem disease with papulo-nodular skin lesions and fever. Arthralgia or arthritis is a common initial presentation of erythema nodosum leprosum. Pure rheumatologic presentation of lepromatous leprosy complicated by erythema nodosum leprosum is extremely rare, mimics connective tissue disease and is treated with steroids.

A patient with a papulo‐nodular lesion on the shoulder

A patient with a papulo‐nodular lesion on the shoulder

Multiple Nonfamilial Trichoepitheliomas: A Rare Case with Review of the Literature

Trichoepitheliomas are benign tumors of follicular origin and often appear in childhood or early adolescence. They present as small, firm papulonodular lesions of normal skin color or translucent. The lesions gradually increase in size and then stabilize. They sit electively on the face, mainly in the nasolabial folds, on the forehead, chin, and cheeks, and sometimes on the scalp and neck. Trichoepitheliomas can be divided into three subgroups: multiple familial Trichoepitheliomas, solitary non-hereditary Trichoepitheliomas, and desmoplastic Trichoepitheliomas. Nonfamilial multiple trichoepitheliomas are rarely described. We report a case of a 12-year-old child whose clinical history and clinicopathologic correlation allowed us to retain the diagnosis of multiple non-familial trichoepitheliomas.

ANNULAR SKIN TUMORS AND LYMPHOPROLIFERATIVE DISEASES.

Determining the shape of the skin lesion may provide a diagnostic clue in dermatology practice, more commonly for inflammatory diseases but also for skin tumors. The annular formation may develop by diverse mechanisms in skin tumors. Annular lesions may occur from the onset of the tumor as sparing the central area or depression and/or ulceration in the center of the tumor or outward expansion of the primary lesion. Clustering of multiple papulonodular lesions sparing the central area, or relatively independent processes acting on the central and peripheral components of the tumor may also result in an annular appearance. We have explored a wide variety of benign or malignant skin tumors and lymphoproliferative diseases forming an annular shape.

Infantile Systemic Hyalinosis: A Clinical Masquerader for Clinicians

Infantile systemic hyalinosis (ISH) is an extremely rare disorder inherited in an autosomal recessive manner. The syndrome clinically presents with multiple joint contractures with chronic severe pain, papulonodular skin lesions, hypotonia, gingival enlargement, developmental delay, and systemic manifestations. In this case report, we present the case of a 21-month-old Indian girl with ISH. This case demonstrates that ISH, though rare, should be considered in differential diagnosis in patients with subcutaneous nodules and raised lesions on the face and neck.

Does immune dysregulation contribute towards development of hypopigmentation in Indian post kala-azar dermal leishmaniasis?

Post kala-azar dermal leishmaniasis (PKDL), a sequel of apparently cured visceral leishmaniasis (VL) presents with papulonodular (polymorphic) or hypopigmented lesions (macular) and is the proposed disease reservoir. As hypopigmentation appears consistently in PKDL, especially the macular form, this study aimed to delineate immune factors that singly or in combination could contribute towards this hypopigmentation. At lesional sites, the presence of melanocytes and CD8+ T-cells was assessed by immunohistochemistry and mRNA expression of melanogenic markers (tyrosinase, tyrosinase-related protein-1 and MITF) by droplet digital PCR, while plasma levels of cytokines and chemokines were measured by a multiplex assay. In comparison with skin from healthy individuals, macular PKDL demonstrated a near total absence of Melan-A+ cells at dermal sites, while the polymorphic cases demonstrated a 3.2-fold decrease, along with a dramatic reduction in the expression of key enzymes related to the melanogenesis signalling pathway in both forms. The levels of circulating IFN-γ, IL-6, IL-2, IL-1β, TNF-α and IFN-γ-inducible chemokines (CXCL9/10/11) were elevated and was accompanied by an increased lesional infiltration of CD8+ T-cells. The proportion of CD8+ T-cells correlated strongly with plasma levels of IFN-γ (r=0.8), IL-6 (r=0.9, p < 0.05), IL-2 (r=0.7), TNF-α (r=0.9, p < 0.05) and IL-1β (r=0.7), as also with CXCL9 (r=0.5) and CXCL10 (r=0.6). Taken together, the absence/reduction in Melan-A suggested hypopigmentation in PKDL was associated with the destruction of melanocytes, following the impairment of the melanogenesis pathway. Furthermore, the presence of CD8+ T-cells and an enhanced IFN-γ-associated immune milieu suggested the generation of a pro-inflammatory landscape that facilitated melanocyte dysfunction/destruction.

Tristar Vessels: A Newly Described Dermoscopic Vessel Pattern of Miescher’s Nevus

Purpose: Although it is very common in people over 30 years old, little is known about the validity of dermoscopic criteria to differentiate Miescher’s nevus (MN) from other papulonodular lesions located on the head and neck. The aim of this study is to evaluate the dermoscopic findings in MN on the head and neck in patients over 40 years by using dermoscopy.&#x0D; Methods: In this descriptive cross-sectional study 170 MN were evaluated by using polarized light dermoscopy. frequencies of dermoscopic findings in MN were determined using Statistical Package for Social Sciences for Windows 15.0.&#x0D; Results: The most frequently detected dermoscopic pattern in MN was globular pattern (64.5%), less often homogeneous (32.4%) or cobblestone pattern (2.9%). Globules were observed in 75.3% of MN and dots were seen in 12.4%. They frequently had skin-colored (87.1%) or light-brown (49.4%) homogeneous structureless areas. Elongated comma vessels (47.1%) were the most common vascular findings. Although comma vessels (33.5%) and linear vessels (4.7%) were also observed, 11.2% of MN presented “tristar vessels” (a newly described vascular pattern) that showed regular ‘tristar like ramifications’ in the distal part of the vessels.&#x0D; Conclusions: Firstly identified “tristar vessel pattern” is a dermoscopic description of MN that can be a useful clue to differential diagnosis.