Characteristic Cutaneous Lesions Research Articles

Upper Limb Compartment Syndrome-An Extremely Rare Life-Threatening Complication of Cutaneous Anthrax.

(1) Background: Cutaneous anthrax is a disease caused by a Gram-positive bacillus, spore-forming Bacillus anthracis (BA). Cutaneous anthrax accounts for 95% of all anthrax cases, with mortality between 10-40% in untreated forms. The most feared complication, which can be life-threatening and is rarely encountered and described in the literature, is compartment syndrome. (2) Methods: We report a series of six cases of cutaneous anthrax from the same endemic area. In two of the cases, the disease was complicated by compartment syndrome. The systematic review was conducted according to systematic review guidelines, and the PubMed, Google Scholar, and Web of Science databases were searched for publications from 1 January 2008 to 31 December 2023. The keywords used were: "cutaneous anthrax" and "compartment syndrome by cutaneous anthrax". (3) Results: For compartment syndrome, emergency surgical intervention for decompression was required, along with another three surgeries, with hospitalization between 21 and 23 days. In the systematic review, among the 37 articles, 29 did not contain cases focusing on compartment syndrome of the thoracic limb in cutaneous anthrax. The results were included in a Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) flow diagram. (4) Conclusions: Early recognition of the characteristic cutaneous lesions and compartment syndrome with early initiation of antibiotics and urgent surgical treatment is the lifesaving solution.

AIDS-related disseminated Kaposi Sarcoma: a case report

Kaposi sarcoma (KS) is the most common malignant tumor in HIV-infected individuals. Although its most common form consists of skin and mucosal lesions, it can also present itself in a disseminated way affecting the gastrointestinal tract, lungs and eye structures. We present the case of a 27-year-old male patient with HIV infection and disseminated Kaposi sarcoma. Disseminated KS is an uncommon form of the disease. Therefore, it is important to take this into account, particularly in HIV-infected individuals with characteristic cutaneous lesions.

Cutaneous polyarteritis nodosa diagnosis and treatment: A retrospective case series

To the Editor: Cutaneous polyarteritis nodosa (CPAN) is a small- to medium-vessel vasculitis with manifestations including subcutaneous nodules, livedo reticularis, ulcers, and purpura.1,2 It is diagnosed via characteristic cutaneous lesions, absence of systemic vasculitic features, and supporting histopathologic findings.1-3 There are limited data to guide the management of CPAN. We reviewed CPAN diagnosis and treatment at our center.

Occult insulinoma with treatment refractory, severe hypoglycaemia in multiple endocrine neoplasia type 1 syndrome; difficulties faced during diagnosis, localization and management; a case report

BackgroundMultiple endocrine neoplasia type 1 (MEN 1) syndrome is a rare, complex genetic disorder characterized by increased predisposition to tumorigenesis in multiple endocrine and non-endocrine tissues. Diagnosis and management of MEN 1 syndrome is challenging due to its vast heterogeneity in clinical presentation.Case presentationA 23-year-old female, previously diagnosed with Polycystic Ovarian Syndrome (PCOS) and pituitary microprolactinoma presented with drowsiness,confusion and profuse sweating developing over a period of one day. It was preceded by fluctuating, hallucinatory behavior for two weeks duration. There was recent increase in appetite with significant weight gain. There was no fever, seizures or symptoms suggestive of meningism. Her Body mass index(BMI) was 32 kg/m2.She had signs of hyperandrogenism. Multiple cutaneous collagenomas were noted on anterior chest and abdominal wall. Her Glasgow Coma Scale was 9/15. Pupils were sluggishly reactive to light. Tendon reflexes were exaggerated with up going planter reflexes. Moderate hepatomegaly was present. Rest of the clinical examination was normal. Laboratory evaluation confirmed endogenous hyperinsulinaemic hypoglycaemia suggestive of an insulinoma. Hypercalcemia with elevated parathyroid hormone level suggested a parathyroid adenoma. Presence of insulinoma, primary hyperparathyroidism and pituitary microadenoma, in 3rd decade of life with characteristic cutaneous tumours was suggestive of a clinical diagnosis of MEN 1 syndrome. Recurrent, severe hypoglycaemia complicated with hypoglycaemic encephalopathy refractory to continuous, parenteral glucose supplementation and optimal pharmacotherapy complicated the clinical course. Insulinoma was localized with selective arterial calcium stimulation test. Distal pancreatectomy and four gland parathyroidectomy was performed leading to resolution of symptoms.ConclusionsRenal calculi or characteristic cutaneous lesions might be the only forewarning clinical manifestations of an undiagnosed MEN 1 syndrome impending a life-threatening presentation. Comprehensive management of MEN 1 syndrome requires multi-disciplinary approach with advanced imaging modalities, advanced surgical procedures and long-term follow up due to its heterogeneous presentation and the varying severity depending on the disease phenotype.

Subacute cutaneous lupus erythematosus, and positive anti-MDA5 antibody in clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease: A case report and literature review

BackgroundClinically amyopathic dermatomyositis (CADM) is a subtype of DM with characteristic cutaneous lesion with normal creatinine kinase levels. Presence of anti-melanoma differentiation-associated gene 5 (MDA5) antibody is found to be associated with rapidly progressive interstitial lung disease (RP-ILD). Aim of the work: to report a CADM patient with positive anti-MDA5 antibody and RP-ILD with clinical features of systemic lupus erythematosus (SLE) who clinically responded to a combination of cyclophosphamide and other conventional immunosuppressant. Case presentation: A 44-year-old Indian woman presented with classical cutaneous lesions of DM with normal creatinine kinase levels amongst other clinical features. She was initially diagnosed with SLE before developing RP-ILD and a positive MDA5 antibody. Anti-nuclear antibody, anti-dsDNA and anti-Sm antibody were negative. Serum ferritin level was very high (1599 ng/mL) as compared to C-reactive protein (23.4 mg/L). Anti-Ro-52 and anti-PM-Scl 75 were positive. High resolution computed tomography (HRCT) of the lungs revealed features consistent with ILD. Histology of her skin biopsy was consistent with subacute cutaneous lupus erythematosus (SCLE). Her diagnosis was revised to CADM with overlapping SCLE. She responded to a combination of hydroxychloroquine, cyclosporine-A, mycophenolate mofetil, pulse methylprednisolone and pulse cyclophosphamide 750 mg/month for 6 months. Her cutaneous lesions gradually improved with normalization of serum ferritin level. Repeated HRCT showed no further progression of the pulmonary fibrosis. Conclusion: CADM with positive anti-MDA5 antibody associated with RP-ILD is rare with a high mortality rate. Early recognition and prompt treatment with a combination of immuno-suppressant may improve the outcome of this complex disease.

Ocular manifestations and surgical treatment of the linear nevus sebaceous syndrome

Objective: To report the clinical and pathological features and surgical treatment of the linear nevus sebaceous syndrome. Methods: It was a retrospective case series study. The clinical records of 11 patients (14 eyes) who were diagnosed as linear nevus sebaceous syndrome between April 2009 and February 2018 at Beijing Tongren Hospital were analyzed. Data collected included sex, age, clinical manifestations, disease site, pathological features, surgical treatment and therapeutic effects. Results: There were six males and five females, aged 6 months to 7 years (mean, 2.8 years). The nevus sebaceous was located at the head (9 patients), face (5 patients) and neck (3 patients). The most common ocular manifestations were blepharocoloboma (13 eyes) and external ocular mass (9 eyes). Surgical treatment was performed in 9 patients (11 eyes), including lid reconstruction in all these eyes, corneal and conjunctival mass resection in four patients (4 eyes), eyelid tumor resection in 5 patients (5 eyes), and symblepharon separation and conjunctival sac reconstruction in 3 patients (5 eyes). Pathological examinations of the external ocular, corneal and conjunctival masses (6 eyes) confirmed the diagnosis of complex choristoma. All operated patients had improved appearance. Conclusions: Linear nevus sebaceous syndrome has characteristic cutaneous lesions and ocular manifestations. Histopathologically, all epibulbar lesions prove to be complex choristomas. The appearance of patients with linear nevus sebaceous syndrome can be significantly improved by oculoplastic surgery. (Chin J Ophthalmol, 2020, 56: 846-852).

Cutaneous Larva Migrans Infestation Over Buttocks and Perineal Region: A Case Series of Five Toddlers From Sri Lanka and Literature Review

Cutaneous larva migrans (CLM) is a cutaneous infestation caused by a hookworm larva. We report five toddlers who presented to the pediatric clinic with characteristic cutaneous lesions of CLM over the buttock and perianal region over periods of variable duration. Lesions of four children were typical and linear and one child had an atypical lesion. All were diagnosed as cutaneous larva migrans based on clinical history and examination. Complete recovery in all five children was achieved following treatment with oral albendazole.

Newborn Girl with Rapidly Progressive Linearly Distributed Vesicles and Pustules

One-day old infant girl, born at term, presents with blanching erythematous macules, vesicles and pustules scattered on her upper and lower extremities. These lesions progressed rapidly and began to coalesce and take on a linear configuration, even appearing on her trunk and face. A punch biopsy revealed eosinophilic spongiosis. Based on skin findings and histopathology this girl was diagnosed with Incontinentia pigmenti (IP) in accordance with the IP Diagnostic Criteria 2013. IP is a rare, X linked dominant genetic syndrome occurring in 1 of 50,000 births. IP presents with characteristic cutaneous lesions and in varying frequency, extracutaneous manifestations. The cutaneous manifestations appear in four progressive stages. The first stage consists of multifocal and coalescing erythematous macules and patches with overlying tense vesicles on the patient’s trunk and extremities. These lesions are distributed along Blashko lines. In stage two, the lesions become hyperkeratotic appearing similar to viral warts. The lesions in the third stage darken and become hyperpigmented. They may appear as swirled macules. In the final stage hypopigmentation of the lesion occurs. Cutaneous symptoms often do not require additional medical care. Extracutaneous findings include dental, neurologic, and ocular anomalies. Requiring increased frequency of follow up by specialists during early childhood.

Incontinentia pigmenti – a description of three cases

Incontinentia pigmenti (IP) or the Bloch-Sulzberger syndrome is a rare genetic dermatosis that occurs in females, which is classified as an ectodermal dysplasia. Papulovesicular eruptions appear on the skin in the first days of a patient’s life and then, with the lapse of time they become verrucous and hyperpigmentation lesions evolving into incontinentia pigmenti achromians. Apart from such characteristic cutaneous lesions, female patients suffering from incontinentia pigmenti may also present other cutaneous symptoms, as well as motor system and ophthalmological dysfunctions. In 54 per cent of patients dental symptoms are observed, those including mainly hypodontia, pegged teeth and delayed tooth eruption. <b>Aim.</b> The aim of this study was to describe three cases of female patients diagnosed with the IP syndrome who were being treated in the Department of Orthodontics of the Medical University of Warsaw. <b>Case descriptions.</b> Descriptions of three cases of female patients with diagnosed IP were presented. The patients had cutaneous lesions typical for IP. Medical interviews and physical examinations were conducted with them all; radiographs were performed and diagnostic casts were made. Class III malocclusions were diagnosed as well as dental abnormalities characteristic for IP (hypodontia, malformation of teeth). Conclusions. An early diagnosis allows for a child to receive a multispecialist medical and dental care. An interdisciplinary approach makes it possible to reach, functionally and esthetically, satisfactory results in the treatment and maintain patients’ teeth in good health until the period of adultness thus ensuring that the physical and mental state of female patients affected by the syndrome improves.

Blue Rubber Bleb Nevus Syndrome

AbstractBlue rubber bleb nevus syndrome (BRBNS) is a rare congenital disorder characterized by multifocal venous malformations mainly of the skin, soft tissue, and gastrointestinal tract. However, it may occur in any tissue including the nervous system. This syndrome most commonly occurs sporadically but can be associated with an autosomal dominant inheritance. Among the cases reported in the literature, the female/male ratio was ∼1:1. The diagnosis of BRBNS is based on the presence of characteristic cutaneous lesions with or without gastrointestinal bleeding and/or the involvement of other organs. Typical skin manifestations consist of soft, easily compressive, bluish papules similar to rubber-like nipples. Aside the skin, vascular lesions are usually found in the gastrointestinal tract, anywhere from the oral to the anal mucosa, but predominantly in the small bowel. Endoscopy provides the opportunity to treat and diagnose the lesions. BRBNS should be differentiated from hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu syndrome), Klippel–Trenaunay syndrome, and Maffucci syndrome. The prognosis of BRBNS depends on which organs are involved and the extent of involvement. Most patients can live a long life with the disease, but the quality of life is limited due to gastrointestinal bleeding, oral drug therapy, and blood transfusions. Sudden massive gastrointestinal hemorrhage remains the most frequent cause of death. No curative therapy is currently available for this syndrome. The cutaneous lesions are usually asymptomatic and do not require treatment. The most important clinical problem is the management of acute or chronic bleeding from the multiple gastrointestinal venous malformations: a conservative approach should be instituted whenever the clinical features and the bleeding episodes are mild and only in a life-threatening situation surgery may be required. In the recent years, however, molecular targeted therapy with the mammalian target of rapamycin inhibitor sirolimus has been anecdotally employed with reduction in bleeding and shrinkage of vascular malformations.

Cutaneous manifestation of reactive arthritis: Case report

Abstract Introduction Reactive arthritis (ReA) is one of the forms of seronegative spondyloarthropathies. The difficulties in the diagnosis of reactive arthritis, despite a complex clinical picture, result from the lack of unequivocal diagnostic criteria, especially in the initial period. Diverse clinical manifestation of ReA may require the cooperation of many specialists. Aim The case of a 59-year-old man with reactive arthritis caused by an acute Yersinia enterocolitica infection 3 weeks before was described. Case study A 59-year-old patient, so far healthy, was admitted due to fever for a few days, pain and swelling of crurotalar joints, pain in the left part of the lumbosacral region, escalating at night, and additional complaints impeding urinating. Joint involvement was accompanied by numerous cutaneous and mucosal lesions. Results and discussion In this case the presence of characteristic cutaneous symptoms as keratoderma blenorrhagicum and balanitis circinata allowed to identify the disease quickly, despite a short course of the disease. The presence of antibodies of Y. enterocolitica IgA was noticed, without the presence of IgG and IgM. The presence of HLA B27 antigen was positive. In this case, the occurrence of many characteristic cutaneous lesions enabled a quick identification of the disease, despite the difficulties with determining the etiological factor. Conclusions The diagnosis of ReA is clinical, based on the history and physical examination findings. A high index of suspicion is required because no laboratory tests, markers or imaging finding allow diagnosing of ReA. The most important is proper cooperation between a rheumatologist and a dermatologist.

Gardner Syndrome with Extra and Intra-Abdominal Desmoid Tumors and Adrenal Involvement: PET/CT Findings

Gardner syndrome, also known as familial colorectal polyposis, is an autosomal dominant disease and is part of the spectrum of familial adenomatous polyposis (FAP) syndromes. It is characterized by the presence of multiple polyps in the colon together with neoplasms outside the colon. Gardner syndrome is also associated with desmoid tumors in approximately 15% of affected individuals. Desmoid tumors are rare benign slow-growing neoplasms, arising from connective tissue (musculoaponeurotic fibrous tissue). Due to their locally aggressive nature, the term aggressive fibromatosis have been used to describe these tumors. Seven percent of patients with FAP or its variants may also have adrenal masses, most being benign and asymptomatic. We report the PET/CT of a 42 year old patient who was diagnosed with Gardner syndrome, demonstrating the rare coexistence of the intestinal polyps, extra and intra-abdominal desmoids and adrenal adenoma by PET/CT. In addition, the characteristic cutaneous lesions and osteomas of the skull were also identified.

Cutaneous plasmacytosis: a clinicopathologic study of 12 cases in Taiwan revealing heterogeneous underlying causes.

Cutaneous plasmacytosis is clinically characterized by multiple pigmented papules and plaques that occur mainly on the trunk and many plasma cells in the lesional skin. Most of the cases reported have occurred in Japan. Whether cutaneous plasmacytosis is a unique reactive disease or a neoplastic condition has not been established. We collected 12 cases fulfilling the criteria for a diagnosis of cutaneous plasmacytosis at our institution from 2001 to 2013. We analyzed clinicopathologic features and performed immunohistochemical and immunoglobulin gene rearrangement studies. All 12 patients presented with characteristic cutaneous lesions mainly on the trunk and proximal extremities. Many plasma cells were observed in the lesional skin, and the basal layer was hyperpigmented. Four patients had numerous plasma cells in lymph nodes, and one patient also had increased plasma cells in bone marrow. One patient fulfilled the criteria for immunoglobulin G4 (IgG4)-related lymphadenopathy. Elevated serum IgG and IgG4 concentrations were found in seven patients. Monoclonal immunoglobulin gene rearrangements were detected in four patients. The median duration of follow-up was four years. One patient was lost from follow-up. Partial remission of the cutaneous lesions was observed in five patients, and progressive disease was noted in five patients. One patient died of respiratory failure. Our study suggests that the so-called "cutaneous plasmacytosis" has heterogeneous underlying causes with or without systemic involvement and may be associated with clonal immunoglobulin gene rearrangements and IgG4-related lymphadenopathy. No effective treatment is available for this condition.

Fixed drug eruption to quinine: a case report and review of the literature

Fixed drug eruptions (FDE) are characteristic cutaneous lesions that occur in an individual sensitized to a specific drug and may appear with more rapid onset (a few hours) upon re-exposure.1 They account for roughly 10% of cutaneous adverse drug reactions and may occur at any site but have a predilection for hands, lips, and genitalia, and last 1 to 3 weeks after cessation of the offending drug. Lesions are often sharply demarcated and usually appear as slightly raised erythematous plaques, although other forms have been reported.

A characteristic cutaneous lesion over the sting site of the wasp, Vespa tropica

A characteristic cutaneous lesion over the sting site of the wasp, Vespa tropica

Lipoid proteinosis with bilateral amygdalae calcifications, headache, and cognitive impairments

Lipoid proteinosis (LP) is a systemic autosomal recessive disorder caused by mutations in the ECM1 (extracellular matrix protein 1) gene1 and is occasionally associated with cognitive impairment, headache, and temporal lobe epilepsy.2 A 37-year-old woman with characteristic cutaneous lesions (figure 1), bilateral cataracts, and lens subluxation was evaluated for diffuse cognitive impairment and headache. Neuroimaging revealed bilateral amygdaloid calcifications typical for LP (figure 2). Sequencing the ECM1 gene identified homozygosity for a splice-site mutation, c.195 + 1G > C in intron 1. This case illustrates the various clinical manifestations of LP (none suggestive of amygdala involvement), which should be considered in the differential diagnosis of cerebral calcifications.

Ecthyma Gangrenosum in a Previously Healthy Infant

Ecthyma Gangrenosum in a Previously Healthy Infant

Erysipelothrix rhusiopathiae Isolates Recovered from Fish, a Harbour Seal (Phoca vitulina) and the Marine Environment are Capable of Inducing Characteristic Cutaneous Lesions in Pigs

In order to determine the diversity and pathogenicity of Erysipelothrix spp. isolates recovered from marine fish, a harbour seal (Phoca vitulina) and the marine environment, 14 isolates were characterized by genotyping, serotyping, determination of the surface protective antigen (spa) gene type and assessment of virulence in a pig bioassay. All 14 isolates were Erysipelothrix rhusiopathiae. Isolates were determined to be of serotypes 2 (n = 3), 3 (n = 1), 4 (n = 1), 12 (n = 1), 15 (n = 1) or 21 (n = 6), and one isolate cross-reacted with serotypes 5 and 21. The spa gene analysis determined that 64.3% (n = 9) were spaA and 35.7% (n = 5) were spaB1. In pigs, 10/14 isolates induced small plaques to diamond-shaped cutaneous lesions consistent with Erysipelothrix spp. infection. The results of this study indicate that the marine E. rhusiopathiae isolates have greater genetic and antigenic diversity than pig isolates and are capable of inducing classical skin lesions in pigs.

Erythema multiforme major following treatment with infliximab

The growth in the use of anti-tumor necrosis factor α (TNF-α) agents for treatment of inflammatory conditions has led to increased recognition of the side effects associated with this class of drugs. We report a case of a patient who developed erythema multiforme (EM) major with characteristic oral and cutaneous lesions following treatment with the anti-TNF-α medication infliximab therapy for Crohn's disease (CD). To our knowledge, this is the first reported case of infliximab-induced EM secondary to the treatment of CD. It is important for dental clinicians evaluating patients using anti-TNF-α agents to be aware of this possible complication.

Blue Rubber Bleb Nevus Syndrome: Successful Treatment With Sirolimus

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old girl with diagnosed BRBNS who had multiple venous malformations all over her body, importantly, throughout the gastrointestinal tract, mouth, esophagus, stomach, small bowel, and colon. She presented with recurrent massive gastrointestinal bleeding and soft tissue hematoma despite prednisolone and α-interferon therapy. We started low-dose sirolimus as an antiangiogenic agent. The vascular masses were reduced rapidly and there was no gastrointestinal bleeding and muscular hematoma after sirolimus therapy. There was no drug adverse reaction at 20-month follow-up. To the best of our knowledge, this is the first report related to the use of sirolimus in a patient with BRBNS.