Erythroderma Research Articles

1040 RESOLUTION OF EXFOLIATIVE DERMATITIS AND ALOPECIA AREATA OPHIASIS WITH THE TREATMENT OF OBSTRUCTIVE SLEEP APNEA

The comorbidity of atopic dermatitis and obstructive sleep apnea can have a reciprocal improvement with the use of continuous positive airway pressure. Patient is a 17 year old African American female with a history of severe exfoliative dermatitis exacerbations secondary to uncontrolled atopic dermatitis (AD) with eczema herpeticum who presented with dermatopathic lymphadenitis, alopecia areata ophiasis, night sweats, nocturia, habitual snoring with hypertrophy of the tonsils to the sleep medicine department for her snoring and nocturnal sweating. Night time sweats produced an intense pruritic response. Nocturia occurred four times a night. Autoimmune and infectious disease work ups were negative. Skin and lymph node biopsies revealed dermatopathic lymphadenitis secondary to exfoliative dermatitis. Treatments included avoidance of suspected AD triggers, emollients, hypo-allergenic soaps and topical steroids. The application of topical steroids resulted in exfoliative dermatitis with eczema herpeticum and staph infections multiple times. Gabapentin improved her pruritic condition and a slight improvement in her skin and scalp was noted. A sleep study revealed the patient to have an Apnea-Hypopnea index of 16.4, excessive sweating and severe snoring were also noted on her sleep study. The mother of the patient declined the use of immune modulating therapies including topical steroids, methotrexate, cyclosporine, and tacrolimus as well as the recommendation for a tonsillectomy. Patient and mother agreed to the use of CPAP and photographing the progression of the disease. No change in the patient’s medications nor consistency were noted. After 10 months of excellent CPAP compliance, photographical evidence as well as physical examination revealed the patient’s hair returned as well as her an improvement in her skin was noted. Nocturia resolved after the use of CPAP. Patient reported two weeks of not using her CPAP machine and reported a new onset of hair loss and worsening of eczema. The use of continuous positive airway pressure has the potential for reducing inflammatory responses in the skin for patients suffering from atopic dermatitis as well as obstructive sleep apnea, reducing the need for immune modulating therapies.

A previously undescribed cutaneous paraneoplastic syndrome in a cat with thymoma

Exfoliative dermatitis is a well-recognized cutaneous paraneoplastic syndrome (PNS) associated with thymoma in cats, of which the clinical and histopathological presentation has been well-characterized. To describe a novel clinical skin manifestation associated with thymoma in a cat. A 14-year-old neutered female domestic short hair cat. Physical, abdominal ultrasonographic, thoracic radiographic, ultrasonographic and computed tomographic examinations, histopathological assessment of the skin and mediastinal mass. The cat was presented with noninflammatory alopecia, with a dorsal multifocal distribution. Examination of the alopecic areas using a dermascope indicated an apparent lack of follicular ostia. Histopathological assessment of alopecic areas confirmed follicular and epidermal atrophy, trichilemmal keratinization and mild orthokeratotic hyperkeratosis. Diagnostic imaging revealed a mediastinal mass, which was surgically removed. Histopathological and immunohistopathological examination of the mass was consistent with a thymoma, associated with multiloculated cyst formation and multifocal cholesterol granulomas. Following surgery, hair re-growth was noted in the previously alopecic areas. The cat was euthanized 3.5months later because of recurrent chylothorax suspected to be a postoperative complication. The alopecic lesions had improved markedly. Thymoma-associated PNS might not always manifest as an exfoliative dermatitis and should be considered in the differential diagnosis of multifocal noninflammatory alopecia.

Thymoma associated exfoliative dermatitis in a rabbit with hepatic cirrhosis

A 6.5-year-old, male, non-castrated pet rabbit was presented with generalized exfoliative dermatitis, depression and anorexia. Skin laboratory examinations were negative for ectoparasites, bacteria and fungi whereas thoracic radiography revealed a large mediastinal mass. Complete blood and biochemical examinations did not show anything abnormal. Thymoma was suspected and therapeutic options were discussed but the owner declined. Supportive and symptomatic therapy was of no avail and severe deterioration of systemic symptoms necessitated euthanasia. Necropsy revealed a whitish-gray mass on the anterior and lower part of the mediastinum and pale voluminous liver. Histopathology confirmed the presence of thymoma and the hypothesis of thymoma-associated exfoliative dermatitis along with hepatic cirrhosis.

Idiopathic Generalized Exfoliative Dermatitis and Association with Antihypertensive Drugs and Statins: A Retrospective Case-Control Study

Background/Aims: We aim to examine the hypothesis that antihypertensive drugs and statins may be responsible for the development of idiopathic generalized exfoliative dermatitis (GED) with a case-control study. Methods: All inpatients who were hospitalized under the dermatology service at the Tan Tock Seng Hospital, Singapore, between 1 May 2013 and 31 May 2015, were analysed. Idiopathic GED cases had consistent clinical and histological features but no apparent cause despite comprehensive evaluation. Controls were randomly selected from inpatients with other dermatological conditions in a 1: 1 ratio during the same period. Their relationship was analysed using univariate (χ² or Fisher exact tests) and multivariate logistic regression analysis. Results: There were 78 cases and 83 controls. Of the 78 cases, 42 patients had a history of treatment with antihypertensive drugs or statins. Cases were not found to be more likely on angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, β-blockers, calcium channel blockers, thiazides or statins compared to controls (OR: 0.81; 95% CI: 0.43–1.51; p = 0.507). Conclusions: There was insufficient evidence to suggest a significant relationship between the chronic use of antihypertensive drugs or statins and idiopathic GED in this study, despite previous evidence reporting this might be so. Further case-control studies with larger sample sizes are needed to evaluate this association.

Cutaneous paraneoplastic syndromes

A variety of cutaneous abnormalities can be seen in patients with malignant diseases, some of which are infectious, with others representing direct involvement of the skin by the underlying disorder. Yet another group of lesions can be regarded as associated markers of the malignant process, and, as such, are termed "paraneoplastic." This review considers the latter collection of conditions, grouping them by the generic type of malignancy that is usually linked to the paraneoplasia. Some of the processes show a predominant association with alimentary tract malignancies (acanthosis nigricans, acrodermatitis paraneoplastica, florid cutaneous papillomatosis, necrolytic migratory erythema, palmoplantar keratoderma, pancreatic fat necrosis, and pityriasis rotunda). Others are usually linked to a hematolymphoid malignancy (acquired ichthyosis, exfoliative erythroderma, necrobiotic xanthogranuloma, pemphigus paraneoplastica, plane xanthoma, pyoderma gangrenosum, scleromyxedema, Sweet syndrome, and leukocytoclastic vasculitis). Finally, yet another collection of paraneoplastic skin disorders can associate themselves with anatomically-diverse malignancies (Leser-Trelat syndrome, Trousseau syndrome, dermatomyositis, erythema gyratum repens, hypertrichosis lanuginosa acquisita, papuloerythroderma of Ofuji, tripe palms, and multicentric reticulohistiocytosis). Recognition of these processes by the pathologist can be a valuable step in the characterization of underlying malignant diseases.

Drug-induced psoriasis

Psoriasis is known to be triggered by a number of factors including drugs. Some therapeutic agents for the treatment of psoriasis are also known to have a paradoxical effect and alter the course of the disease. The drugs may either cause de novo psoriasis or are responsible for aggravating preexisting psoriasis. The distinction is not always clear-cut and is clinically often indistinguishable from psoriasis vulgaris. The morphological types can vary from plaque psoriasis to pustular psoriasis and even erythroderma. There are certain established agents which are known to trigger psoriasis. Many of the biological agents and targeted therapies available today can trigger psoriasis by activating signaling pathways. The lag time between the intake of drug and onset of psoriasis is highly variable and thus requires a high index of suspicion. Due to the various systemic comorbidities, patients with psoriasis often receive polypharmacy and hence it is important for dermatologists, rheumatologists, and physicians to be aware of the possible triggers.

A rare case of erythroderma with extensive tinea infection in a tertiary care hospital

Erythroderma, or generalized exfoliative dermatitis, is a disease characterized by erythema and scaling of greater than 90% of the body's surface. The consequential dysmetabolism is potentially life threatening. A detailed history is necessary to identify and treat the underlying cause of this dermatitis. We are presenting a rare case of erythroderma with extensive tinea infection in 50 years old Indian male.

Sezary syndrome in a 65-year-old schizophrenic patient

Sezary syndrome (SS) is the leukemic variant of cutaneous T-cell lymphoma characterized by a triad of erythroderma, peripheral lymphadenopathy, and the presence of circulating atypical lymphoid cells (Sezary cells) in the blood. We present a 65-year-old female with a recent-onset schizophrenia, presenting with a 3-year history of recurrent generalized pruritus, erythroderma, and a 2-month history of recent-onset, mushroom-like skin tumors. Examination revealed generalized erythroderma affecting more than 80% of total body surface area and significant peripheral lymphadenopathy. Peripheral blood buffy coat examination showed Sezary cells which constituted more than 18% of total circulating lymphoid cells, and histology report of the wedge biopsy of skin tumor confirmed mycosis fungoides. The patient was commenced on cytotoxic chemotherapy with some improvement in symptoms. However, she relapsed 2 months later, with development of new fungating tumors and died from overwhelming sepsis. We report this case to highlight the occurrence of two comorbidities together, each of which may negatively worsen the progression of the other, and to report a case of SS as one of the rare causes of generalized exfoliative dermatitis. Thus, there is a need for early skin biopsy and histology in any elderly patient presenting with recurrent generalized pruritus and erythroderma.

STAPHYLOCOCCAL SCALDED SKIN SYNDROME IN A VERY LOW BIRTH WEIGHT PRETERM INFANT

Staphylococcal scalded skin syndrome (SSSS) is an exfoliative dermatitis produced by the toxins of some strains of staphylococci, predominantly phage Group 2, strains 71 and 55. It has been reported mostly in children under 5 years of age with few cases only reported in very preterm infants. The disease can be life threatening in very low birth weight preterm babies. We are reporting one such case to emphasize the importance for clinicians to not only recognize the clinical manifestations of SSSS but also the need to closely monitor infants, especially VLBW infants with SSSS for bacterial sepsis and other complications.

Mutation analysis of interleukin-36RN gene in a child with erythroderma

A 2-year-old male child presented with recurrent diffuse desquamative red macules all over the body, without pustules or ulcers. The patient had repeated fever, which peaked at 39.3 ℃. The patient was diagnosed with erythroderma. Whole genome sequencing showed 2 compound heterozygous mutations (c.28C>T and c.368C>T) in the interleukin (IL) -36RN gene. The mutation c.28C>T was inherited from his father, leading to p.Arg10X and premature termination of amino acid transcription. The mutation c.368C>T was inherited from his mother, causing p.Thr123 Met. No mutation was found in the IL-1RN gene in the patient. The compound heterozygous mutations c.28C>T and c.368C>T may be responsible for erythroderma in this child. Key words: Dermatitis, exfoliative; Psoriasis; Interleukin-1; DNA mutational analysis; Interleukin-36 receptor antagonist

A possible case of exfoliative dermatitis due to ibuprofen in an Eleven-month-old infant

Ibuprofen is a NSAID belonging to the class of propionic acid derivatives which is widely used for its analgesic, antipyretic and anti-inflammatory action. Well-known adverse effects of ibuprofen include gastric irritability leading to nausea and vomiting as well as allergic manifestations such as urticaria and skin rashes. Severe ADRs include renal papillary necrosis, SJS/TEN, and thrombotic events leading to myocardial infarction and stroke. Authors present a case of exfoliative dermatitis in an 11-month-old infant possibly due to ibuprofen. An 11-month-old infant was prescribed syrup ibuprofen by a local medical practitioner for unclear reasons. Three days after ibuprofen therapy, the infant developed erythematous, crusting exfoliative lesions predominantly over the face with a few lesions over the lower abdomen. Subsequently, the infant was admitted to Kempegowda Institute of Medical Sciences and Research Center Hospital, Bangalore. A diagnosis of drug-induced exfoliative dermatitis was made after ruling out other causes. Treatment was initiated with intravenous and topical dexamethasone along with saline compressions and amoxicillin + clavulanic acid for secondary bacterial infection as well as topical emollient cream applied over the affected areas. The lesions improved significantly with the above management and the infant recovered enough to be discharged from the hospital after 3 days. The reaction was assessed to be “possible” as per Naranjo and WHO-UMC causality assessment scales, “moderately severe” on modified Hartwig’s severity assessment scale and “not preventable” according to Schumock and Thornton criteria. Severe and serious reactions such as exfoliative dermatitis can be caused by commonly used drugs like ibuprofen.

DRESS Syndrome- A Rare Condition

DRESS syndrome (Drug reaction with Eosinophilia and Systemic Symptoms) is a rare but potentially life-threatening drug hypersensitivity reaction characterized by fever, exfoliative dermatitis and maculopapular rash, lymphadenopathy, eosinophilia, leucocytosis, and involvement of internal organs as liver, lung, heart, and kidney. The disorder starts within 2–8 weeks after taking an offending drug. Long latency between drug treatment and onset of symptoms along with variability of presentation make the diagnosis quite difficult. Here, we are presenting a case report of 58 year old male presented with short history of high grade fever with extensive skin rash. Investigations revealed leucocytosis with gross eosinophilia. He gave history of taking carbamazepine for more than last 3 months prescribed by his neurologist. According to the RegiSCAR scoring system, our case could be classified as definite DRESS syndrome. After withdrawal of the offending drug carbamazepine and starting high dose of systemic corticosteroid, he improved dramatically. As this syndrome is quite uncommon, so we are reporting this case.Bangladesh J Medicine Jul 2018; 29(2) : 91-93

Profile of erythroderma in children in hospital dermatology departments in Lomé, Togo: study of 28 cases.

The aim of this study was to describe the epidemiological, clinical, and therapeutic profile as well as the outcomes of children with erythroderma (also known as exfoliative dermatitis) in hospital dermatology departments in Lomé, Togo. This retrospective descriptive study examined the records of patients aged 0 to 15 years who were treated for erythroderma in dermatology departments in Lomé from January 1997 to December 2016. The study included 28 patients, with a mean age of 6.3 ± 5 years. The mean duration of progression of erythroderma before the consultation was 39.2 days. Pruritus was present in 60% of the children, fever in 13.3%, and impairment of general status in 3.3%. Among the children aged 3 months and older, 84.2% had dry squamous erythrodermic dermatosis, and among those younger than 3 months, 15.8%. Among those with erythrodermic dermatosis, atopic dermatitis (70.6%) was the most frequent dermatosis. No cause was found in 32.1% cases. Course and outcome were favorable in 53.3% of cases; one child died, and the rate of loss to follow-up was 43.3%. Erythroderma is a rare disease in children in hospital dermatology departments in Lomé. Erythrodermic dermatosis, particularly atopic eczema, remains the most frequent cause.

P wave dispersion and cardiac troponin i concentration in canine visceral leishmaniasis*

Canine Visceral Leishmanisis (CVL) is mostly fatal chronic systemic disease caused by Leishmaniainfantum (L. infantum) in the Old World countries. In infection, dogs plays both host and reservoir role. By this context, infected dogs are a threat to both people and other dogs. In dogs infected with L. infantum, cardiac involvement in addition to the other affected organs and systems were also reported in the updated literatures. In the present study, the aim was to clinically evaluate the probable cardiac damage in dogs with CVL according to its stage via measuring atrial conduction time by use of Pd determined within computerized electrocardiography and cardiac troponin I (CTnI) level. A total of 24 dogs, of both sexes and various ages, referred with one or more of the clinical findings such as hypertrichosis, periocular alopecia, weight loss, onychogryphosis, skin lesions (severe scaling, exfoliative dermatitis compatible with alopecia) and/or anorexia, lymphadenopathy, hepatosplenomegaly. In the healthy control group for comparison and in dogs with CVL to determine presence, nature and level of cardiac damage, the evaluations were performed with computerized 12-lead ECG device [(1 mV/cm amplitude in resting and 50 mm/sec) (Pd measurement)] and serum CTnI concentrations were measured by using species specific commercial test kit. Eighteen dogs infected with CVL (IFAT positive) with/without different clinical sign were enrolled into three different groups (n=6); of the above mentioned clinical sign, to those of which with single clinical sign (oligosymptomatic) were included in the group I; those with multiple or several clinical signs (polysymptomatic) were included in the group II; and asymptomatic dogs were included group III. CVL-negative healthy dogs without any disease were left in group IV. High levels of cTnI concentration were detected in 10 of 18 dogs infected CVL with all polysymptomatic dogs. In all cases of the control group, cTnI levels were in the reference range [<0.03 ng/dL]. Even the comparison of each groups, no statistically significance (p>0.05) was found between CVL positive and control dogs. Mean ± standard deviation of Pd values were 22.76±3.12, 22.03±0.80, 22.73±0.80 and 25.67±1.41 in the control group, asymptomatic group, oligosymptomatic group, and polysymptomatic group, respectively. In comparison between groups, polysymptomatic group was significantly different than control (p = 0.026), asymptomatic (p = 0.012) and oligosymptomatic (p = 0.027) groups. Although a statistically significant difference was not found between CVL positive and control dogs in the present study, it was suggested that the individual increase may be associated with myocarditis due to disease. Besides, it may be claimed that the mean Pd values determined in especially polysymptomatic dogs was higher compared to the control group, whereas this may be accepted in the reference ranges based on mean Pd values reported in healthy dogs. However considering infected dogs population as 6 in each group, it may be safely claimed that further investigations regarding greater number of cases the may be warranted.

Malnutrition and skin disease in Far East prisoners-of-war in World War II.

During the Second World War, thousands of captured British and Commonwealth troops were interned in prisoner-of-war (POW) camps in the Far East. Imprisonment was extremely harsh, and prisoners developed multiple pathologies induced by physical hardship, tropical infections and starvation. Immediately after the war, several POW doctors published their clinical experiences, including reports of skin disease caused by malnutrition. The most notable deficiency dermatoses seen in Far East POWs were ariboflavinosis (vitamin B2 or riboflavin deficiency) and pellagra (vitamin B3 or niacin deficiency). A lack of vitamin B2 produces a striking inflammatory disorder of scrotal skin. Reports of pellagra in POWs documented a novel widespread eruption, developing into exfoliative dermatitis, in addition to the usual photosensitive dermatosis. A review of the literature from 70years ago provides a reminder of the skin's response to malnutrition.

A 50-Year-Old Male with Dapsone Hypersensitivity Syndrome

Dapsone, diamino-diphenyl sulfone, is an antibacterial and anti-inflammatory drug which is used worldwide for treating many diseases, such as leprosy, dermatitis herpetiformis, linear IgA bullous dermatosis, chronic bullous dermatosis of childhood, bullous eruption of systemic lupus erythematosus, erythema elevatum diutinum, leukocytoclastic vasculitis, polyarteritis nodosa and other kinds of vasculitis, prurigo nodularis, nodulocystic acne, cutaneous mycetoma, pustular psoriasis, malaria, pneumocystis carinii pneumonia etc. Since it is widely used, the adverse effects of this drug attract the attention of doctors from different specialities. Dapsone can cause several adverse effects, the most serious one is idiosyncratic systemic hypersensitivity syndrome, namely dapsone hypersensitivity syndrome (DHS), which is potentially fatal, characterized by fever, facial edema with infiltrated papules, generalized papulopustular or exanthematous rash which may extend to exfoliative dermatitis, eosinophilia, lymphadenopathy, hematologic involvement and organ involvement such as hepatitis, nephritis, pneumonitis, encephalitis, myocarditis occurring after 3–6 weeks of drug therapy. Here we have described a case of dapsone hypersensitivity syndrome developed after dapsone therapy for prurigo nodularis. The case is being reported to emphasize the need for timely diagnosis and prompt treatment of this rare complication for successful outcomes. Physicians should be aware of this infrequent but potentially fatal severe form of adverse reaction that can mimic other conditions.J Enam Med Col 2018; 8(2): 108-113

IA case of canine squamous cell carcinoma secondary to solar keratosis (actinic carcinoma in situ)

We present the case of a squamous cell carcinoma developed in a pre-existing solar keratosis of the trunk in a whitehaired Dogue Argentino. Deep pyoderma along with exfoliative erythroderma were the initial clinical findings. Several months after the diagnosis of solar keratosis, the dog presented with multiple nodules and ulcers on the flank and abdomen and the diagnosis of malignancy was confirmed microscopically.

Feline leishmaniosis due to Leishmania infantum (syn. L. chagasi)

From the beginning of the 20th century, feline infections by at least eight different Leishmania species have been sporadically reported, worldwide. In the Mediterranean countries, feline infection imposed by L. infantum, the aetiological agent of canine leishmaniosis and human visceral and cutaneous leishmaniasis, is of particular importance, because cats may represent a "secondary reservoir" of the parasite in the nature. Although the prevalence of the infection may be as high as 60%, most infected cats are resistant and remain asymptomatic, while only occasionally, they may present cutaneous and/or ocular lesions, and perhaps systemic clinical signs. In feline leishmaniosis, ulcers and nodules, usually localized at mucocutaneous junctions, planum nasale, face and ear pinnae, constitute the mainstream of cutaneous lesions. In a small number of cases, exfoliative dermatitis and pododermatitis have been noted, whereas a cause-and-effect relationship, between feline leishmaniosis and the presence of papules, crusts and generalized alopecia remains speculative. Ocular lesions, such as those associated with conjunctivitis, (ulcerative) keratitis, posterior or anterior uveitis, chorioretinitis and panopthalmitis are relatively common. The only systemic signs that have been constantly associated with feline leishmaniosis are peripheral lymphadenomegaly, splenomegaly, hepatomegaly and chronic nephritis that may lead to chronic renal failure. The most consistent laboratory abnormality is hyperglobulinaemia that is usually polyclonal. Feline leishmaniosis can co-exist with various other infectious and non-infectious diseases that may alter its clinical picture and/or influence the immunological response of the infected cat. Diagnosis is usually based on the results of cytology,histopathology, immunohistochemistry, culture, serology and polymerase chain reaction (PCR). Apart from the advantages and limitations inherent to each of these methods, their diagnostic value depends on many factors, such as the biological sample being used, the reagents and the particular technique employed. Leishmania amastigotes can be detected by applying bone marrow, lymph node, spleen, cutaneous and ocular cytology, histopathology, and/or immunohistochemistry, which are 100% specific. Serology may underestimate or even overestimate the frequency of the infection in the endemic areas, depending on the methodology and the cut-off value, whereas PCR, that can be performed on the tissues mentioned before, plus the peripheral blood may be a better option for epidemiological studies. Although a uniformly effective treatment for symptomatic cats has not been established yet, long-term allopurinol administration, along with the management of any concurrent diseases, is the only treatment to suggest at moment.

Analysis of keratin 1 gene mutation and phenotypes in a pair of twins with bullous congenital ichthyosiform erythroderma

Objective To identify mutations in keratin genes (KRT1 and KRT10) in a pair of twins with bullous congenital ichthyosiform erythroderma (BCIE) , and to explore the relationship between the causative genes and phenotypes. Methods Clinical data were collected from a pair of twins with BCIE and their family members. Peripheral blood samples were obtained from the twins, their old brother and parents, and DNA was extracted from these blood samples. Polymerase chain reaction (PCR) was performed to amplify all the coding exons and their flanking sequences of the KRT1 and KRT10 genes, and 100 unrelated healthy persons served as controls. Results The 11-year-old male proband presented with recurrent blisters, hypertrophy and desquamation all over the body for 11 years. His twin brother had similar skin lesions. Skin examination of the proband showed diffuse erythema covered with thick scaly crusts on the trunk and extremities. Blisters, bullae and erosions due to ruptured blisters were observed locally with tenderness on palpation. There were obvious hyperkeratotic and hard lesions on the big joints of the extremities. Diffuse hyperkeratosis could be seen on the palms and soles. A mutation c.591 + 1G > A was identified at position 1 in intron 1 of the KRT1 gene in the twins, but not in the 3 healthy family members or the 100 unrelated healthy controls. Conclusion The mutation c.591 + 1G > A at position 1 in intron 1 of the KRT1 gene may contribute to the clinical phenotype of the twins with BCIE. Key words: Ichthyosiform erythroderma, congenital; Keratin-1; Keratin-10; DNA mutational analysis

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