Bullous Eruption Research Articles

Rothmund–Thomson syndrome presenting with bullous eruption: A rare case report

Rothmund–Thomson syndrome is a rare, autosomal recessive genodermatosis characterized by an early-onset poikiloderma, skeletal abnormalities, short stature, premature aging, and increased susceptibility to malignancy. We report a case of a 1-year-old male child with bullous lesions and pigmentary changes over the face and extremities. Strict photoprotection and careful surveillance for malignancy forms the mainstay of treatment. The case is being reported due to its rarity and the diagnostic dilemmas associated with it.

Zmiany pęcherzowe u pacjenta ze świerzbem – rzadka postać powszechnie występującego schorzenia

Zmiany pęcherzowe u pacjenta ze świerzbem – rzadka postać powszechnie występującego schorzenia

Bullous acral eruption related to secukinumab

Skin reactions related to secukinumab are uncommon. Although the initial phase 3 studies of this medication reported infection and urticaria as adverse cutaneous events, other cases of unique adverse events have since been reported. We are presenting a patient who developed an exuberant hand and foot reaction after starting secukinumab.

Bullous disorders associated with anti–PD-1 and anti–PD-L1 therapy: A retrospective analysis evaluating the clinical and histopathologic features, frequency, and impact on cancer therapy

Bullous disorders associated with anti-programmed cell death 1 (PD-1)/programmed cell death ligand 1 (PD-L1) therapy are increasingly reported and may pose distinct therapeutic challenges. Their frequency and impact on cancer therapy are not well established. To evaluate the clinical and histopathologic findings, frequency, and impact on cancer therapy of bullous eruptions due to anti-PD-1/PD-L1 therapy. We retrospectively reviewed the medical records of patients evaluated by the oncodermatology clinic and consultative service of Yale New Haven Hospital from 2016 to 2018. We identified 9 of 853 patients who developed bullous eruptions (∼1%) that were treated with an-PD-1/PD-L1 therapy at our institution during the study period: 7 presented with bullous pemphigoid, 1 presented with bullous lichenoid dermatitis, and 1 presented with linear IgA bullous dermatosis in the context of vancomycin therapy. In all, 8 patients required systemic steroids, 5 required maintenance therapy, and 8 required interruption of immunotherapy. All 9 patients had an initial positive tumor response or stable disease, but 4 went on to develop disease progression. This was a retrospective study from a single tertiary care center. Bullous disorders developed in approximately 1% of patients treated with anti-PD-1/PD-L1 therapy at our institution and frequently resulted in interruption of immune therapy and management with systemic corticosteroids and occasionally steroid-sparing agents.

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

Phenytoin induced Stevens Johnson syndrome: a case report

Stevens-Johnson syndrome (SJS) is a rare consequence of hypersensitivity reaction precipitated by certain drugs and viral infections. It is an idiosyncratic drug reaction usually associated with drugs like anti-epileptics, non-steroidal anti-inflammatory compounds and antibiotics. The overall incidence of this entity is very low and is life-threatening if undiagnosed and untreated. The syndrome is characterized by purpuric macules and bullous eruptions involving the mucous membrane which may be followed by systemic manifestations. The mechanism of SJS due to drugs is not fully defined. Delayed Hypersensitivity reaction mediated by T lymphocytes in response to a drug is thought to be responsible. Here authors present a case of SJS induced by phenytoin in an adult male. The case warrants the need of adopting a meticulous approach while prescribing phenytoin. The case is being reported to accentuate the importance of adverse drug reactions and to emphasize the importance of reporting such reactions ensuring efficient pharmacovigilance.

Dyshidrosiform palmoplantar pemphigoid with low‐titer autoantibodies against BP180 NC16A

AbstractDyshidrosiform pemphigoid (DP) is a variant of bullous pemphigoid, presenting with a bullous or vesicular eruption localized on the soles and palms, and thus, DP has clinical resemblance with dyshidrosiform dermatitis. Here, we report a case of DP with literature review, focusing on the titer of antibodies directing the NC16A domain and other regions on BP180. Our case had a low titer of anti‐BP180 NC16A antibodies, and our survey of literature disclosed that 10 of 20 cases of DP had low titers (index: <150, or <87.5 U/mL) of anti‐BP180 NC16A antibodies. It is an issue whether DP is caused merely by a low titer of anti‐BP180 NC16A antibodies or by BP‐autoantibodies targeting other regions of BP180. We therefore examined our case with full‐length BP180 ELISA and found the absence of such antibodies. Our study suggests that DP is associated with low‐titer anti‐NC16A antibodies but not with the presence of antibodies against other parts of BP180.

Bullous Pemphigoid-like Skin Eruption during Treatment with Rivaroxaban: A Clinical Case Study.

Little has been documented about hypersensitivity reactions caused by treatment with rivaroxaban. This paper reports a bullous pemphigoid-like skin eruption that occurred in a 76-year-old female patient during rivaroxaban treatment. This case highlights the vigilance required by healthcare workers in recognising potential adverse effects of newly marketed drugs and in making medication changes when necessary. A bullous pemphigoid-like eruption due to treatment with rivaroxaban has not, to the best of the Authors’ knowledge, been reported previously in the literature.LEARNING POINTSRivaroxaban can cause a bullous eruption apparently similar to epithelial toxic necrolysis (or to Stevens-Johnson syndrome).There is an apparent similarity between skin adverse events caused by the different anticoagulants.The anticoagulant responsible for the skin side-effects can be identified on clinical grounds by the correct differential diagnosis.

Delayed Allergic Cutaneous Hypersensitivity to Icodextrin

Rashes pose a unique challenge to general internists. Here we present a case of a 51-year-old female with end-stage renal disease (ESRD) on peritoneal dialysis who presented to a General Internal Medicine clinic with a vesiculobullous rash on palms and soles 6 months after using icodextrin dialysate, a widely used osmotic agent in peritoneal dialysis. The investigations were positive for an elevated eosinophil count, elevated IgE level and a skin biopsy consistent with an allergic reaction. Her symptoms and labs normalized after stopping icodextrin. There are some reports of allergic reactions to icodextrin which usually occur within a month of initiation of the agent and are self-limiting. Here we hope to report a case of a reaction that occurred 6 months after using icodextrin.
 Resume
 Les éruptions cutanées posent un défi particulier aux internistes généraux. Nous rapportons ici le cas d’une femme de 51 ans atteinte d’insuffisance rénale terminale (IRT), sous dialyse péritonéale, qui se présente à une clinique de médecine interne générale avec une éruption cutanée vésiculo-bulleuse sur les paumes et la plante des pieds. Depuis six mois, la patiente utilise un dialysat d’icodextrine, un agent osmotique largement utilisé en dialyse péritonéale. Les analyses ont révélé une numération élevée des éosinophiles, un taux élevé d’IgE et une biopsie cutanée a indiqué la présence d’une réaction allergique. Les symptômes et les résultats d’analyses se sont normalisés après l’arrêt de l’icodextrine. Des réactions d’allergie à l’icodextrine ont déjà été rapportées, mais celles-ci surgissent habituellement dans le mois suivant le début de l’usage de l’agent et se résorbent d’elles-mêmes. Nous désirons signaler un cas de réaction survenue six mois après le début de l’utilisation de l’icodextrine.
 
 

A rare case of neurocutaneous disorders of the newborn: Incontinentia pigmenti

Incontinentia Pigmenti (IP) is a rare X-linked dominant disorder which is mostly lethal for males. It effects hair, teeth, nails, eyes and central nervous system along with skin. A twelve day old female patient was referred to the dermatology departmant due to skin eruptions that began the day before. There was a widespread erythema and many papuls and vesicles were seen on the eryhematous skin in linear sequence. Spongiosis with eosinophilia and necrotic keratinocytes were seen on histopathologic examination. The patient was diagnosed with incontinentia pigmenti. Vesicles and bullous eruption are rarely seen in newborns. The physcians should not avoid histopathologic examination especially in females. Since incontinentia pigmenti is a rare but multisystemic disorder, early diagnosis is crucial.

Linear IgA bullous dermatosis associated with ulcerative proctitis: treatment challenge

Linear IgA bullous dermatosis is a rare bullous disease in children and adults that can be associated with autoimmune conditions, malignancies, infections, or medication exposure. The definitive diagnosis relies on the biopsy. A 58-year-old man presented to our clinic with a pruritic vesicular and bullous eruption. Histology showed the classic findings of a subepidermal blister with neutrophilic infiltrate and linear IgA deposition along the dermal-epidermal junction. Upon further evaluation, he was diagnosed with ulcerative proctitis. His therapy was complicated owing to side effects and lack of response to the standard treatment options. Dapsone, a first-line therapy, caused symptomatic methemoglobinemia whereas niacinamide with doxycycline were not effective. He required intravenous and oral steroids to reach improvement followed by transitioning to methotrexate.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus: report of a case and review of the literature

Severe bullous eruptions in systemic lupus erythematosus (SLE) patients include bullous SLE, Rowell syndrome, toxic epidermal necrolysis (TEN), and TEN-like eruption of acute cutaneous lupus (TEN-like ACLE). TEN-like ACLE, a rare manifestation of SLE that closely mimics TEN, can be distinguished by characteristic clinical and laboratory findings. A 27-year-old man with SLE who developed TEN-like ACLE after initiating mycophenolate mofetil for active SLE is reported. The reports of 37 women and six men including our patient with TEN-like ACLE were also reviewed. The diagnosis of SLE or subacute cutaneous lupus erythematosus was either previously confirmed or established at the time of diagnosis of TEN-like ACLE in 41 patients. Fever was present in 59% of patients. The onset of TEN-like ACLE was either subacute (73%) or acute (27%). Thirteen cases did not clarify the nature of disease onset. The skin lesions often presented initially on sun-exposed sites (29 patients) and involved one or more mucous membranes (21 patients). A new medication may have caused the TEN-like ACLE in 67% of the patients. Systemic corticosteroids either alone or combined with hydroxychloroquine, intravenous immunoglobulin, or mycophenolate mofetil were the most commonly used treatment. Patients with TEN-like ACLE patients had an 89% survival.

Toxic Epidermal Necrolysis vs Lupus Flare?

SESSION TITLE: Critical Care 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS) are severe mucocutaneous reactions characterized by extensive necrosis and detachment of epidermis. Systemic lupus erythematosus (SLE) patients are at increased risk of SJS/TEN. Here we discuss a patient with SLE who developed TEN. CASE PRESENTATION: A 45 year old female with discoid and systemic lupus erythematosus presented to dermatology with bullous skin lesions 2 days after starting gabapentin, naproxen, and duloxetine. Despite stopping her new medications, she developed a facial rash which spread to her trunk and extremities, affecting 30% body surface area. She had denuded oral mucosa and conjunctival hyperemia with no photosensitivity or eosinophilia. Complements were normal. Wedge biopsy showed acute on chronic vacuolar interface dermatitis with epidermal ulceration and partial to full-thickness epidermal necrosis, suggestive of chronic discoid lupus with superimposed bullous eruption. The patient was admitted to the ICU and treated with intravenous immunoglobulin for three days. Home medications were held. Supportive care included intravenous hydration, reverse isolation in heated environment, specialized wound care, oral and eye care. Methicillin resistant staphylococcus aureus bacteremia was treated. The patient clinically improved and was in reepithelialization phase at time of discharge. DISCUSSION: The incidence of TEN is 0.4-1.2% per million. In patients with SJS/TEN, the prevalence of SLE is estimated to be 1.2% (compared to 0.02-0.05% in the general population). It is unclear if the predisposition for TEN in SLE patients is because of the underlying inflammatory state or because of medications SLE patients often require, such as NSAIDs. Differentiating SLE from TEN is complicated by clinical and histologic similarities. Pathology often demonstrates subepidermal vacuoles, necrotic keratinocytes, and confluent epidermal necrosis, which is found in SLE and TEN. Bullous eruptions and erosive mucosal involvement are typical features in TEN, but can also be found in SLE. This patient developed skin and mucosal lesions after starting new medications and had improvement without steroids, which makes active SLE less likely. Normal complement and serology also supports the clinical diagnosis of TEN over active SLE. CONCLUSIONS: Mortality in patients with SJS and TEN exceeds 10 and 30% respectively. Because the management of active SLE lesions and TEN differ greatly, early recognition of TEN can be life saving. High index of suspicion, early biopsy, and serology can be helpful in differentiating such cases. Reference #1: M. Ziemer, et al, “Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature,” Brit J Dermatol 2012; 166 (3), 575-600. Reference #2: Ranario JS, et al. Bullous lesions in a patient with systemic lupus erythematosus. J Clin Aesthet Dermatol 2014; 7:44-49 DISCLOSURE: The following authors have nothing to disclose: Gauri Behari, Sukhdeep Malhi, Bradley Tymchuk, Ronnie Self II, Suman Lata, Ibrahim El-Abbassi No Product/Research Disclosure Information

005 A Case of Pemphigus Exacerbated by HSV Flare

Autoimmune mucocutaneous blistering diseases are a group of diseases impacted by both genetic and environmental factors. Pemphigus vulgaris (PV) is an intraepidermal disease involving both the skin and the mucosa with autoantibodies targeting desmogleins 1 and 3. The exact etiology of PV is not completely understood, however studies have outlined exacerbation of PV in the setting of viral infection. In particular, case reports have noted that treatment resistant PV was often associated with herpes simplex virus (HSV) infection. HSV has also been considered a trigger for developing PV. Here we present a 34-year-old African American female with a generalized bullous eruption and mucosal involvement following a HSV flare. Results from lesional and perilesional biopsies showed findings consistent with a diagnosis of PV and, interestingly, a concurrent dermatophytosis. Treatment of this patient was ultimately directed towards immunosuppression and resolving the concurrent fungal infection. However, optimal biologic treatment has been limited due to lack of insurance coverage and cost.

Elderly Man With Bullous Eruption on the Feet

Elderly Man With Bullous Eruption on the Feet

Dermatomyosite amyopathique avec anticorps anti-MDA-5, associée à une pemphigoïde bulleuse, un syndrome de Sjögren et un lymphome de type MALT

The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement. A 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption. Based on the investigations performed, a diagnosis was made of bullous pemphigoid. At the same time, amyopathic dermatomyositis was discovered together with interstitial lung disease. Systemic steroids were introduced in combination with rituximab. A favourable outcome was achieved. Anti-MDA5 dermatomyositis must be considered systematically in all cases of pulmonary involvement associated with cutaneous signs of dermatomyositis, in which no muscular involvement is generally seen. This condition accounts for up to 7% of DM and carries a severe prognosis due to pulmonary involvement.

Severe generalized bullous drug eruption secondary to alemtuzumab (Lemtrada)—Outcome of re-exposure

Severe generalized bullous drug eruption secondary to alemtuzumab (Lemtrada)—Outcome of re-exposure

Nivolumab-induced bullous lichenoid drug eruption

Nivolumab-induced bullous lichenoid drug eruption

Clinical Case of Toxic Epidermal Necrolysis

Abstract Toxic epidermal necrolysis or Lyell’s syndrome is a severe life-threatening adverse drug reaction with a high mortality rate. The drugs most commonly involved are: antibiotics; anticonvulsants; antiretroviral drugs; nonsteroidal anti-inflammatory drugs, allopurinol. Case report: A 68-year-old female, presented with complaints of fever and extensive rashes on the skin of the face, the neck and the trunk, severe itching of the skin, ulcerations and erythema of the conjunctiva and the oral cavity and difficulty in swallowing. She has a period of two or three days of general discomfort and fatigue, rash, fever up to 38°C, sore throat, arthralgia, myalgia, loss of appetite and have been treated with antipyretics, antibiotic – ampicillin, antihistamines, vitamins. Her state worsened during the next 3 days, therefore she was hospitalized in the Toxicology clinic of University Hospital “N. I. Pirogov”. Multiple maculopapular and bullous eruptions, plaques were present all over the body, blisters that cover a substantial portion of the body. The entire skin covering the body surface was denuded and peeled off with minor manipulation – the Nikolsky’s sign. Intraorally, multiple oral ulcers of the buccal mucosa, tongue, palate, labial mucosa, and soft palate were seen. Hemorrhagic erosions were also seen on both the upper and lower lips. Conjunctivitis and ulceration of genitalia were also noted. The lesions got slowly better with serum therapy, fluid and electrolyte replacement, systemic corticosteroids, antihistamines, antibiotic, vitamins, H2 blockers, topical care of mucosal changes. Evolution was satisfactory with epidermization and the patient was discharged from the hospital after 1 month.

Generalized Bullous Drug Eruption to Faropenem - Hitherto Unreported.

Generalized Bullous Drug Eruption to Faropenem - Hitherto Unreported.