- Email: [email protected]
Pustular polymorphic eruption and neutrophilic dermatosis associated with acute coccidioidomycosis
5477
4682
Pustular polymorphic eruption and neutrophilic dermatosis associated with acute coccidioidomycosis Leah Swanson, MD, Mayo Clinic; Elika Hoss, MD, Mayo Clinic; Mark Pittelkow, MD, Mayo Clinic; Steven Nelson, MD, Mayo Clinic Coccidioidomycosis is a fungal infection endemic to the southwest US with increasing incidence. Varied cutaneous presentations including neutrophilic dermatoses are reported however rarely. A 49-year-old otherwise healthy woman presented with one month of nonproductive cough and one day of diffuse rash. She had a temperature of 39.38C, leukocytosis of 16.3, lobar consolidation on chest x-ray and was empirically started on antibiotics. Given negative viral and bacterial studies, fluconazole was initiated for concern of coccidioidomycosis. On the face and back she had 5-10 mm coalescing pink papules studded with pustules. On the chest, abdomen, arms, and legs she had pink targetoid papules with purpuric centers. Histopathology of pustular lesion showed neutrophilic dermal inflammation, pustule formation, and papillary dermal edema. Infectious stains and cultures were negative. Findings were most consistent with Sweet syndrome. Initial Coccidioides antibody studies and spherulin skin test were negative. She improved with topical corticosteroids and fluconazole. Three weeks after presentation, Coccidioides IgG antibody converted to positive by enzyme immunoassay. Cutaneous manifestations of coccidioidomycosis are reactive or represent skin infection by hematogenous dissemination or primary cutaneous inoculation. In disseminated and primary cutaneous infections, Coccidioides is observed microscopically and grows in culture from biopsy. Reactive eruptions, secondary to host immune responses, contain no Coccidioides and include erythema nodosum, acute exanthems, erythema multiforme, interstitial granulomatous dermatitis, and Sweet syndrome. Sweet syndrome is characterized by fever, leukocytosis, tender red skin lesions, and on histopathology a diffuse, dense, dermal neutrophilic infiltrate. Sweet syndrome is often preceded by upper respiratory tract infection, and several cases have been reported in association with acute pulmonary coccidioidomycosis. Pustules, a prominent feature in this case, superimposed on classic plaques of Sweet syndrome have rarely been reported in association with coccidioidomycosis. Systemic corticosteroids could exacerbate coccidioidomycosis and should be avoided. Although used to treat moderate to severe pulmonary infection, antifungals are not utilized in the treatment of reactive eruptions. Dermatologists should consider this fungal infection in patients with pustular polymorphic eruptions and travel to endemic regions.
Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome with recurrent vasculitis Dorene Niv, DO, St. Joseph Mercy Dermatology; James Ramirez, MD, St. Joseph Mercy Dermatopathology; David Fivenson, MD, St. Joseph Mercy Dermatology Autoinflammatory syndromes are characterized by recurrent episodes of sterile inflammation, in the absence of circulating autoantibodies and/or autoreactive Tcells. PASH syndrome is a recently identified hereditary autoinflammatory syndrome consisting of multiple neutrophilic dermatoses including: pyoderma gangrenosum (PG), acne, and hidradenitis suppurativa (HS). The triad of PG, acne, and HS occurs in extremely rare instances. To our knowledge, the clinical tetrad of PG, acne, HS, and vasculitis has not been reported. A 36-year-old man presented with abscesses increasing in size on the buttocks and axillae. His past medical history included recurrent PG on the lower extremities, generalized severe acne, and leukocytoclastic vasculitis (LCV) that developed while on 100 mg daily prednisone for a large PG lesion. Physical exam revealed cysts, papules and pustules with hyperpigmentation and scarring on the back and chest. Multiple inflammatory abscesses with sinus tracts and scarring were present on the lower back and buttocks. A 14 cm ulcer with violaceous, undermined borders, and palpable purpura extensively on the lower extremities. Lab evaluation revealed a bimodal IgG and IgA monoclonal gammopathy. Genetic testing of the proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1) gene revealed no mutations. LCV was confirmed by histology and direct immunofluorescence. Dapsone up to 300 mg daily was added to the prednisone and the PG lesion and LCV lesions resolved. Mutations in the promoter region of the PSTPIP1 gene have been identified in patients with PASH. Our patient had a steroid resistant vasculitis that developed while other PASH symptoms were also active. LCV has not been reported as a feature of an a feature of any of the autoinflammatory syndromes that include symptoms of PG and acne (PAPA, PAPASH, PASH) despite being a common complication of systemic autoimmune diseases like lupus and rheumatoid arthritis. Our patient fits the PASH phenotype but had no detected PSTPIP1 mutations, this is either a simulant of this syndrome or has some other genetic defect that can give similar symptoms. Other genes in PASH syndrome have been reported, including a mutation in nicastrin. Several treatments for PASH syndrome exist with varying degrees of efficacy, but no treatment has been accepted as standard therapy. Prolonged antibiotic therapy, topical tacrolimus ointment, and surgical ablation of abscesses and sinus tracts related to HS lesions have all been described. Systemic therapy with TNFa inhibitors, IL-1 receptor antagonists, corticosteroids, cyclosporine, and/or dapsone (on which our patient is now controlled) is often necessary given the chronic and relapsing nature of the disease. This case underscores the neutrophilic dermatoses can simulate genetic autoinflammatory syndromes, and that other neutrophilic disorders (ie, LCV) may complicate the management of these patients.
Commercial support: None identified.
Commercial support: None identified.
4795 Pyoderma gangrenosum- Case report Noelle Teixeira, B Pharm, Faculdade Santa Marcelina; Melissa Maeda, MBBS, Faculdade Santa Marcelina; Leonardo Narciso, MBBS, Hospital Santa Marcelina Pyoderma gangrenosum is a rare neutrophilic dermatosis. it is estimated that its incidence occurs between 3 to 10 cases per million people/year. The diagnosis is eminently clinical, and may be associated with systemic diseases. According to the literature, 50-80% of cases of this disease are related to other systemic diseases or surgical trauma, but during clinical investigation of the case the patient was not found no associated conditions beyond intense emotional stress, which makes this event even more special. The patient described features the PG in various locations and one of the only regions not affected was the head. The literature reports a good response to treatment of the PG with cyclosporina but the study patient had no good answer to this medication, one more peculiarity of this case. The treatment of choice for this case was the use of systemic corticosteroids chronically (prednisone by mouth 20 mg/day) and the debridement of necrotic lesions when necessary. The use of corticosteroids in this case is continuous because retreating attempts the same patient presented incidence of new lesions. Following to the case was concluded that, in addition to the high morbidity and mortality due to the PG described in the literature, in this patient the disease possessed very strong psychosocial influences, triggering the same chronic depression frame and the loss of self-esteem because of the scars deforming. The aim of this work is to draw attention to this uncommon and rare condition and provide a basis for diagnosis and treatment. A female patient, 47, white skin color, natural Quebrango, Alagoas, from S~ao Paulo, Brazil, retired employee of the hospital cleaning area. Seven years ago she had multiple bullous and painful lesions on left foot (Figure 1) accompanied by intense pain followed by necrosis and scarring. Denies trauma or any factor associated with the emergence of bubbles. Months later the patient reported appearance of lesions in the groin starts as a circular hyperemia with increased temperature at the site of injury, accompanied by severe burning pain. After a day of appearance of the same came a black crust on the injury concomitant to a feeling of ‘‘being eaten’’ inside. Disruption of the crust led to a serosanguineous secretion of extravasation with putrid odor. After improvement if felt intense itching at. Since then patient is presenting with injuries the same as presented in the groin wound above the lower limbs and upper bilaterally, groin, back and abdomen (Figure 2 and 3), following with a history of 38 hospitalizations due to this problem. The bullous lesion pattern was displayed only once. The same reports that in periods of stress injuries arise most frequently, as well as periods in which reduces prednisolone, steroid continuous use by the same dose of 20 mg in the morning. Was suspected of pyoderma gangrenosum was confirmed after biopsy of the lesion. Patient committed emotionally.
5653 Q-switched laser treatment of tattoos using a transparent perfluorodecalin-infused patch: A pivotal trial Brian Biesman, MD, Nashville Centre for Laser and Facial Surgery Objective: Q-switched laser treatment of tattoos produces opaque epidermal whitening that prevents multiple sequential passes from being made. Perfluorodecalin (PFD) is an optical clearing agent that prevents development of epidermal whitening when Q-switched lasers are used to treat tattoos. This pivotal trial was designed to quantitate the number of Q-switched laser passes that can be made through a transparent PFD-infused patch relative to the number that can be made through a conventional through-air laser interface in a defined, 5-minute session. Design: This was a split tattoo trial in which thirty subjects (mean age 37 years, range 19-54; 16 females and 14 males) with primarily black or dark blue tattoos were enrolled. One half of each tattoo was treated in the conventional manner through an air-tissue interface the other half was treated through a transparent PFD-infused patch. A Q-switched 755-nm Alexandrite laser was used to perform all treatments. The number of treatments that could be performed in a 5-minute time period was measured. Results: An average of 3.7 6 0.7 passes could be made through the PFD patch as compared to 1.4 6 0.6 passes through the air-tissue interface. On average, an additional 2.3 more passes (standard deviation ¼ 0.7; sign test P \.0001) could be made with the laser on the patch side of the tattoo relative to the side treated without the patch. There were no unanticipated adverse events in either treatment group. The rate of transient edema and erythema were lower in the PFD treatment group (36.7 vs 63% and 33.3 vs 70%, respectively). At the 1-month follow up visit, all subjects (30/30) expressed a desire to complete all further laser-assisted tattoo removal treatments with the PFD patch. Conclusion: The transparent PFD-infused patch enabled significantly more laser passes in a defined 5-minute laser treatment session than conventional treatment. Treatment of tattoos with a Q-switched Alexandrite laser through the patch was safe and well tolerated. Commercial support: This study was supported by ON Light Sciences, Inc.
Commercial support: None identified.
AB210
J AM ACAD DERMATOL
JUNE 2017
4682
Pustular polymorphic eruption and neutrophilic dermatosis associated with acute coccidioidomycosis Leah Swanson, MD, Mayo Clinic; Elika Hoss, MD, Mayo Clinic; Mark Pittelkow, MD, Mayo Clinic; Steven Nelson, MD, Mayo Clinic Coccidioidomycosis is a fungal infection endemic to the southwest US with increasing incidence. Varied cutaneous presentations including neutrophilic dermatoses are reported however rarely. A 49-year-old otherwise healthy woman presented with one month of nonproductive cough and one day of diffuse rash. She had a temperature of 39.38C, leukocytosis of 16.3, lobar consolidation on chest x-ray and was empirically started on antibiotics. Given negative viral and bacterial studies, fluconazole was initiated for concern of coccidioidomycosis. On the face and back she had 5-10 mm coalescing pink papules studded with pustules. On the chest, abdomen, arms, and legs she had pink targetoid papules with purpuric centers. Histopathology of pustular lesion showed neutrophilic dermal inflammation, pustule formation, and papillary dermal edema. Infectious stains and cultures were negative. Findings were most consistent with Sweet syndrome. Initial Coccidioides antibody studies and spherulin skin test were negative. She improved with topical corticosteroids and fluconazole. Three weeks after presentation, Coccidioides IgG antibody converted to positive by enzyme immunoassay. Cutaneous manifestations of coccidioidomycosis are reactive or represent skin infection by hematogenous dissemination or primary cutaneous inoculation. In disseminated and primary cutaneous infections, Coccidioides is observed microscopically and grows in culture from biopsy. Reactive eruptions, secondary to host immune responses, contain no Coccidioides and include erythema nodosum, acute exanthems, erythema multiforme, interstitial granulomatous dermatitis, and Sweet syndrome. Sweet syndrome is characterized by fever, leukocytosis, tender red skin lesions, and on histopathology a diffuse, dense, dermal neutrophilic infiltrate. Sweet syndrome is often preceded by upper respiratory tract infection, and several cases have been reported in association with acute pulmonary coccidioidomycosis. Pustules, a prominent feature in this case, superimposed on classic plaques of Sweet syndrome have rarely been reported in association with coccidioidomycosis. Systemic corticosteroids could exacerbate coccidioidomycosis and should be avoided. Although used to treat moderate to severe pulmonary infection, antifungals are not utilized in the treatment of reactive eruptions. Dermatologists should consider this fungal infection in patients with pustular polymorphic eruptions and travel to endemic regions.
Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome with recurrent vasculitis Dorene Niv, DO, St. Joseph Mercy Dermatology; James Ramirez, MD, St. Joseph Mercy Dermatopathology; David Fivenson, MD, St. Joseph Mercy Dermatology Autoinflammatory syndromes are characterized by recurrent episodes of sterile inflammation, in the absence of circulating autoantibodies and/or autoreactive Tcells. PASH syndrome is a recently identified hereditary autoinflammatory syndrome consisting of multiple neutrophilic dermatoses including: pyoderma gangrenosum (PG), acne, and hidradenitis suppurativa (HS). The triad of PG, acne, and HS occurs in extremely rare instances. To our knowledge, the clinical tetrad of PG, acne, HS, and vasculitis has not been reported. A 36-year-old man presented with abscesses increasing in size on the buttocks and axillae. His past medical history included recurrent PG on the lower extremities, generalized severe acne, and leukocytoclastic vasculitis (LCV) that developed while on 100 mg daily prednisone for a large PG lesion. Physical exam revealed cysts, papules and pustules with hyperpigmentation and scarring on the back and chest. Multiple inflammatory abscesses with sinus tracts and scarring were present on the lower back and buttocks. A 14 cm ulcer with violaceous, undermined borders, and palpable purpura extensively on the lower extremities. Lab evaluation revealed a bimodal IgG and IgA monoclonal gammopathy. Genetic testing of the proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1) gene revealed no mutations. LCV was confirmed by histology and direct immunofluorescence. Dapsone up to 300 mg daily was added to the prednisone and the PG lesion and LCV lesions resolved. Mutations in the promoter region of the PSTPIP1 gene have been identified in patients with PASH. Our patient had a steroid resistant vasculitis that developed while other PASH symptoms were also active. LCV has not been reported as a feature of an a feature of any of the autoinflammatory syndromes that include symptoms of PG and acne (PAPA, PAPASH, PASH) despite being a common complication of systemic autoimmune diseases like lupus and rheumatoid arthritis. Our patient fits the PASH phenotype but had no detected PSTPIP1 mutations, this is either a simulant of this syndrome or has some other genetic defect that can give similar symptoms. Other genes in PASH syndrome have been reported, including a mutation in nicastrin. Several treatments for PASH syndrome exist with varying degrees of efficacy, but no treatment has been accepted as standard therapy. Prolonged antibiotic therapy, topical tacrolimus ointment, and surgical ablation of abscesses and sinus tracts related to HS lesions have all been described. Systemic therapy with TNFa inhibitors, IL-1 receptor antagonists, corticosteroids, cyclosporine, and/or dapsone (on which our patient is now controlled) is often necessary given the chronic and relapsing nature of the disease. This case underscores the neutrophilic dermatoses can simulate genetic autoinflammatory syndromes, and that other neutrophilic disorders (ie, LCV) may complicate the management of these patients.
Commercial support: None identified.
Commercial support: None identified.
4795 Pyoderma gangrenosum- Case report Noelle Teixeira, B Pharm, Faculdade Santa Marcelina; Melissa Maeda, MBBS, Faculdade Santa Marcelina; Leonardo Narciso, MBBS, Hospital Santa Marcelina Pyoderma gangrenosum is a rare neutrophilic dermatosis. it is estimated that its incidence occurs between 3 to 10 cases per million people/year. The diagnosis is eminently clinical, and may be associated with systemic diseases. According to the literature, 50-80% of cases of this disease are related to other systemic diseases or surgical trauma, but during clinical investigation of the case the patient was not found no associated conditions beyond intense emotional stress, which makes this event even more special. The patient described features the PG in various locations and one of the only regions not affected was the head. The literature reports a good response to treatment of the PG with cyclosporina but the study patient had no good answer to this medication, one more peculiarity of this case. The treatment of choice for this case was the use of systemic corticosteroids chronically (prednisone by mouth 20 mg/day) and the debridement of necrotic lesions when necessary. The use of corticosteroids in this case is continuous because retreating attempts the same patient presented incidence of new lesions. Following to the case was concluded that, in addition to the high morbidity and mortality due to the PG described in the literature, in this patient the disease possessed very strong psychosocial influences, triggering the same chronic depression frame and the loss of self-esteem because of the scars deforming. The aim of this work is to draw attention to this uncommon and rare condition and provide a basis for diagnosis and treatment. A female patient, 47, white skin color, natural Quebrango, Alagoas, from S~ao Paulo, Brazil, retired employee of the hospital cleaning area. Seven years ago she had multiple bullous and painful lesions on left foot (Figure 1) accompanied by intense pain followed by necrosis and scarring. Denies trauma or any factor associated with the emergence of bubbles. Months later the patient reported appearance of lesions in the groin starts as a circular hyperemia with increased temperature at the site of injury, accompanied by severe burning pain. After a day of appearance of the same came a black crust on the injury concomitant to a feeling of ‘‘being eaten’’ inside. Disruption of the crust led to a serosanguineous secretion of extravasation with putrid odor. After improvement if felt intense itching at. Since then patient is presenting with injuries the same as presented in the groin wound above the lower limbs and upper bilaterally, groin, back and abdomen (Figure 2 and 3), following with a history of 38 hospitalizations due to this problem. The bullous lesion pattern was displayed only once. The same reports that in periods of stress injuries arise most frequently, as well as periods in which reduces prednisolone, steroid continuous use by the same dose of 20 mg in the morning. Was suspected of pyoderma gangrenosum was confirmed after biopsy of the lesion. Patient committed emotionally.
5653 Q-switched laser treatment of tattoos using a transparent perfluorodecalin-infused patch: A pivotal trial Brian Biesman, MD, Nashville Centre for Laser and Facial Surgery Objective: Q-switched laser treatment of tattoos produces opaque epidermal whitening that prevents multiple sequential passes from being made. Perfluorodecalin (PFD) is an optical clearing agent that prevents development of epidermal whitening when Q-switched lasers are used to treat tattoos. This pivotal trial was designed to quantitate the number of Q-switched laser passes that can be made through a transparent PFD-infused patch relative to the number that can be made through a conventional through-air laser interface in a defined, 5-minute session. Design: This was a split tattoo trial in which thirty subjects (mean age 37 years, range 19-54; 16 females and 14 males) with primarily black or dark blue tattoos were enrolled. One half of each tattoo was treated in the conventional manner through an air-tissue interface the other half was treated through a transparent PFD-infused patch. A Q-switched 755-nm Alexandrite laser was used to perform all treatments. The number of treatments that could be performed in a 5-minute time period was measured. Results: An average of 3.7 6 0.7 passes could be made through the PFD patch as compared to 1.4 6 0.6 passes through the air-tissue interface. On average, an additional 2.3 more passes (standard deviation ¼ 0.7; sign test P \.0001) could be made with the laser on the patch side of the tattoo relative to the side treated without the patch. There were no unanticipated adverse events in either treatment group. The rate of transient edema and erythema were lower in the PFD treatment group (36.7 vs 63% and 33.3 vs 70%, respectively). At the 1-month follow up visit, all subjects (30/30) expressed a desire to complete all further laser-assisted tattoo removal treatments with the PFD patch. Conclusion: The transparent PFD-infused patch enabled significantly more laser passes in a defined 5-minute laser treatment session than conventional treatment. Treatment of tattoos with a Q-switched Alexandrite laser through the patch was safe and well tolerated. Commercial support: This study was supported by ON Light Sciences, Inc.
Commercial support: None identified.
AB210
J AM ACAD DERMATOL
JUNE 2017