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P6721Patient with subcorneal pustular dermatosis: SneddoneWilkinsonsyndrome
Mar�ıa Salazar Nievas, San Cecilio Hospital, Granada, Spain; Mar�ıa AntoniaFernandez Pugnaire, San Cecilio Hospital, Granada, Spain; Mar�ıa TeresaGuti�errez Salmer�on, San Cecilio Hospital, Granada, Spain; Vicente Crespo Lora,San Cecilio Hospital, Granada, Spain
Overview: SneddoneWilkinson disease or subcorneal pustular dermatosis is astrange benign disease characterized by pustules or vesicles that quickly becomepustular. Therefore, sometimes the differential diagnosis can be complex.
Case report: A 46-year-old man was worried about the gradual appearance oferythematous and vesicular lesions on the trunk, legs, and arms that had beenspread, forming plaques of different sizes. The patient did not show relevant medicalhistory or other symptoms. His doctor provided him topical antifungals, under thesuspicion that he was suffering tinea corporis with no predictable improvement.Later, the patient was referred to the department of dermatology. The examinationsshown he presented small pustular and vesicular lesions on an erythematous base,which form plaques with well-defined edges and with circinate appearance inextremities and trunk. The differential diagnosis of psoriasis, mycosis fungoides,tinea corporis and SneddoneWilkinson syndrome was established. A punch biopsywas done with a sample taken from the edge of a plate located in the abdomen. Inthe hematoxylineeosin staining is shown clearly a subcorneal pustular dermatosis.Giemsa staining, PAS, and direct immunofluorescence were negative. The diagnosisof SneddoneWilkinson syndrome was set under these evidences. The patient wastreated with oral corticosteroids at doses of 1.5 mg/kg/day with improvement, notbeing necessary to establish another treatment. The patient remains stable, withdecreasing doses of oral corticosteroids and revisions in the department ofdermatology.
Conclusion: SneddoneWilkinson syndrome is an entity that often becomes chronicand affects the quality of life of patients. Some associations with monoclonalgammopathy have been detected, but it seems that they were casual, so there is noneed to do additional studies in patients affected by this syndrome. Oral cortico-steroids are usually ineffective and often it is necessary to set a treatment withdapsone. However, our patient improved significantly with oral prednisone. Lesionsare usually located in armpits, abdomen, groins, and flexor areas. Nevertheless, thiscase had an atypical distribution, with predominant extremities and trunk extensorareas troubles. This is an uncommon case of SneddoneWilkinson syndrome and westress the importance of a complete differential diagnosis to achieve the correcttreatment.
APRIL 20
cial support: None identified.
CommerP6668Patients’ experiences with hidradenitis suppurativa: A qualitative study ofsymptoms and impacts
Alexa Kimball, Department of Dermatology, Harvard Medical School, Boston,MA, United States; Martin Okun, Global Pharmaceutical Research andDevelopment, Abbott Laboratories, Abbott Park, IL, United States; MuraliSundaram, Global Health Economics and Outcomes Research, AbbottLaboratories, Abbott Park, IL, United States; Vasudha Mukherjee Bal, AdelphiValues, Boston, MA, United States
Backgroud: Hidradenitis suppurativa (HS) is a chronic skin condition characterizedby recurrent inflamed nodules, abscesses, scarring, and pain. HS can have a widerange of physical, psychological, and social impacts. Few studies have examined theeffects of this debilitating condition from the patient’s perspective.
Methods: We used open-ended, semistructured interviews to investigate symptomsand impacts of HS reported by 20 patients with moderate to severe HS (75%women;age range, 18-56 years). Patients were 1 to 12.9 years postdiagnosis, had 1 to 4anatomic areas of involvement, and were mostly Hurley stage II. Transcripts of theseinterviews were analyzed using a combination of grounded theory and traditionalcontent analysis methods to identify patient-reported HS symptoms and impacts,and assess frequency and degree of bother.
Results: Patients with HS used a variety of terms to describe their skin lesions,including ‘‘cysts,’’ ‘‘boils,’’ ‘‘hidradenitis,’’ ‘‘lesions,’’ ‘‘HS,’’ ‘‘acne,’’ and ‘‘abscess.’’ Themost frequently reported symptoms were pain (reported by 100% of patients),redness (85%), sensitivity/tenderness (60%), swelling/inflammation (60%), skinhardness (45%), cysts feeling hot or burning (40%), and pressure around cysts (40%).Other symptoms mentioned included a burning sensation, bad smell, itchy skin,fever, tightness of skin, and tingling. Patients were asked to rate their mostbothersome symptoms. Most patients (85%) reported pain as their most bothersomesymptom followed by swelling/inflammation (20%) and sensitivity/tenderness(10%). Frequent impacts, reported by at least 50% of patients, included clothingbeing uncomfortable, self-consciousness/embarrassment; sadness/depression, anx-iousness/annoyance; difficulties with physical activities such as moving arms,walking, sitting, and playing sports; and impacts on social, work/school, and sexualactivities. Patients also described financial, sleep, energy, health, and cognitiveimpacts. Impacts on ‘‘attendance of social activities/going out’’ and ‘‘sex activity’’were most frequently identified as the most bothersome (25% of patients each).
Conclusion: Patientswith HS reported a diverse range of unpleasant symptoms, withpain the most frequent and bothersome. HS also had considerable impact onactivities of daily living, work/school attendance, physical activities, and emotionalstates. These results form a new resource for understanding the HS experience asreported directly by patients.
y was funded by Abbott Laboratories.
This stud13
P5924Pityriasis rubra pilaris and arthritis: Is there a link?
Jacquelyn Levin, DO, Largo Medical Center, Largo, FL, United States
A 54-year-old white man presented with a rash all over for more than 10 years. Inaddition to constantly shedding skin, he admits to joint paint in the knees, feet, andhands for nearly 8 years. His joint pain causes morning stiffness at times and notablepain with walking, especially when ascending the stairs. He denies constitutionalsymptoms, sun sensitivity, and muscle weakness. He has a medical history ofhypothyroidism and denies a history of HIV, congestive heart failure, diabetes,psoriasis, or hepatitis. He denies any family history of psoriasis or rheumatoidarthritis. On our examination, the patient had erythroderma with follicular accen-tuation and scale on the trunk and arms, diffuse yellowish scaling on palms andsoles, scattered pustules on soles, and yellow hyperkeratotic toenails. Biopsies takenfrom the back and the palm demonstrate some alternating orthokeratosis andparakeratosis in both the vertical and horizontal directions, hypergranulosis in areas,acanthosis, a sparse to moderate lymphocytic perivascular infiltrate of the dermis,and dilated hair follicles that are and filled by orthokeratotic follicular plugs andextend above the level of the adjacent epidermis. Laboratory studies were negativefor antinuclear antibodies, rheumatoid factor (RF), HIV, and hepatitis.Rheumatologic evaluations revealed a seronegative arthritis with no erosion damageevident on radiographic examination. However, there was evidence of acroosteol-ysis, leading to autoamputation. Ourworking diagnosis is PRP type II (atypical adult)with a seronegative arthritis. There are at least 8 reported cases in the literature ofPRP with rheumatologic associations including seronegative arthritis and dermat-omyositis. These cases in the literature of PRP with a seronegative arthritis reportsimilar characteristics to our case, including the onset of the joint pain within yearsof the rash, acroosteolysis, and negative RF. Therapy suggestions in the literature forPRP with associated arthritis include acitretin, TNF-alfa inhibitors, methotrexate,and cyclosporine. Our Patient had minimal improvement on acitretin, adalimumab,methotrexate, and combinations of these 3 medicines however had significantimprovement in both skin and joint findings with infliximab.
cial support: None identified.
CommerP6295Plantar lichen sclerosus et atrophicus
R. Erin Lopez, MD, Department of Dermatology, Geisinger Medical Center,Danville, PA, United States; David L. Troutman, DPM, Department ofDermatology, Geisinger Medical Center, Danville, PA, United States; Eric W.Hossler, MD, Department of Dermatology, Geisinger Medical Center, Danville,PA, United States
Background: Lichen sclerosus et atrophicus is a chronic dermatitis that is usuallyfound on the anogenital skin. To our knowledge, we report the first case of plantarlichen sclerosus et atrophicus in the English literature.
Case report: A 72-year-old woman presented with a growing, tender area on her leftlateral sole for the past 3 years. She had been seen by several outside podiatrists andwas treated with several rounds of cryotherapy and pulsed-dye laser for presumedplantar warts. On examination, she had a tender 5-cm pink hyperkeratotic plaquewith an atrophic white border on left lateral sole. Two punch biopsies wereperformed; each demonstrated compact hyperorthokeratosis, a widened homoge-nized papillary dermis, and a sparse underlying band-like lymphoid infiltrate withunderlying dermal hyalinization. The findings were consistent with lichen sclerosuswith underlying changes of morphea. The patient was treated with clobetasol 0.05%cream nightly under occlusion. After 1 month, the plaque was significantly thinnerand her pain was somewhat improved. At 2 months, she showed continuedimprovement with only slight persistent discomfort with pressure.
Conclusion: We report an unusual presentation of lichen sclerosus et atrophicuswith features of underlying morphea on the sole. We are aware of only 2 previouscases of lichen sclerosus on the palms or soles, one in the Brazilian literature and theother in the French literature. Physicians and podiatrists should be aware of thisunusual entity and be familiar with its management.
cial support: None identified.
CommerJ AM ACAD DERMATOL AB57