"Lupus erythematosus tumidus with discoid lupus erythematosus-induced alopecia of the scalp" [Case Report]Lehrhoff, Stephanie; Tzu, Julia; PATEL, RISHI; Sanchez, Miguel; Franks, Andrew G Jr
Lupus erythematosus tumidus (LET) is an uncommon variant of cutaneous lupus erythematosus. Lupus erythematodes tumidus is characterized by smooth, erythematous plaques on sun-exposed areas without surface changes, such as follicular plugs, atrophy, or scale. Histopathologic features include a lymphocytic, perivascular and periadnexal infiltrate with abundant interstitial, superficial, and deep dermal mucin without appreciable epidermal and vacuolar changes. Typically, LET is responsive to treatment with systemic antimalarials. We present a unique case of alopecia associated with LET, which was partially responsive to hydroxychloroquine. We also note that the plaque of LET is adjacent to the plaque of discoid lupus erythematosus..
"Metastatic hepatocellular carcinoma in a liver transplant patient presenting as cheek nodules"Mandell, Jenny A; Tlougan, Brook E; PATEL, RISHI R; Warycha, Melanie A; Kamino, Hideko; Haglof, Karen J
"Delayed and recurring blisters in the donor graft site of a burn patient"Anolik, Robert; Loyd, Aaron; PATEL, RISHI; Magro, Cynthia; Franks, Andrew G Jr
A 79-year-old woman presented for evaluation of non-healing skin graft donor sites. The patient underwent split thickness skin graft repair two-and-a-half years ago as a consequence of severe burns from a fire that affected 10 to 15 percent of her body. Donor sites included her thighs and flanks. After initial healing, intermittent and paroxysmal, eroded and crusted, erythematous plaques have continued to arise at various donor sites. Normal skin has remained uninvolved. Histopathologic analysis showed a poor basement membrane zone. The patient's findings represented delayed and recurring blistering in the donor graft site that is uncommonly observed in burn patients.
"Eruptive collagenomas"Batra, Priya; Loyd, Aaron; PATEL, RISHI; Walters, Ruth; Stein, Jennifer A
A 24-year-old woman presented with hypopigmented papules of the abdomen that had been present for four years without a family history of similar cutaneous findings or associated medical problems. Histopathologic features confirmed the diagnosis of a connective-tissue nevus that was composed of collagen. Eruptive collagenomas are a rare form of acquired collagenomas, which are characterized by the sudden appearance of asymptomatic papules and nodules on the lower trunk and extremities; the lesions are composed of haphazardly arranged collagen fibers. The pathogenesis is unknown, lesions are persistent, and therapeutic options have not been reported.
"Livedo racemosa, secondary to drug-induced systemic lupus erythematosus"Defelice, Taylor; Lu, Phoebe; Loyd, Aaron; PATEL, RISHI; Franks, Andrew G Jr
We present a 40-year-old man with erythematous-to-violaceous, broken, reticulated patches on the upper chest, back, and extremities, which is consistent with livedo racemosa. The cutaneous findings appeared after an increase in dilantin dose and subsequently improved after a reduction in dilantin dose. Furthermore, antinuclear antibodies and antihistone antibodies were detected. We therefore believe that the livedo racemosa is a cutaneous manifestation of a drug-induced systemic lupus erythematosus. We review the distinctive features of livedo racemosa as well as its associations with several disorders. Although there are no effective treatments for livedo racemosa, patients often are placed on low-dose aspirin and counseled to avoid smoking in an effort to protect against their increased risk of stroke and arterial thrombosis.
"Primary cutaneous anaplastic large-cell lymphoma"Newlove, Tracey; Loyd, Aaron; PATEL, RISHI; Jelinek, Josef; Latkowski, Jo-Ann
Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma that is characterized by solitary or localized nodules or plaques. Histopathologic features include a diffuse, non-epidermotropic infiltrate with cohesive sheets of large anaplastic CD30+ tumor cells. This entity must be distinguished from systemic ALCL with cutaneous involvement and lymphomatoid papulosis. Treatment modalities include clinical monitoring, radiation therapy, and surgical excision, with systemic chemotherapy reserved for disseminated or extracutaneous disease.
"Secondary follicular mucinosis associated with systemic lupus erythematosus"O'Reilly, Kathryn; Brauer, Jeremy; Loyd, Aaron; PATEL, RISHI; Walters, Ruth; Franks, Andrew G Jr
A 61-year-old woman presented with a five-month history of an intermittent eruption of papules and nodules on her face and neck. Past medical history included systemic lupus erythematosus. Histopathologic examination was consistent with secondary follicular mucinosis in association with systemic lupus erythematosus. This rare entity has been described in one prior report as a precursor to the clinical onset of systemic lupus erythematosus. Follicular mucinosis occurs as either a primary idiopathic form or a secondary form associated with either benign inflammatory processes or malignant conditions, such as cutaneous T-cell lymphoma. Numerous treatments for primary follicular mucinosis have been described, which include isotretinoin and glucocorticoids, whereas treatment of the underlying disease is necessary in the secondary form. The association with lymphoma mandates long-term clinical monitoring of patients with particularly recalcitrant, widespread, or chronic follicular mucinosis.
"Acquired perforating calcific collagenosis after topical calcium chloride exposure"PATEL, RISHI R; Zirvi, Monib; Walters, Ruth F; Kamino, Hideko
A 24-year-old healthy man presented with a 6-week history of numerous umbilicated coalescing erythematous papules with some scale and crust on his anterior medial thighs. The eruption began 1 to 2 weeks after he spilled calcium chloride rock salts on his pants while salting the sidewalk during a snow storm. The salts dissolved and remained in contact with his skin for at least 4 hours until he was able to change clothes. A skin biopsy shows thick and thin collagen fibers with partial calcification in the papillary and upper reticular dermis associated with a sparse infiltrate of neutrophils, lymphocytes and mononuclear histiocytes. There are foci of transepidermal elimination of calcified fibers with adjacent epidermal hyperplasia and ortho- and parakeratosis. Von Kossa stain highlights calcification of the fibers, and trichrome stain confirms the fibers are collagen. A Verhoeff-van Gieson stain shows no abnormality of elastic fibers. The patient was treated with topical betametasone diproprionate cream twice daily for 3 weeks, as well as a short course of oral levofloxacin and topical gentamicin cream. The lesions resolved over 3 weeks with residual scarring. We report a unique case of acquired perforating calcific collagenosis secondary to topical calcium chloride exposure. Patel RR, Zirvi M, Walters RF, Kamino H. Acquired perforating calcific collagenosis after topical calcium chloride exposure.
"Necrolytic acral erythema"Patel, Utpal; Loyd, Aaron; PATEL, RISHI; Meehan, Shane; Kundu, Roopal
Necrolytic acral erythema (NAE) is a recently recognized dermatosis almost exclusively associated with hepatitis C virus (HCV) infection and closely related to a group of necrolytic erythemas and metabolic syndromes. NAE is characterized by pruritic, symmetric, well-demarcated, hyperkeratotic, erythematous-to-violaceous, lichenified plaques with a rim of dusky erythema on the dorsal aspects of the feet and extending to the toes. Based on morphology and histopathologic features, NAE can be difficult to distinguish from certain groups of necrolytic erythemas, which include necrolytic migratory erythema, acrodermatitis enteropathica, biotin deficiency, niacin deficiency, and essential fatty acid deficiencies. The condition is particularly important for clinicians to diagnose because the majority of the patients present to dermatologists without a known history of HCV infection. Thus, NAE can serve as a cutaneous marker for underlying HCV infection. Resolution of NAE can be achieved by treatment of the underlying HCV infection and the use of oral zinc therapy.
"Keratitis-ichthyosis-deafness (KID) syndrome"Gonzalez, Mercedes E; Tlougan, Brook E; Price, Harper N; PATEL, RISHI; Kamino, Hideko; Schaffer, Julie V
A 21-year-old man presented with a life-long history of diffusely thickened skin with a grainy-to-ridged surface, verrucous perioral plaques with radial fissures, and diffuse palmoplantar keratoderma with a stippled appearance. These skin findings were accompanied by sensorineural hearing loss and keratoconjunctivitis, a clinical triad diagnostic of keratitis-ichthyosis-deafness (KID) syndrome. The patient also had a history of recurrent infections and cysts on the scalp. This report draws attention to inflammatory nodules (representing ruptured folliculitis), cysts, and recurrent infections on the scalp as manifestations of KID syndrome and reviews the increasingly recognized risk of follicular tumors and squamous-cell carcinomas in patients with this conditions.
"Porokeratosis palmaris et plantaris disseminata or a disseminated late-onset variant of porokeratotic eccrine ostial and dermal ductal nevus (PEODDN) with follicular involvement"Hartman, Rachael; Rizzo, Carina; PATEL, RISHI; Kamino, Hideko; Shupack, Jerome L
A 48-year-old woman presented with a four-year history of pruritic, hyperkeratotic, spiny papules that began on her chest and spread to her extremities, groin, palms, face, and scalp where it caused non-scarring alopecia. Histopathologic features included cornoid lamella, which is the hallmark of porokeratosis. However, the patient's constellation of findings does not meet diagnostic criteria for any of the five clinical variants of porokeratosis. Her presentation is most compatible with either porokeratosis palmaris et plantaris disseminata (PPPD), which is a rare variant of punctate porokeratosis that can involve any area of the body or late-onset porokeratotic eccrine ostial and dermal ductal nevus (PEODDN), which is a rare, benign hamartoma of the eccrine sweat glands with porokeratotic histopathologic features that has been reported to occasionally have systemic involvement. Treatment of either condition is difficult although there have been reported successes with cryotherapy, surgical excision, and CO2 laser. Since porokeratosis is a disorder of keratinization and our patient has prominent follicular involvement, isotretinoin may be another reasonable therapeutic option.
"Genital porokeratosis"Liang, Christine; Batra, Priya; PATEL, RISHI; Kamino, Hideko
A 22-year-old man presented with a two-year history of warts on the penis, scrotum, and thighs. Physical examination showed multiple annular plaques with thin, threadlike borders on the penis and scrotum. The biopsy specimen showed a cornoid lamella with underlying dyskeratotic cells that was consistent with porokeratosis. Genital porokeratosis is a rare condition that may be misdiagnosed as a sexually transmitted disease.
"POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes)"Liang, Christine; Gonzalez, Mercedes; PATEL, RISHI; Meehan, Shane; Kamino, Hideko; Franks, Andrew G Jr
A 62-year-old woman with hypothyroidism presented with a seven-year history of paresthesias, itching, and edema of the skin. Physical examination showed indurated, edematous plaques on the lower extremities. A biopsy specimen showed increased mucin deposition that was consistent with myxedema, and monoclonal IgM was observed on immunofixation. The constellation of findings, which included paresthesias, endocrinopathy, monoclonal gammopathy, and skin changes was consistent with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome, which is a rare multisystemic disease that is associated with an underlying plasma-cell dyscrasia.
"Fixed cutaneous sporotrichosis"Mahlberg, Matthew J; PATEL, RISHI; Rosenman, Karla; Cheung, Wang; Wang, Nadia; Sanchez, Miguel
Sporotrichosis is a subcutaneous mycosis that is caused by Sporothrix schenckii. Whereas the most common clinical presentation is the lymphocutaneous form, the fixed cutaneous form is not uncommon. Important clues from the clinical history, such as travel and occupation, can help to raise the suspicion of this infection in the differential diagnosis. However, histopathologic and tissue culture, which often require multiple specimens, are necessary to confirm the diagnosis. We present a patient with fixed cutaneous sporotrichosis whose delayed diagnosis led to appreciable scars and morbidity.
"Cutaneous piloleiomyomata"Smith, Gideon; Heidary, Noushin; PATEL, RISHI; Rosenman, Karla; Meehan, Shane A; Kamino, Hideko; Sanchez, Miguel
A 49-year-old man presented with an eight-month history of intermittently painful, subcutaneous nodules that were increasing in size, number, and pain intensity. A biopsy specimen showed smooth muscle proliferation, which also stained positive for actin, and was consistent with piloleiomyoma. The patient was placed initially on gabapentin and then nifedipine with very limited success in pain control. The lesions continued to proliferate, and the patient was referred to surgery for excision.
"Familial benign chronic pemphigus (Hailey-Hailey disease)"Warycha, Melanie; PATEL, RISHI; Meehan, Shane; Merola, Joseph F
A 57-year-old woman presented with a 27-year history of vesicles and crusted erosions of the intertriginous folds. Prior treatments had included topical glucocorticoids, mupirocin, and minocycline, all of which provided minimal relief. A skin biopsy specimen was consistent with Hailey-Hailey disease. Hailey-Hailey disease is the result of mutations in the ATP2C1 gene and is characterized by recurrent vesicles and erosions in intertriginous areas. Whereas topical and/or systemic antibiotic/antifungal agents in combination with topical glucocorticoids is the mainstay of treatment, case reports have documented dramatic improvement with oral retinoids, calcitriol, and tacrolimus.
"Erythema nodosum in association with newly diagnosed hairy cell leukemia and group C streptococcus infection"PATEL, RISHI R; Kirkland, Eugene B; Nguyen, Dennis H; Cooper, Brenda W; Baron, Elma D; Gilliam, Anita C
GRANTS:P30-AR 39750/AR/NIAMS NIH HHS/United States;R01 AR 049284/AR/NIAMS NIH HHS/United States
Erythema nodosum is an inflammatory reaction of the skin characterized by tender erythematous patches or nodules, usually located on the lower extremities. This report illustrates an association of erythema nodosum with a rare malignancy and an uncommon infectious agent in humans. There are many diseases associated with erythema nodosum; we propose that hairy cell leukemia and group C streptococcus be considered among this list.
"Outcomes of root canal treatment and restoration, implant-supported single crowns, fixed partial dentures, and extraction without replacement: a systematic review"Torabinejad, Mahmoud; Anderson, Patricia; Bader, Jim; Brown, L Jackson; Chen, Lie H; Goodacre, Charles J; Kattadiyil, Mathew T; Kutsenko, Diana; Lozada, Jaime; PATEL, RISHI; Petersen, Floyd; Puterman, Israel; White, Shane N
STATEMENT OF PROBLEM: Dentists and patients are regularly confronted by a difficult treatment question: should a tooth be saved through root canal treatment and restoration (RCT), be extracted without any tooth replacement, be replaced with a fixed partial denture (FPD) or an implant-supported single crown (ISC)? PURPOSE: The purpose of this systematic review was to compare the outcomes, benefits, and harms of endodontic care and restoration compared to extraction and placement of ISCs, FPDs, or extraction without tooth replacement. MATERIAL AND METHODS: Searches performed in MEDLINE, Cochrane, and EMBASE databases were enriched by hand searches, citation mining, and expert recommendation. Evidence tables were developed following quality and inclusion criteria assessment. Pooled and weighted mean success and survival rates, with associated confidence intervals, were calculated for single implant crowns, fixed partial dentures, and initial nonsurgical root canal treatments. Data related to extraction without tooth replacement and psychosocial outcomes were evaluated by a narrative review due to literature limitations. RESULTS: The 143 selected studies varied considerably in design, success definition, assessment methods, operator type, and sample size. Direct comparison of treatment types was extremely rare. Limited psychosocial data revealed the traumatic effect of loss of visible teeth. Economic data were largely absent. Success rates for ISCs were higher than for RCTs and FPDs, respectively; however, success criteria differed greatly among treatment types, rendering direct comparison of success rates futile. Long-term survival rates for ISCs and RCTs were similar and superior to those for FPDs. CONCLUSIONS: Lack of comparative studies with similar outcomes criteria with comparable time intervals limited comparison of these treatments. ISC and RCT treatments resulted in superior long-term survival, compared to FPDs. Limited data suggested that extraction without replacement resulted in inferior psychosocial outcomes compared to alternatives. Long-term, prospective clinical trials with large sample sizes and clearly defined outcomes criteria are needed.