"Experience With Molluscum Contagiosum and Associated Inflammatory Reactions in a Pediatric Dermatology Practice: The Bump That Rashes"Berger, Emily M; Orlow, Seth J; PATEL, RISHI R; Schaffer, Julie V
OBJECTIVE To investigate the frequency, epidemiology, clinical features, and prognostic significance of inflamed molluscum contagiosum (MC) lesions, molluscum dermatitis, reactive papular eruptions resembling Gianotti-Crosti syndrome, and atopic dermatitis in patients with MC. DESIGN Retrospective medical chart review. SETTING University-based pediatric dermatology practice. PATIENTS A total of 696 patients (mean age, 5.5 years) with molluscum. MAIN OUTCOME MEASURES Frequencies, characteristics, and associated features of inflammatory reactions to MC in patients with and without atopic dermatitis. RESULTS Molluscum dermatitis, inflamed MC lesions, and Gianotti-Crosti syndrome-like reactions (GCLRs) occurred in 270 (38.8%), 155 (22.3%), and 34 (4.9%) of the patients, respectively. A total of 259 patients (37.2%) had a history of atopic dermatitis. Individuals with atopic dermatitis had higher numbers of MC lesions (P < .001) and an increased likelihood of molluscum dermatitis (50.6% vs 31.8%; P < .001). In patients with molluscum dermatitis, numbers of MC lesions increased during the next 3 months in 23.4% of those treated with a topical corticosteroid and 33.3% of those not treated with a topical corticosteroid, compared with 16.8% of patients without dermatitis. Patients with inflamed MC lesions were less likely to have an increased number of MC lesions over the next 3 months than patients without inflamed MC lesions or dermatitis (5.2% vs 18.4%; P < .03). The GCLRs were associated with inflamed MC lesion (P < .001), favored the elbows and knees, tended to be pruritic, and often heralded resolution of MC. Two patients developed unilateral laterothoracic exanthem-like eruptions. CONCLUSIONS Inflammatory reactions to MC, including the previously underrecognized GCLR, are common. Treatment of molluscum dermatitis can reduce spread of MC via autoinoculation from scratching, whereas inflamed MC lesions and GCLRs reflect cell-mediated immune responses that may lead to viral clearance..
"Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus (lupus ab-igne)"Berger, Emily; Robinson, Maria; PATEL, RISHI; Franks, Andrew G Jr
A 63-year-old woman with a ten-year history of systemic lupus erythematosus presented with a three-year history of a large, pruritic lesion that was located on the back and a pruritic lesion that was located on the right thumb. She was a baker by profession for many years during which time she often stood with her back to a hot oven. Physical examination of the lower back showed a large, atrophic, and reticulated plaque with a hyperpigmented border. On the right thumb was a well-demarcated, erythematous plaque with adjacent loss of the medial nail plate on the affected finger. Histopathology of both skin lesions showed cutaneous lupus erythematosus. This patient displays the Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus. We propose the term lupus ab-igne to describe her skin lesions. There are a few reports of the Koebner phenomenon occurring in patients with lupus erythematosus in response to contact dermatitis, tattoos, zoster, operative scars, scratching, or pressure from clothing. To our knowledge, this is the first report of heat as the physical factor leading to the formation of cutaneous lupus lesions..
"Poikilodermatous mycosis fungoides"Bloom, Bradley; Marchbein, Shari; Fischer, Max; Kamino, Hideko; PATEL, RISHI; Latkowski, Jo-Ann
Poikilodermatous mycosis fungoides (MF) is a variant of MF, formerly referred to as poikiloderma vasculare atrophicans. The lesions are classically characterized by large plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases. The plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk. Poikilodermatous MF has an early stage (IA-IIA) at diagnosis and a male predominance. Poikilodermatous MF shows an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Recent studies suggest a predominance of a CD8+, CD4- immunophenotype. Treatment modalities are similar to classic MF with phototherapy being the most common first-line therapy. Poikilodermatous MF has an excellent prognosis..
"Amelanotic melanoma: a detailed morphologic analysis with clinicopathologic correlation of 75 cases"Cheung, Wang L; PATEL, RISHI R; Leonard, Aimee; Firoz, Bahar; Meehan, Shane A
Amelanotic melanoma can have a varied appearance both clinically and microscopically. Here, we present our experiences with 75 cases of amelanotic melanoma defined clinically as a non-pigmented lesion and histopathologically as a tumor lacking significant melanization. We evaluated microscopic features such as morphology, mitotic count, nuclear atypia and presence of solar elastosis. Our amelanotic melanomas exhibited the following morphology: epitheloid (72%), spindled (18.7%) or desmoplastic (5.3%). In addition, we obtained patient information and clinical presentations on most of the cases (74/75; 98.7%) and follow-up data on 40% (30/75) of the cases. The majority of amelanotic melanomas in men were found on the trunk (13/45; 29%), head and neck (12/45; 26.7%), and lower limb (13/45; 29%) and in women were found on the lower limb (12/30; 40%), upper limb (10/30; 33.3%) and head and neck (6/30; 20%). In addition, we found that an increase in mitotic index correlated with worse survival (p < 0.026), whereas there were no differences in survival for other pathological features, such as nuclear atypia or solar elastosis. Furthermore, in cases with available tissue, all amelanotic melanoma expressed microphthalmia-associated transcription factor and tyrosinase, suggesting that the tumor cells retained melanocytic lineage and an enzyme in melanin formation, respectively. As the occurrence of amelanotic melanoma and the expression melanoma markers were similar to pigmented melanoma, we favor that amelanotic melanoma represents a subtype of melanoma rather than poorly differentiated or de-differentiated melanoma. Cheung WL, Patel RR, Leonard A, Firoz B, Meehan SA. Amelanotic melanoma: a detailed morphologic analysis with clinicopathologic correlation of 75 cases.
"Acrokeratosis verruciformis"Defelice, Taylor; Robinson, Maria; PATEL, RISHI; Kamino, Hideko
We present a case of acrokeratosis verruciformis of Hopf in a 58-year-old woman with multiple, skin-colored, flat-topped, hyperkeratotic papules on the dorsal aspects of the hands and feet. Her lesions appeared at age 13 and her son developed similar lesions at about the same age. Acrokeratosis verruciformis of Hopf is a rare genodermatosis, which is distinct from keratosis follicularis; sporadic cases have also been described. Management options include cryotherapy, keratolytics, superficial ablation with CO2 or Nd:YAG lasers, topical retinoids, and acitretin..
"Anetoderma secondary to antiphospholipid antibodies"Eungdamrong, John; Fischer, Max; PATEL, RISHI; Meehan, Shane; Sanchez, Miguel
Anetoderma is an elastolytic disorder that is associated with a number of infectious and autoimmune disorders. We present a case of a patient with generalized anetoderma, who was later found to have positive antinuclear antibodies and antiphospholipid antibodies (APAs). Numerous other cases have been reported in literature and some authors have suggested that anetoderma is a highly specific sign of APAs, with or without other manifestations of systemic lupus erythematosus or antiphospholipid syndrome . Thus, work up for connective-tissue disorders should be considered in any patients who present with this skin finding..
"Generalized essential telangiectasia"Gordon Spratt, Elizabeth A; Defelice, Taylor; Robinson, Maria; PATEL, RISHI R; Sanchez, Miguel
Generalized essential telangiectasia, which is a rare condition that is characterized by the progressive development of telangiectases on the skin, is a clinical diagnosis of exclusion. We present a 65-year-old man with a ten-month history of an asymptomatic eruption of the trunk and proximal aspects of the arms and hands that was comprised of macules and patches of telangiectases. The clinical presentation, associated diseases, hypotheses regarding pathogenesis, differential diagnoses, and reports on treatment modalities are reviewed. The relatively new association of this entity with systemic signs that include hemorrhage as well as the occurrence of generalized essential telangiectasia in patients with a history of hepatitis is discussed..
"Antiphospholipid-antibody-associated panniculitis"Hunt, Raegan D; Robinson, Maria; PATEL, RISHI; Franks, Andrew G Jr
A 60-year-old man presented with intermittent, tender, erythematous nodules on the legs that were associated with mild arthralgias. He was otherwise asymptomatic but reported a history of lupus anticoagulant antibodies that were discovered incidentally on laboratory screening at the approximate time that his lesions first occurred. A biopsy specimen showed a septal and lobular panniculitis with neutrophils, histiocytes, numerous eosinophils, foci of fibrosis, and fat necrosis but no vascular pathology. An elevated activated partial thromboplastin time (PTT), appreciably elevated levels of anti-beta-2 glycoprotein I antibody (IgM and IgG), and moderately elevated levels of anticardiolipin antibody (IgM and IgG) were present. The onset and recurrence of his skin condition coincided with increased antiphospholipid antibody levels and treatment with 81 mg aspirin daily was associated with improvement..
"Pityriasis rubra pilaris"Leger, Marie; Newlove, Tracey; Robinson, Maria; PATEL, RISHI; Meehan, Shane; Ramachandran, Sarika
Pityriasis rubra pilaris (PRP) is an inflammatory condition of uncertain etiology. We present a case of PRP that demonstrates acantholysis on histopathologic examination, which is a characteristic feature that increasingly is recognized to aid in the diagnosis. We also review the clinical presentations and treatment options for this condition..
"Serratia marcescens folliculitis and concomitant acne vulgaris"Lehrhoff, Stephanie; Yost, John; Robinson, Maria; PATEL, RISHI; Sanchez, Miguel
We present a unique case of S. marcescens folliculitis of the trunk in a 46-year-old woman with a history of facial acne vulgaris during her teen years. Her eruption occurred at the time of elective ambulatory surgery when she was treated with pre and post-operative antibiotics. The diagnosis of S. marcescens folliculitis was made on the basis of histopathologic features and tissue culture of a skin biopsy specimen of a pustule after her eruption was unresponsive to conventional treatment for inflammatory acne vulgaris. The history and pathophysiology of gram-negative folliculitis in the setting of acne vulgaris is reviewed..
"Diffuse large B-cell lymphoma"Mundi, Jyoti P; Leger, Marie; Terushkin, Vitaly; Fischer, Max; PATEL, RISHI; Meehan, Shane; Latkowski, Jo-Ann
We present a 56-year-old man with a two-year history of erythematous nodules and plaques on the forehead, frontal aspect of the scalp, and left side of the neck. Histopathologic findings are compatible with a diagnosis of diffuse large B-cell lymphoma (DLBCL). We present a brief review of primary cutaneous DLBCLs and address the putative association between DLBCLs and hepatitis C virus infection..
"Dissecting cellulitis of the scalp"Mundi, Jyoti P; Marmon, Shoshana; Fischer, Max; Kamino, Hideko; PATEL, RISHI; Shapiro, Jerry
Dissecting cellulitis of the scalp is a chronic, relapsing, inflammatory disease of the scalp that results in scarring alopecia. We present a case of a 32-year-old man with recalcitrant disease who is now responding to treatment with isotretinoin. The pathogenesis, clinical presentation, disease associations, and histopathological findings are reviewed. Treatment can be challenging. The literature on medical and surgical therapeutic options is reviewed..
"Eruptive vellus hair cysts"Patel, Utpal; Terushkin, Vitaly; Fischer, Max; Kamino, Hideko; PATEL, RISHI
Eruptive vellus hair cyst (EVHC), described initially in 1977, is a benign dermatologic condition that is characterized by the sudden appearance of monomorphic, follicular, asymptomatic, small papules in children and young adults. The diagnosis is based on the histopathologic findings of stratified-squamous epithelium with a granular layer that surrounds a cystic space filled with laminated keratin and a variable number of vellus hair cysts. EVHC can be associated with steatocystoma multiplex. A current hypothesis suggests that EVHC originates from a cystic change at the insertion of the pilosebaceous duct. EVHC is primarily treated for cosmesis with retinoids, surgery, and lasers..
"Verrucous lymphangioma circumscriptum"Terushkin, Vitaly; Marmon, Shoshana; Fischer, Max; PATEL, RISHI; Sanchez, Miguel R
A 19-year-old woman with a seven-year history of pruritic, grouped, verrucous papules and plaques on the left arm presented for consultation. The lesion slightly flattened in appearance with topical glucocorticoid treatment. The histopathological features were consistent with lymphangioma circumscriptum. This entity is the most common subtype of lymphangioma and presents with grouped, clear vesicles that may appear pink to purple. However, a rare verrucous type can present a diagnostic challenge. Whereas the definitive treatment option is surgical excision, other treatments, which include sclerotherapy, radiotherapy, and laser therapy, have been reported to cause resolution or improvement. In the future, propranolol may hold promise as a medical therapy for lymphangioma..
"Spiradenomas"Tran, Kathleen; Defelice, Taylor; Robinson, Maria; PATEL, RISHI R; Sanchez, Miguel
We report a 52-year-old man with a one-year history of multiple, firm, skin-colored nodules on the vertex of the scalp. Histopathologic examination was consistent with a spiradenoma, which is a rare, benign adnexal tumor of controversial histogenesis. Multiple spiradenomas may arise in association with Brooke-Spiegler syndrome, which is an autosomal dominant condition of multiple cyclindromas, trichoeptheliomas, and cyclindromas..
"Brooke-Spiegler syndrome"Trufant, Joshua; Robinson, Maria; PATEL, RISHI
A 50-year-old woman presented with a long history of multiple, flesh-colored papules and nodules on her central face, neck, and upper shoulders, as well as pink papules on her scalp. They were neither pruritic nor tender, but the patient desired treatment for cosmesis. Histopathologic examination of a representative facial lesion was consistent with a trichoepithelioma and an upper forehead papule was consistent with cylindroma. She reported a positive family history of similar lesions..
"Ras mutation cooperates with beta-catenin activation to drive bladder tumourigenesis"Ahmad, I; PATEL, R; Liu, Y; Singh, L B; Taketo, M M; Wu, X-R; Leung, H Y; Sansom, O J
GRANTS:Cancer Research UK/United Kingdom;Medical Research Council/United Kingdom
Mutations in the Ras family of proteins (predominantly in H-Ras) occur in approximately 40% of urothelial cell carcinoma (UCC). However, relatively little is known about subsequent mutations/pathway alterations that allow tumour progression. Indeed, expressing mutant H-Ras within the mouse bladder does not lead to tumour formation, unless this is expressed at high levels. The Wnt signalling pathway is deregulated in approximately 25% of UCC, so we examined if this correlated with the activation of MAPK signalling in human UCC and found a significant correlation. To test the functional significance of this association we examined the impact of combining Ras mutation (H-Ras(Q61L) or K-Ras(G12D)) with an activating beta-catenin mutation within the mouse bladder using Cre-LoxP technology. Although alone, neither Ras mutation nor beta-catenin activation led to UCC (within 12 months), mice carrying both mutations rapidly developed UCC. Mechanistically this was associated with reduced levels of p21 with dependence on the MAPK signalling pathway. Moreover, tumours from these mice were sensitive to MEK inhibition. Importantly, in human UCC there was a negative correlation between levels of p-ERK and p21 suggesting that p21 accumulation may block tumour progression following Ras mutation. Taken together these data definitively show Ras pathway activation strongly cooperates with Wnt signalling to drive UCC in vivo.
"Leukemia cutis in association With Grover's disease"Aldabagh, Bishr; PATEL, RISHI R; Honda, Kord
Grover's disease (GD), or transient acantholytic dermatosis, is a persistent recurrent dermatosis that usually occurs in men older than 50 years. Rare cases of GD and hematologic malignancy in the same cutaneous biopsy specimen have been reported. We report a case of GD in association with leukemia cutis. A 72-year-old man with a history of myelodysplastic syndrome presented with numerous pruritic papules on the torso, which were clinically diagnosed as GD. A skin biopsy revealed foci of suprabasal acantholysis and dyskeratosis consistent with GD and dense aggregates of mononuclear atypical cells in the superficial dermis consistent with leukemia cutis. Direct immunofluorescence was negative. This case illustrates the need to consider a diagnostic skin biopsy in any patient who presents with classic clinical findings of GD if there is any indication that the patient may be at higher risk for a hematologic malignancy.