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128


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
PMID: 21163175
ISSN: 1087-2108
CID: 115804

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
PMID: 21163164
ISSN: 1087-2108
CID: 115806

Hypertrophic discoid lupus erythematosus

Farley-Loftus, Rachel; Elmariah, Sarina B; Ralston, Jonathan; Kamino, Hideko; Franks, Andrew G Jr
Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed
PMID: 21163152
ISSN: 1087-2108
CID: 115878

Sarcoidal tattoo granuloma

Anolik, Robert; Mandal, Rajni; Franks, Andrew G Jr
A 41-year-old man presented for evaluation of a widespread eruption. The eruption started six months beforehand as bumps within pre-existing black tattoos over his trunk and arms. One month later, the patient developed eye pain and the diagnosis of a uveitis was made. A different eruption soon followed, which the patient described as patches of rough skin. Finally, the patient developed discrete patches of hair thinning. The clinical examination, history, skin biopsy findings, and elevated angiotensin-1 converting enzyme and immunoglobulin levels supported the diagnosis of systemic sarcoidosis, which manifested as a sarcoidal tattoo granuloma, perifollicular cutaneous sarcoidosis, and uveitis
PMID: 21163170
ISSN: 1087-2108
CID: 115892

Atypical mycobacteria infection in an immunocompromised patient

Berger, Emily; Batra, Priya; Ralston, Jonathan; Sanchez, Miguel R; Franks, Andrew G Jr
A 61-year-old woman with systemic lupus erythematosus and Sjogren syndrome presented with a two-month history of symptomatic nodules on the buttocks and thighs that progressed to involve the dorsal aspects of the hands. On examination, infiltrative papules, nodules, and plaques were present in these regions. Biopsy specimens demonstrated granulomatous inflammation and acid-fast bacilli with the use of a Fite stain, although a culture and polymerase chain reaction analysis were negative. The patient continues to improve on long-term clarithromycin therapy. Atypical mycobacterial infections are becoming more common, especially in immunocompromised patients. Antimicrobial therapy, either with a single agent or multiple agents, often is prolonged. A high index of suspicion is warranted in immunocompromised patients, which includes those with connective-tissue diseases that are active or that require immunosuppression. In these patients, the differential diagnosis includes infectious as well as inflammatory, reactive, or neoplastic processes
PMID: 21163172
ISSN: 1087-2108
CID: 115893

Laugier Hunziker syndrome

Jabbari, Ali; Gonzalez, Mercedes E; Franks, Andrew G Jr; Sanchez, Miguel
Laugier Hunziker syndrome is a rare disorder that is characterized by adult-onset hyperpigmented macules of the lips, oral cavity, and fingertips. Longitudinal melanonychia is present in the majority of cases. We present a 45-year-old woman with adult-onset hyperpigmented macules of the oral cavity as well as linear melanonychia that involved multiple fingernails. The history, clinical examination, and paucity of laboratory abnormalities or systemic findings support a diagnosis of Laugier Hunziker syndrome
PMID: 21163174
ISSN: 1087-2108
CID: 115894

Targeting the ICB2 site of the topoisomerase IIalpha promoter with a formamido-pyrrole-imidazole-pyrrole H-pin polyamide

Franks, Andrew; Tronrud, Christopher; Kiakos, Konstantinos; Kluza, Jerome; Munde, Manoj; Brown, Toni; Mackay, Hilary; Wilson, W David; Hochhauser, Daniel; Hartley, John A; Lee, Moses
The synthesis, DNA binding characteristics and biological activity of an N-formamido pyrrole- and imidazole-containing H-pin polyamide (f-PIP H-pin, 2) designed to selectively target the ICB2 site on the topoIIalpha promoter, is reported herein. Thermal denaturation, circular dichroism, isothermal titration calorimetry, surface plasmon resonance and DNase I footprinting studies demonstrated that 2 maintained the selectivity of the unlinked parent monomer f-PIP (1) and with a slight enhancement in binding affinity (K(eq)=5 x 10(5)M(-1)) to the cognate site (5'-TACGAT-3'). H-pin 2 also exhibited comparable ability to inhibit NF-Y binding to 1, as demonstrated by gel shift studies. However, in stark contrast to monomer 1, the H-pin did not affect the up-regulation of topoisomerase IIalpha (topoIIalpha) in cells (Western blot), suggesting that the H-pin does not enter the nucleus. This study is the first to the authors' knowledge that reports such a markedly different cellular response between two compounds of almost identical binding characteristics
PMID: 20615712
ISSN: 1464-3391
CID: 115889

Skin manifestations of internal disease

Franks, Andrew G Jr
Internal diseases can manifest in a myriad of skin dermatoses ranging from single disorders such as calciphylaxis, cryoglobulinemia, amyopathic dermatomyositis, and Raynaud phenomenon, to spectrum disorders such as the neutrophilic dermatoses and morphea. In this article the underlying causes, triggering events, constitutional symptoms, clinical features and presentations, appearance at various stages, and pathogenesis are described. The course of the diseases and probable healing outcomes are outlined. Finally, examination and diagnostic methods, and therapies and treatments are provided
PMID: 19932330
ISSN: 1557-9859
CID: 105510

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) [Case Report]

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
PMID: 19891917
ISSN: 1087-2108
CID: 105293

Eosinophilic fasciitis/generalized morphea overlap [Case Report]

Heidary, Noushin; Cheung, Wang; Wang, Nadia; Kamino, Hideko; Franks, Andrew G Jr
A 50-year-old woman presented with a three-month history of violaceous, non-tender, indurated plaques on the chest, abdomen, breasts, and proximal portions of the arms and legs. An incisional biopsy specimen showed changes consistent with a diagnosis of inflammatory morphea. Over the course of one year, the patient began to develop signs and symptoms suggestive of a diagnosis of eosinophilic fasciitis, which included the characteristic groove sign on the upper extremities. Although our patient did not exhibit peripheral or histopathologic evidence of eosinophilia, the diagnosis of eosinophilic fasciitis could still be made because the aforementioned phenomena are not required for diagnosis. Multitude treatment regimes have been reported in the literature as single case reports or small patient series. Our patient was maintained on methrotrexate, oral glucocorticoids, and etanercept with improvement of skin lesions and mobility
PMID: 19891910
ISSN: 1087-2108
CID: 107723